1
Q
which cerebral lobe is involved? • Difficulties with task sequencing • Difficulties with executive skills • Expressive aphasia (Broca’s) • Anosmia
A
Frontal lobe
Broca’s area: located in the posterior aspect of the frontal lobe, in the inferior frontal gyrus
2
Q
which cerebral lobe is involved? • Primitive reflexes • Perseveration (repeatedly asking same question or doing same task) • Changes in personality • Inability to generate a list • Disinhibition
A
Frontal lobe
3
Q
which cerebral lobe is involved?
• Apraxias: loss of the ability to execute learned purposeful movements
• Neglect
• Astereognosis (unable to recognise object by feeling) = tactile agnosia
• Homonymous inferior quadrantanopia
• Sensory inattention
• Acalculia: inability to perform mental arithmetic
A
Parietal lobe
4
Q
what is Gerstmann’s syndrome? where is the lesion?
A
Gerstmann's syndrome (lesion of dominant parietal): o Alexia: in ability to read o Acalculia oFinger agnosia oRight-left disorientation.
5
Q
which cerebral lobe is involved?
• Homonymous superior quadrantanopia
• Prosopagnosia (difficulty recognising
faces)
A
Temporal lobe
6
Q
which cerebral lobe is involved?
• Wernike’s (recepTive) aphasia
• Memory impairment
• Auditory agnosia
A
Temporal lobe
7
Q
which cerebral lobe is affected?
• Cortical blindness (blindness due to damage to visual cortex, may present as Anton syndrome: there is blindness but patient is unaware or denies blindness)
• Homonymous hemianopia
A
occipital lobe
8
Q
which cerebral lobe is affected?
• Visual agnosia (seeing but not percieving objects - it is different to neglect since in agnosia the objects are seen and followed but cannot be named)
A
occipital lobe
9
Q
which column is involved in joint position and light touch?
A
Dorsal column dysfunction: (joint position and light touch)
10
Q
which column is involved in pinprick and temperature ?
A
Spinothalamic dysfunction: (pinprick and temperature)
11
Q
DVLA rules for:
• First seizure with no abnormality of ix
- For patients with established epilepsy
• Stroke or TIA
• Multiple TIAs over short period of times
• Craniotomy e.g. For meningioma
• Pituitary tumour: craniotomy:
• trans-sphenoidal surgery ‘can drive when there is no debarring residual impairment likely to affect safe driving’
• Narcolepsy/cataplexy
A
- First seizure: 6 months off driving (if the licence holder has undergone assessment by an appropriate specialist and no relevant abnormality has been identified on investigation, for example EEG and brain scan where indicate). For patients with established epilepsy they must be fit free for 12 months before being able to drive.
- Stroke or TIA: 1 month off driving
- Multiple TIAs over short period of times: 3 months off driving
- Craniotomy e.g. For meningioma: 1 year off driving (With benign tumors and if there is no seizure history, licence can be reconsidered in 6 months if remains seizure free)
- Pituitary tumour: craniotomy: 6 months; trans-sphenoidal surgery ‘can drive when there is no debarring residual impairment likely to affect safe driving’
- Narcolepsy/cataplexy: cease driving on diagnosis, can restart once ‘satisfactory control of symptoms’
12
Q
Syncope and DVLA rule • Simple faint: • Unexplained, low risk of recurrence: • Explained and treated: • Unexplained:
A
Syncope • Simple faint: no restriction • Unexplained, low risk of recurrence: 4 weeks off • Explained and treated: 4 weeks off • Unexplained: 6 months off
13
Q
what is cataplexy?
A
Cataplexy describes the loss of muscular tone caused by strong emotion (e.g. laughter, being frightened).
Around two-thirds of patients with narcolepsy have cataplexy
14
Q
Homonymous hemianopia:
- where is the lesion if the defect is incongruent?
- where is the lesion if the defect in congruent?
- where is the lesion if there is macula sparing?
A
Homonymous Hemianopia
• Incongruous defects = optic tract lesion
• Congruous defects (defect is approximately the same in each eye) :
optic radiation lesion or occipital cortex
• Macula sparing: lesion of occipital cortex
15
Q
Homonymous quadrantanopia:
Where is the lesion if it is a superior quadrantanopia?
Where is the lesion if it is an inferior quadrantanopia?
A
- Superior: lesion of temporal lobe
- Inferior: lesion of parietal lobe
- Mnemonic = PITS (Parietal-Inferior, Temporal-Superior)
16
Q
Bitemporal hemianopia:
- where is the lesion?
- if the lesion is an upper quadrant defect where is the compression and what is this commonly caused by?
- if the lesion is a lower quadrant defect where is the compression and what is this commonly caused by?
A
Lesion of optic chiasm
• Upper quadrant defect > lower quadrant defect = inferior chiasmal
compression, commonly a pituitary tumour
Lower quadrant defect > upper quadrant defect = superior chiasmal compression, commonly a craniopharyngioma
17
Q
what are 4 causes of nystagmus?
A
- Visual disturbances
- Lesions of the labyrinth
- The central vestibular connections
- Brain stem or cerebellar lesions.
18
Q
what does the medial longitudinal bundle do?
A
Medial Longitudinal Bundle → coordinates lateral rectus of one side with medial rectus of the other
19
Q
what causes upbeat vs downbeat nystagmus?
A
Upbeat nystagmus: Cerebellar vermis lesions
Downbeat nystagmus - foramen magnum lesions
-Arnold-Chiari malformation
20
Q
Subdural haemorrhage:
- when is this most commonly seen?
- what is this caused by?
A
- Most commonly secondary to trauma e.g. Old person/alcohol falling over
- Initial injury may be minor and is often forgotten
- Caused by bleeding from damaged bridging veins between cortex and venous sinuses
21
Q
The combination of falls, alcohol excess, fluctuating episodes of confusion and focal neurology points towards a diagnosis of ?hemorrhage. The phrase ‘fluctuating conscious level’ is common in questions and should always bring to mind ?hemorrhage
A
subdural
22
Q
what are three features of subdural haemorrhage?
A
Features
• Headache
• Classically fluctuating conscious level
• Raised ICP
23
Q
what is the treatment for subdural haemorrhage?
A
Treatment
• Needs neurosurgical review
• Burr hole
24
Q
what are 4 causes of subarachnoid haemorrhage?
A
- 85% are due to rupture of berry aneurysms (conditions associated with berry aneurysms include adult polycystic kidney disease, Ehlers-Danlos syndrome and coarctation of the aorta).
- AV malformations.
- Trauma.
- Tumours
25
what are 2 investigations for subarachnoid haemorrhage?
| -if both were negative with a high level of suspicion, what should be done next?
Investigations
• CT: negative in 5%.
• LP: done after 12 Hrs (allowing time for
xanthochromia to develop) If the CSF examination did not reveal xanthochromia, or there was still a high level of clinical suspicion, then cerebral angiography would be the next step
26
what are 3 complications for subarachnoid haemorrhage?
Complications
• Rebleeding (in 30%)
• Obstructive hydrocephalus (due to blood in ventricles)
• Vasospasm leading to cerebral ischemia
27
what is seen on the ECG with subarachnoid haemorrhage?
Intracranial hemorrhage can cause changes in the ECG which are typically deep symmetrical T- wave inversion and prolonged QT interval
28
what is the management of subarachnoid haemorrhage?
* Neurosurgical opinion: no clear evidence over early surgical intervention against delayed intervention
* Nimodipine (e.g. 60mg / 4 hrly, if BP allows) has been shown to ↓ the severity of neurological deficits but doesn't ↓ rebleeding
29
Extradural haemorrhage:
- where is the bleeding?
- what is this most commonly caused by?
- where is this most commonly seen?
Bleeding into the space between the dura mater and the skull.
Often results from acceleration-deceleration trauma or a blow to the side of the head (pterion region).
The majority of epidural Hematomas occur in the temporal region where skull fractures cause a rupture of the middle meningeal artery.
30
what are 2 features of extradural haemorrhage?
Features
• Features of raised intracranial pressure
• Some patients may exhibit a lucid interval
31
Intracranial venous thrombosis:
- what can these cause?
- what do 50% of patients have?
• Can cause cerebral infarction, much less common than arterial causes
• 50% of patients have isolated sagittal sinus thromboses - the remainder have coexistent lateral
sinus thromboses and cavernous sinus thromboses
32
what are three features of intracranial venous thrombosis?
Features
• Headache (may be sudden onset)
• Nausea & vomiting
• Papilledema
33
sagittal sinus thrombosis:
| -how may this present?
Sagittal sinus thrombosis
• May present with seizures and hemiplegia
• Parasagittal biparietal or bifrontal hemorrhagic infarctions are sometimes seen
34
cavernous sinus thrombosis:
| -what are the clinical features?
Cavernous sinus thrombosis
• Other causes of cavernous sinus syndrome: local infection (e.g. Sinusitis), neoplasia, trauma
• Ophthalmoplegia due to IIIrd, IVth and VIth nerve damage
• Trigeminal nerve involvement may lead to hyperaesthesia of upper face and eye pain
• Central retinal vein thrombosis
• Swollen eyelids
35
What cranial nerve palsies are assoc. with lateral sinus thrombosis?
Lateral sinus thrombosis
| • VIth and VIIth cranial nerve palsies
36
what is the first line treatment for delirium if needed?
haloperidol 0.5mg then lorazepam
37
what is Marchiafava Bignami syndrome?
Marchiafava Bignami syndrome: Corpus callosum degeneration from chronic alcohol excess
38
what is the triad of normal pressure hydrocephalus?
A classical triad of features is seen
• Urinary incontinence
• Dementia and bradyphrenia (slowness of thought)
• Gait abnormality (may be similar to parkinson's disease)
39
what is normal pressure hydrocephalus?
- what is this thought to be secondary to?
- what can cause this?
is a reversible cause of dementia seen in elderly patients. It is thought to be secondary to ↓ CSF absorption at the arachnoid villi. These changes may be secondary to head injury, subarachnoid hemorrhage or meningitis
40
what is seen on imaging in normal pressure hydrocephalus?
Imaging
| • Hydrocephalus with an enlarged fourth ventricle
41
what is the management of normal pressure hydrocephalus?
Management
| • Ventriculoperitoneal shunting
42
who does idiopathic intracranial hypertension affect?
Idiopathic Intracranial Hypertension (also known as pseudotumour cerebri and
formerly benign intracranial hypertension) is a condition classically seen in young, overweight ♀.
43
what are the features of idopathic intracranial hypertension?
* Headache
* Blurred vision
* Papilledema (usually present)
* Enlarged blind spot
* Sixth nerve palsy may be present
44
what are 4 risk factors for idiopathic intracranial hypertension?
* Obesity
* ♀sex
* Pregnancy
* Drugs (in this case it is not idiopathic): oral contraceptive pill, steroids, tetracycline, vitamin A
45
what are 3 investigations for idiopathic intracranial hypertension?
1. CT Scan
2. LP
3. Cerebral MRI with MR Venography
46
what is the management for idiopathic intracranial hypertension?
-5 causes
• Weight loss
• Diuretics e.g. Acetazolamide
• Corticosteroids can be given
• Repeated lumbar puncture
• Surgery: optic nerve sheath decompression and fenestration may be needed to prevent damage
to the optic nerve. A lumboperitoneal or ventriculoperitoneal shunt may also be performed to ↓ intracranial pressure
47
describe the ABCD2 score in TIA
| -is this still used?
The ABCD2 prognostic score has previously been used to risk stratify patients who present with a suspected TIA. However, data from studies have suggested it performs poorly and it is therefore no longer recommended by NICE Clinical Knowledge Summaries
Criteria
Points
Age ≥ 60 years - 1
Blood pressure ≥ 140/90 mmHg - 1
Clinical features
- Unilateral weakness - 2
- Speech disturbance, no weakness - 1
Duration of symptoms
- > 60 minutes - 2
- 10-59 minutes - 1
Patient has diabetes - 1
48
what to do in the management of TIA?
Immediate antithrombotic therapy: give aspirin 300 mg immediately, unless
1. the patient has a bleeding disorder or is taking an anticoagulant (needs immediate admission for imaging to exclude a haemorrhage)
2. the patient is already taking low-dose aspirin regularly: continue the current dose of aspirin until reviewed by a specialist
3. Aspirin is contraindicated: discuss management urgently with the specialist team
49
what is the management for patients if the patient has had more than 1 TIA or has a suspected cardioembolic source/ severe carotid stenosis?
If the patient has had more than 1 TIA ('crescendo TIA') or has a suspected cardioembolic source or severe carotid stenosis:
discuss the need for admission or observation urgently with a stroke specialist
50
what is the management if a patient has had a suspected TIA in the last 7 days?
If the patient has had a suspected TIA in the last 7 days:
| arrange urgent assessment (within 24 hours) by a specialist stroke physician
51
what is the management if a patient has had a suspected TIA which occurred more than a week previously?
If the patient has had a suspected TIA which occurred more than a week previously:
refer for specialist assessment as soon as possible within 7 days
52
what drugs are used for antithrombotic therapy in TIA
clopidogrel is recommended first-line (as for patients who've had a stroke)
aspirin + dipyridamole should be given to patients who cannot tolerate clopidogrel
these recommendations follow the 2012 Royal College of Physicians National clinical guideline for stroke.
53
In Acute stroke:
- what should be kept within normal limits?
- what should not be lowered?
- what should be given ASAP after haemorrhagic stroke excluded?
- when should anticoagulants be initiated for atrial fibrillation?
- when should statins be used?
- blood glucose, hydration, oxygen saturation and temperature should be maintained within normal limits
- blood pressure should not be lowered in the acute phase unless there are complications e.g. Hypertensive encephalopathy
- aspirin 300mg orally or rectally should be given as soon as possible if a haemorrhagic stroke has been excluded
- with regards to atrial fibrillation, the RCP state: 'anticoagulants should not be started until brain imaging has excluded haemorrhage, and usually not until 14 days have passed from the onset of an ischaemic stroke'
- if the cholesterol is > 3.5 mmol/l patients should be commenced on a statin. Many physicians will delay treatment until after at least 48 hours due to the risk of haemorrhagic transformation
54
when should thrombolysis be used in stroke?
Thrombolysis with alteplase should only be given if:
it is administered within 4.5 hours of onset of stroke symptoms (unless as part of a clinical trial)
haemorrhage has been definitively excluded (i.e. Imaging has been performed)
55
11 absolute contraindications to thombolysis for stroke?
- Previous intracranial haemorrhage
- Seizure at onset of stroke
- Intracranial neoplasm
- Suspected subarachnoid haemorrhage
- Stroke or traumatic brain injury in preceding 3 months
- Lumbar puncture in preceding 7 days
- Gastrointestinal haemorrhage in preceding 3 weeks
- Active bleeding
- Pregnancy
- Oesophageal varices
- Uncontrolled hypertension >200/120mmHg
56
5 relative contraindications to thrombolysis for stroke?
- Concurrent anticoagulation (INR >1.7)
- Haemorrhagic diathesis
- Active diabetic haemorrhagic retinopathy
- Suspected intracardiac thrombus
- Major surgery / trauma in the preceding 2 weeks
57
when should thrombectomy be considered in stroke?
NICE recommend a pre-stroke functional status of less than 3 on the modified Rankin scale and a score of more than 5 on the National Institutes of Health Stroke Scale (NIHSS)
58
Which patients should be offerred thrombectomy as soon as possible and within 6 hours of symptom onset, together with intravenous thrombolysis (if within 4.5 hours)?
Offer thrombectomy as soon as possible and within 6 hours of symptom onset, together with intravenous thrombolysis (if within 4.5 hours), to people who have:
Acute ischaemic stroke and confirmed occlusion of the proximal anterior circulation demonstrated by computed tomographic angiography (CTA) or magnetic resonance angiography (MRA)
59
which patients should we:
Offer thrombectomy as soon as possible to people who were last known to be well between 6 hours and 24 hours previously (including wake-up strokes) and who...
Offer thrombectomy as soon as possible to people who were last known to be well between 6 hours and 24 hours previously (including wake-up strokes):
confirmed occlusion of the proximal anterior circulation demonstrated by CTA or MRA and if there is the potential to salvage brain tissue, as shown by imaging such as CT perfusion or diffusion-weighted MRI sequences showing limited infarct core volume
60
when should we:
Consider thrombectomy together with intravenous thrombolysis (if within 4.5 hours) as soon as possible for people last known to be well up to 24 hours previously (including wake-up strokes) and who...
Consider thrombectomy together with intravenous thrombolysis (if within 4.5 hours) as soon as possible for people last known to be well up to 24 hours previously (including wake-up strokes):
who have acute ischaemic stroke and confirmed occlusion of the proximal posterior circulation (that is, basilar or posterior cerebral artery) demonstrated by CTA or MRA and
if there is the potential to salvage brain tissue, as shown by imaging such as CT perfusion or diffusion-weighted MRI sequences showing limited infarct core volume
61
What medicines should be used for secondary prevention in stroke?
Recommendations from NICE include:
clopidogrel is now recommended by NICE ahead of combination use of aspirin plus modified-release (MR) dipyridamole in people who have had an ischaemic stroke
aspirin plus MR dipyridamole is now recommended after an ischaemic stroke only if clopidogrel is contraindicated or not tolerated, but treatment is no longer limited to 2 years' duration
MR dipyridamole alone is recommended after an ischaemic stroke only if aspirin or clopidogrel are contraindicated or not tolerated, again with no limit on duration of treatment
62
when should carotid endarterectomy be considered in stroke?
With regards to carotid artery endarterectomy:
recommend if patient has suffered stroke or TIA in the carotid territory and are not severely disabled
should only be considered if carotid stenosis > 70% according ECST** criteria or > 50% according to NASCET*** criteria
63
which cerebral artery has been affected in a stroke with:
• Contralateral hemiparesis and sensory loss, lower extremity > upper
• Disconnection syndrome (akinetic mute patient)
Anterior cerebral artery
64
which cerebral artery has been affected in a stroke with:
• Contralateral hemiparesis and sensory loss, upper extremity > lower
• Contralateral hemianopia
• Aphasia (Wernicke's)
• Gaze abnormalities
Middle cerebral artery
65
what cerebral artery has been affected in a patient with:
• Contralateral hemianopia with macular sparing
• Disconnection syndrome
posterior cerebral artery
66
How does a lacunar stroke present?
Lacunar
| • Present with either isolated hemiparesis, hemisensory loss or hemiparesis with limb ataxia
67
Describe lateral medullary syndrome
Lateral medulla (posterior inferior cerebellar artery- PICA)
• Ipsilateral: ataxia, nystagmus, dysphagia, facial numbness, cranial nerve palsy
• Contralateral: limb sensory loss
68
what is seen in a pontine stroke?
Pontine
• VI nerve: horizontal gaze palsy
• VII nerve
• Contralateral hemiparesis
69
basilar artery CVA:
- prognosis?
- how do these patients present?
- what visual sx?
- what syndrome can patients have?
- BP?
- what does management involve?
Basilar Artery CVA is typically associated with a poor prognosis
• Most of patients present with nausea, vertigo and vomiting
• Some present with motor deficits, dysarthria and speech involvement or headaches
• May present with visual disturbances. This includes abducens nerve palsy, conjugate gaze palsy,
internuclear ophthalmoplegia and ocular bobbing
• Locked-In Syndrome: patient is awake but is unable to respond in anyway except by vertical gaze and blinking (lesion is in ventral pons)
• 70% of patients presenting with basilar artery territory stroke are hypertensive.
• Management includes vigorous control of hypertension and antiplatelet agents.
70
what is LACS?
```
LACS (lacunar stroke):
Pure motor or pure sensory syndrome or
Ataxic hemiparesis or
Dysarthria or
Clumsy-hand syndrome.
```
71
what is PACS?
```
PACS (partial anterior circulatory stroke): 2 of the 3 elements below, or new higher cerebral
dysfunction.
Higher cerebral dysfunction
Contralateral sensory/motor deficit
Visual field defect.
```
72
what is TACS?
TACS (total anterior circulatory stroke): combination of the 3 elements
Higher cerebral dysfunction
Contralateral sensory/motor deficit
Visual field defect.
73
what is POCS?
```
POCS (posterior circulation syndrome):
Bilateral motor/sensory deficit or
Disorder of conjugate eye movement, or
Solitary visual field defect, or
Cerebellar dysfunction, or
Crossed cranial nerve and sensory/motor signs.
```
74
lateral medullary syndrome:
- what does this arise as a cause of?
- what are the cerebellar features?
- what are the brainstem features?
Lateral Medullary Syndrome: also known as Wallenberg's syndrome, occurs following occlusion of the posterior inferior cerebellar artery, resulting in sensory and sympathetic disturbances
Cerebellar features
• Ataxia
• Nystagmus
Brainstem features
• Ipsilateral: dysphagia, facial numbness, cranial nerve palsy e.g. Horner's
• Contralateral: limb sensory loss (pyramidal tract signs)
75
what is pituitary apoplexy?
Pituitary Apoplexy: sudden enlargement of pituitary tumour secondary to hemorrhage or infarction
76
what are the features of pituitary apoplexy?
Features
• Sudden onset headache similar to that seen in subarachnoid hemorrhage
• Vomiting
• Neck stiffness
• Visual field defects: classically bitemporal superior quadrantic defect
• Extraocular nerve palsies
• Features of pituitary insufficiency e.g. Hypotension secondary to hypoadrenalism
• Electrolytes disturbance.
77
what is the management of pituitary apoplexy?
Management: IV Hydrocortisone should be given to prevent adisonian crisis
78
what is brown-sequard syndrome?
| -what is this caused by?
Brown-Séquard syndrome: is a loss of sensation and motor function (paralysis and ataxia) that is caused by the lateral hemisection of the spinal cord.
79
describe the features of brown sequard?
- loss of fine touch/vibration/proprioception on which side?
- hyper-reflexia and extensor plantar reflex on which side?
- loss of temperature and pain on which side?
- what other signs?
- what are the causes for this?
• Ipsilateral loss of fine touch, vibration and proprioception
• Ipsilateral hyper-reflexia and extensor plantar reflex
• Contralateral loss of pain and temperature sensation occurs affecting the side.
• Segmental anaesthesia at the level of the lesion
• Complete syndrome picture is rare and many patients may only exhibit some features.
• Trauma is a common cause; demyelination due to multiple sclerosis is another common cause
but any lateral cord lesion (ischaemia, hemorrhage, granuloma, tumour etc) may give this picture.
80
what is a syringomyelia?
| -when do symptoms begin?
Syringomyelia is a developmental, slowly enlarging cavitaryexpansion of the cervical cord that produce progressive myelopathy. Symptoms begin insidiously in adolescence or early adulthood, progress irregularly, and may undergo spontaneous arrest for several years; most patients acquire a cervical-thoracic scoliosis.
* Development of cavity (syrinx) within the spinal cord
* If extends into medulla then termed syringobulbia
* Strongly associated (>50%) with the arnold-chiari malformation
81
what is the investigation used for syringomelia?
MRI is the investigation of choice
| Myelography used to confirm the diagnosis but was associated with more deterioration
82
what is transverse myelitis?
| -what signs are seen?
Transverse Myelitis: is an inflammatory lesion that can affect the cord. Constitutional symptoms such as headache and fever are common as is pain. Signs are indistinguishable from those caused by cord compression and again all sensory aspects are equally affected with no sparing of proprioception.
83
what is subclavian steal syndrome?
| -what are neurological symptoms precipitated by?
Subclavian Steal Syndrome: is associated with retrograde flow in the vertebral artery due to proximal subclavian artery stenosis. Neurological symptoms are precipitated by vigorous exercise with the arm above the head, such as painting a wall.
84
Subclavian steal syndrome:
- what is diagnosis often confused with?
- what is the investigation of choice?
- what are the treatment option?
• Diagnosis is often confused with transient ischemic attacks or epilepsy.
• Duplex ultrasound and MRA are the investigations of choice.
• Endarterectomy and stenting are common surgical methods involved in relieving symptoms
associated with this condition.
85
what is subacute combined degeneration of the spinal cord?
Subacute Combined Degeneration of Spinal Cord, also known as Lichtheim's disease, refers to degeneration of the posterior and lateral columns of the spinal cord as a result of vitamin B12 deficiency (most common), vitamin E deficiency or Friedrich's ataxia. It is usually associated with pernicious anemia.
86
what are the features of subacute degeneration of the cord:
- limb signs?
- feet?
- what is it caused by?
- what is the treatment?
Features:
• Patchy losses of myelin in the dorsal and lateral columns.
• Present with progressive weakness of legs, arms, trunk,
tingling and numbness.
• Visual & Mental changes may also be present.
• Bilateral spastic paresis may develop and pressure, vibration
and touch sense are diminished.
• Positive Babinski sign may be seen.
• Prolonged deficiency (> 3 months) of vitamin B12 leads to
irreversible nervous system damage.
• If someone is deficient in vitamin B12 and folic acid, the vitamin B12 deficiency must be treated first to avoid
precipitating subacute combined degeneration of the cord.
• Therapy with vitamin B12 results in partial to full recovery,depending on the duration and extent of neurodegeneration
87
what is narcolepsy?
| -what are the symptoms?
Narcolepsy is a condition causing excessive daytime somnolence and an overwhelming desire to sleep. Symptoms include excessive daytime sleepiness (EDS), involuntary sleep episodes –microsleeps- cataplexy (70%), sleep paralysis hallucinations - hypnagogic (at the onset of sleep) and hypnopompic (on awakening).
88
what is von hippel lindau syndrome?
- how is this inherited?
- what chromosome is assoc. with this?
- what are key features?
Von Hippel-Lindau (VHL) syndrome is an autosomal dominant condition predisposing to neoplasia. It is due to an abnormality in the VHL gene located on short arm of chromosome 3
Retinal and cerebellar hemangiomas are key features of Von Hippel-Lindau syndrome. Retinal hemangiomas are bilateral in 25% of patients and may lead to vitreous hemorrhage
89
what are the features of von hippel lindau syndrome?
• Cerebellar hemangiomas (Can secret crythyropiotiene that causes secondary polycythemia)
• Retinal hemangioma: vitreous hemorrhage
• Renal cysts (premalignant)
• Pheochromocytoma
• Extra-renal cysts: epididymal, pancreatic,
hepatic
• Endolymphatic sac tumours
90
what is friedreich's ataxia?
- how is this inherited?
- what gene and chromosome are assoc?
- what is the typical age of onset?
Friedreich's Ataxia is the most common of the early-onset hereditary ataxias. It is an autosomal recessive, trinucleotide repeat disorder characterized by a GAA repeat in the X25 gene on chromosome 9 (frataxin). Friedreich's ataxia is unusual amongst trinucleotide repeat disorders in not demonstrating the phenomenon of anticipation. The typical age of onset is 10-15 years old
91
what are the neurological features of friedreichs ataxia?
```
Neurological features
• Absent ankle jerks/extensor plantars
• Cerebellar ataxia
• Optic atrophy
• Spinocerebellar tract degeneration
```
92
what are the non-neurological features of friedreichs ataxia?
Other features
• Hypertrophic obstructive cardiomyopathy (90%, most common cause of death)
• Diabetes mellitus (10-20%)
• High-arched palate
93
what is tuberous sclerosis?
- how is this inherited?
- where are the majority of features seen?
Tuberous Sclerosis (TS) is a genetic condition of autosomal dominant inheritance. Like neurofibromatosis, the majority of features seen in TS are neuro-cutaneous
94
what are the cutaneous features of tuberous sclerosis?
Cutaneous features
• Depigmented 'ash-leaf' spots which fluoresce under UV light
• Roughened patches of skin over lumbar spine (Shagreen patches)
• Adenoma sebaceum: butterfly distribution over nose
• Fibromata beneath nails (subungual fibromata)
• Café-au-lait spots may be seen
95
what are the neurological features of tuberous sclerosis?
Neurological features
• Developmental delay
• Epilepsy (infantile spasms or partial)
• Intellectual impairment
96
what are the non-cutaneous and non-neuro features of tuberous sclerosis?
Also
• Retinal hamartomas: dense white areas on retina (phakomata)
• Rhabdomyomas of the heart
• Gliomatous changes can occur in the brain lesions
• Polycystic kidneys, renal angiomyolipomata
97
neurofibromatosis 1 and 2:
- how are these inherited?
- chromosomes?
Neurofibromatosis: both types are inherited in an autosomal dominant fashion. NF1 is also known as von Recklinghausen's syndrome. It is caused by a gene mutation on chromosome 17 which encodes neurofibromin and affects around 1 in 4,000. NF2 is caused by gene mutation on chromosome 22 and affects around 1 in 100,000
98
what are the features of neurofibromatosis type 1?
Café-au-lait spots (= 6, 15 mm in diameter) Axillary/groin freckles
Peripheral neurofibromas
Iris: Lisch nodules in > 90%
Scoliosis
99
what are the features of neurofibromatosis type 2?
Bilateral acoustic neuromas
100
what is hereditary sensorimotor neuropathy?
- how many types exist and how many of these are common to clinical practice?
- what are these due to?
Hereditary Sensorimotor Neuropathy (HSMN) is a relatively new term which encompasses Charcot-Marie-Tooth disease (also known as peroneal muscular atrophy). Over 7 types have been characterized - however only 2 are common to clinical practice
• HSMN type I: primarily due to demyelinating pathology
• HSMN type II: primarily due to axonal pathology
101
HSMN type 1:
- how is this inherited?
- what is this due to?
- when do features start?
- what are features?
- what happens to nerve conduction velocity?
HSMN type I
• Autosomal dominant
• Due to defect in PMP-22 gene (which codes for myelin) (PMP = Peripheral Myelin Protein)
• Features often start at puberty
• Motor symptoms predominate
• Distal muscle wasting, pes cavus, clawed toes
• Foot drop, leg weakness often first features
• Nerve Conduction Velocity is greatly ↓ <30m/second
102
what is motor neurone disease?
- what age does it usually present after?
- what are 4 patterns of disease?
Motor Neuron Disease is a neurological condition of unknown cause which can present with both upper and lower motor neuron signs. It rarely presents before 40 years and various patterns of disease are recognized including amyotrophic lateral sclerosis, primary lateral sclerosis, progressive muscular atrophy and bulbar palsy. In some patients however, there is a combination of clinical patterns
103
Amyotrophic lateral sclerosis (50% of patients)
- what are the signs?
- what gene is responsible in familial cases?
- what does this code for?
• Typically LMN signs in arms and UMN signs in legs
• In familial cases the gene responsible lies on chromosome 21 and codes for superoxide
dismutase
104
what signs are seen in primary lateral sclerosis?
Primary lateral sclerosis
| • UMN signs only
105
Progressive muscular atrophy:
- what are the signs?
- what muscles are affected?
- what is the prognosis?
Progressive muscular atrophy
• LMN signs only
• Affects distal muscles before proximal
• Carries best prognosis
106
Bulbar palsy:
- what are the signs?
- what is the prognosis?
Bulbar palsy
• Palsy of the tongue, muscles of chewing/swallowing and facial muscles due to loss of function of brainstem motor nuclei
• Carries worst prognosis
107
• Fasciculation
• Absence of sensory signs/symptoms (vague sensory symptoms may occur early in the disease
(e.g. Limb pain) but 'never' sensory signs)
• Lower motor neuron signs in arms and upper motor neuron signs in legs
• Wasting of the small hand muscles/tibialis anterior is common (tibialis anterior functions to
stabilise the ankle as the foot hits the ground during the contact phase of walking [eccentric contraction] and acts later to pull the foot clear of the ground during the swing phase [concentric contraction]. It also functions to 'lock' the ankle, as in toe-kicking a ball, when held in an isometric contraction)
-what should all of these prompt thought of?
motor neurone disease
108
Motor neurone disease:
- does this affect external ocular muscles?
- does this have cerebellar signs?
- abdominal reflexes?
- sphincter dysfunction?
* Doesn't affect external ocular muscles
* No cerebellar signs
* Abdominal reflexes are usually preserved and sphincter dysfunction if present is a late feature.
109
what is the diagnosis of motor neurone disease?
The diagnosis of motor neuron disease is clinical, but nerve conduction studies will show normal motor conduction and can help exclude a neuropathy. Electromyography shows a ↓ number of action potentials with ↑ amplitude. MRI is usually performed to exclude the differential diagnosis of cervical cord compression and myelopathy
110
what is the treatement and prognosis for motor neurone disease?
```
Riluzole
• Anti-glutamate drug
• Used mainly in amyotrophic lateral sclerosis
• Prolongs life by about 3 months
• Expensive
```
Respiratory care
• Non-invasive ventilation (usually BiPAP) is used at night
• Studies have shown a survival benefit of around 7 months
Prognosis
• Poor: 50% of patients die within 3 years
111
what is the age distribution of the presentation of epilepsy?
Epilepsy is more likely to occur in young children or people over the age of 65 years; however it can occur at any time.
112
Epilepsy
| what are the two main categories?
Basics
• Two main categories: generalised and partial seizures
• Partial seizures may progress to general seizures
• Other types: myoclonic, atypical absence, atonic and tonic seizures are usually seen in
childhood
113
describe the features of generalised seizures?
- no focal features, consciousness lost immediately
• Grand mal (tonic-clonic)
• Petit mal (absence seizures)
• Partial seizures progressing to generalised seizures
114
describe the features of partial seizures
| -in patients who have automatisms, which part of the brain has the seizure activity most commonly?
- focal features depending on location
• Simple (no disturbance of consciousness or awareness)
• Complex (consciousness is disturbed)
• Temporal lobe → aura, déjà vu, jamais vu; motor → jacksonian
Complex partial seizures can take the form of
automatisms, such as chewing and swallowing, repeatedly scratching the head or searching for an object. Some people may even undress. They can occur as a result of seizure activity in any part of the brain but most commonly arise in the temporal lobes
115
what is the treatment for generalised tonic-clonic seizures?
Generalised tonic-clonic seizures
sodium valproate
second line: lamotrigine, carbamazepine
116
what is the treatment for absence seizures? what may exacerbate these?
Absence seizures (Petit mal)
sodium valproate or ethosuximide
sodium valproate particularly effective if co-existent tonic-clonic seizures in primary generalised epilepsy
carbamazepine may exacerbate
117
what is the treatment for myoclonic seizures - what may exacerbate myoclonic seizures?
Myoclonic seizures
sodium valproate
second line: clonazepam, lamotrigine
carbamazepine may exacerbate these
118
why should caution be exercised when combining sodium valproate with lamotrigine?
Caution should be exercised when combining sodium valproate and lamotrigine as serious skin rashes such as Steven- Johnson's syndrome may be provoked.
119
what is a side affect of vigabatrin?
Vigabatrin – rarley used anti-epileptic
• 40% of patients develop Visual field defects, which may be irreversible
• Visual fields should be checked every 6 months
120
who do absence seizures affect?
Absence Seizures (Petit Mal) are a form of generalised epilepsy that is mostly seen in children. The typical age of onset of 3-10 years old and girls are affected twice as commonly as boys
121
Describe absence seizures:
- how long do they last?
- what may provoke the seizure?
- when do they occur?
- is there awareness?
- what is seen on EEG?
Features
• Absences last a few seconds and are associated with a quick recovery
• Seizures may be provoked by hyperventilation or stress
• The child is usually unaware of the seizure
• They may occur many times a day
• EEG: bilateral, symmetrical 3Hz (spike and wave) pattern
122
describe the management of partial seizures
Management
• Sodium valproate and ethosuximide are first-line treatment
• Good prognosis - 90-95% become seizure free in adolescence
123
Describe the management of focal seizures?
Focal seizures
carbamazepine or lamotrigine
second line: levetiracetam, oxcarbazepine or sodium valproate
124
when can anti-epileptic drugs be stopped? how does this happen?
AED cessation can be considered if seizure free for > 2 years – Stop AEDs over 2-3 months
125
In a patient who has been seizures free for 2 years what is the chance of recurrence and what increases this risk?
Therapy stopped: chance of recurrence in the next 2 years is 43%
Therapy cont.: 10%
Factors that have been shown to increase the risk of seizures include:
• Older age
• Use of multiple anticonvulsants
• History of myoclonic or tonic clonic seizure
• Previous abnormal imaging or EEG
• Seizure while on therapy.
126
Epilepsy in pregnancy:
- what should all women take who are thinking about pregnancy?
- what is the risk of congenital defect?
The risks of uncontrolled epilepsy during pregnancy generally outweigh the risks of medication to the fetus. All women thinking about becoming pregnant should be advised to take folic acid 5mg/day well before pregnancy to minimise the risk of neural tube defects. Around 1-2% of newborns born to non- epileptic mothers have congenital defects. This rises to 3-4% if the mother takes antiepileptic medication
127
In pregnancy:
- how many drugs should be aimed for to control
- how often should antiepileptic drug levels be checked?
- which drug is assoc. with neural tube defects?
- which drug is assoc. with cleft palate?
- which drug may need to be increased in pregnancy?
- which drug is considered the least teratogenic?
• Aim for monotherapy
• There is no indication to monitor antiepileptic drug levels
• Sodium valproate: associated with neural tube defects
• Phenytoin (Epanutin®): associated with cleft palate
• Lamotrigine: studies to date suggest the rate of congenital malformations may be low. The dose
of lamotrigine may need to be increased in pregnancy
• Carbamazepine: often considered the least teratogenic of the older antiepileptics
128
are antiepileptic drugs considered safe in breast feeding?
Breast feeding is generally considered safe for mothers taking antiepileptics with the possible exception of the barbiturates
129
what should pregnant women on phenytoin be advised to take in the last month of pregnancy to prevent clotting disorders in the newborn?
It is advised that pregnant women taking phenytoin are given vitamin K in the last month of pregnancy to prevent clotting disorders in the newborn
130
Infantile spasm AKA West syndrome
- when does this occur?
- what is this managed by?
- what is the triad?
* Occurs between 3-12 month of age, managed by Vegabatrin (S.E causes alopecia, ↓ visual acuity diplopia).
* West syndrome is the triad of infantile spasms, a pathognomonic EEG pattern (called hypsarrhythmia), and mental retardation - although the international definition requires only two out of these three elements.
131
what are non-epileptic attacks broadly divided into?
Non-epileptic attacks are broadly divided into:
• Hyperkinetic/thrashing attacks
• Akinetic/motionless attacks.
132
what should:
Asynchronous limb movements
• Undulating motor activity
• Purposeful movements
• Rhythmic pelvic movements
• Side-to-side head shaking
• Biting the tip of the tongue (as opposed to the side)
• Ictal crying
• Vocalisation during the ‘tonic–clonic’ phase
• Closed eyelids, resistance to eyelid opening
• Lack of cyanosis and rapid post-ictal reorientation
All point towards
pseudoseizure
133
what is the gold standard test for non-epileptic seizures?
Video telemetry
Prolactin is often elevated 15–20 min after a tonic–clonic seizure and should be normal in non-epileptic attacks, but there are a number of problems: serum rises are seen in syncopal episodes; it may be normal, especially in partial epileptic seizures; and the test is often badly carried out in practice (too early or too late).
134
what is parkinson's disease caused by?
Parkinson's Disease is a progressive neurodegenerative condition caused by degeneration of dopaminergic neurons in the substantia nigra
135
what is the triad of Parkinson's disease?
| -is it symmetrical/asymetrical?
bradykinesia, asymetrical tremor and rigidity. The symptoms of Parkinson's disease are characteristically asymmetrical
136
describe the bradykinesia in Parkinson's disease
Bradykinesia
• Poverty of movement also seen: mask-like face
• Difficulty in initiating movement
137
describe the tremor in Parkinson's Disease
Tremor
• Most marked at rest, 3-5 Hz
• Typically 'pill-rolling'
138
describe the rigidity in Parkinson's Disease
Rigidity
• Lead pipe
• Cogwheel: due to superimposed
tremor
139
describe the other symptoms other than the triad of bradykinesia, tremor and rigidity in PD?
```
• Flexed posture
• Short, shuffling steps
• Micrographia
• Drooling of saliva
• Psychiatric features: depression is the most common feature (affects about 40%); dementia,
psychosis and sleep disturbances may also occur
• Impaired olfaction
• REM sleep behaviour disorder
```
140
* Parkinson's disease
* Drug-induced e.g. Antipsychotics, metoclopramide - see below
* Progressive supranuclear palsy
* Multiple system atrophy
* Wilson's disease
* Post-encephalitis
* Dementia pugilistica (secondary to chronic head trauma e.g. Boxing)
* Toxins: carbon monoxide, MPTP
are all causes of what?
Parkinsonion syndrome?
141
describe the different features that drug-induced parkinsonism has from Parkinson's disease
Drug-induced Parkinsonism has slightly different features to Parkinson's disease:
• Motor symptoms are generally rapid onset and bilateral
• Rigidity and rest tremor are uncommon
142
which three types of drugs can cause parkinson's?
Drugs causing Parkinsonism
• Phenothiazines: e.g. Chlorpromazine
• Butyrophenones: haloperidol, droperidol
• Metoclopramide
143
does Domperidone cause extra-pyramidal side-effects?
Domperidone does not cross the blood-brain barrier and therefore does not cause extra-pyramidal side-effects
144
when is treatment started for parkinson's disease?
Currently accepted practice in the management of patients with Parkinson's disease (PD) is to delay treatment until the onset of disabling symptoms
145
what is the first line treatment for PD?
For first-line treatment:
if the motor symptoms are affecting the patient's quality of life: levodopa
if the motor symptoms are not affecting the patient's quality of life: dopamine agonist (non-ergot derived), levodopa or monoamine oxidase B (MAO‑B) inhibitor
146
what are the 5 drugs types used in Parkinson's disease and how do they work?
- Dopamine receptor agonists
- Levodopa
- MAO-B inhibitor
- Amantadine
- COMT inhibitors
- Antimuscarinics
Dopamine receptor agonists (favored for patients > 75yrs old)
• E.g. Bromocriptine, Pramipexole, Ropinirole, Cabergoline, Apomorphine
• Ergot-derived dopamine receptor agonists (bromocriptine, cabergoline) have been
associated with pulmonary, retroperitoneal and cardiac fibrosis. The Committee on Safety of Medicines advice that an ESR, creatinine and CXR should be obtained prior to treatment and patients should be closely monitored
• Ropinirole is least associated with tissue fibrosis.
• Patients should be warned about the potential for dopamine receptor agonists to cause impulse
control disorders and excessive daytime somnolence
Levodopa
• Usually combined with a decarboxylase
inhibitor (e.g. Carbidopa or benserazide) to prevent peripheral metabolism of levodopa to dopamine
• ↓ effectiveness with time (usually by 2 years)
• Unwanted effects: dyskinesia, 'on-off' effect
• Not used in neuroleptic induced parkinsonism
• Favoed for patients < 75yrs
MAO-B (Monoamine Oxidase-B) inhibitors
• E.g. Selegiline
• Inhibits the breakdown of dopamine secreted by the dopaminergic neurons
Amantadine
• Mechanism is not fully understood, probably ↑ dopamine release and inhibits its uptake at
dopaminergic synapses
COMT (Catechol-O-Methyl Transferase) inhibitors
• E.g. Entacapone
• COMT is an enzyme involved in the breakdown of dopamine, and hence may be used as an
adjunct to levodopa therapy
• Used in established PD
Antimuscarinics
• Block cholinergic receptors
• Now used more to treat drug-induced parkinsonism rather than idiopathic Parkinson's disease
• Help tremor and rigidity
• E.g. procyclidine, benzotropine, trihexyphenidyl (benzhexol)
147
```
For Levodopa:
Motor symptoms
ADLs
Motor Complications
Adverse events (excessive sleepiness, hallucinations and impulse control disorders)
```
Adverse effects?
More improvement in motor symptoms
More improvement in ADLs
More motor complications
Fewer specified adverse events
```
Adverse effects of L-Dopa • Dyskinesia
• 'On-off' effect
• Postural hypotension
• Cardiac arrhythmias
• Nausea & vomiting
• Psychosis
• Reddish discolouration of urine upon standing
```
148
```
For Dopamine agonists:
Motor symptoms
ADLs
Motor Complications
Adverse events (excessive sleepiness, hallucinations and impulse control disorders)
off time
risk of hallucinations
```
```
Less improvement in motor symptoms
Less improvement in activities of daily living
Fewer motor complications
More specified adverse events
More off‑time reduction
More risk of hallucinations
```
149
```
For MOA-B inhibitors:
Motor symptoms
ADLs
Motor Complications
Off-time
Adverse events (excessive sleepiness, hallucinations and impulse control disorders)
risk of hallucinations
```
```
Less improvement in motor symptoms
Less improvement in activities of daily living
Fewer motor complications
Fewer specified adverse events
Off‑time reduction
Fewer adverse events
Lower risk of hallucinations
```
150
```
For COMT inhibitors:
Motor symptoms
ADLs
Off-time
Adverse events (excessive sleepiness, hallucinations and impulse control disorders)
risk of hallucinations
```
```
Improvement in motor symptoms
Improvement in activities of daily living
Off‑time reduction
More adverse events
Lower risk of hallucinations
```
151
```
For Amantadine:
Motor symptoms
ADLs
Off-time
Adverse events (excessive sleepiness, hallucinations and impulse control disorders)
risk of hallucinations
```
No evidence of improvement in motor symptoms
No studies for:
Off-time
Adverse events (excessive sleepiness, hallucinations and impulse control disorders)
risk of hallucinations
152
what can be used for drooling of saliva in PD?
Consider glycopyrronium bromide to manage drooling of saliva in people with Parkinson's disease.
153
what can be used for excessive daytime sleepiness in PD?
If excessive daytime sleepiness develops then patients should not drive. Medication should be adjusted to control symptoms. Modafinil can be considered if alternative strategies fail.
154
what can be done for orthostatic hypotension in PD?
If orthostatic hypotension develops then a medication review looking at potential causes should be done. If symptoms persist then midodrine (acts on peripheral alpha-adrenergic receptors to increase arterial resistance) can be considered.
155
what syndrome is a type of multi-system atrophy?
| -what are the three features?
Multiple system atrophy: Shy-Drager syndrome is a type of multiple system atrophy
Features
• Parkinsonism
• Autonomic disturbance (atonic bladder, postural hypotension)
• Cerebellar signs
156
what is supranuclear palsy?
-males or females are worse affected?
what is this AKA?
Progressive Supranuclear Palsy is degenerative disease involving the gradual deterioration and death of selected areas of the brain. Both ♂ and ♀ are affected approximately equally and there is no racial, geographical or occupational predilection. Approximately 6 people per 100,000.
* AKA Steele-Richardson-Olszewski syndrome
* A 'Parkinson Plus' syndrome
157
what are the features of supranuclear palsy?
```
Features
• Impairment of vertical gaze (down gaze worse than up gaze - patients may complain of difficultly reading or descending stairs)
• Parkinsonism
• Falls
• Slurring of speech
• Cognitive impairment
```
158
what is the response to L-Dopa in supranuclear palsy?
Management
| • Poor response to L-dopa
159
what is the characteristic pathological feature in lewy body dementia?
Lewy Body Dementia is an increasingly recognised cause of dementia, accounting for up to 20% of cases. The characteristic pathological feature is cytoplasmic neuronal inclusions (Lewy bodies) in the substantia nigra, paralimbic and neocortical areas
160
what drugs should be avoided in lewy body dementia?
Neuroleptics should be avoided in Lewy body dementia as patients are extremely sensitive and may develop irreversible Parkinsonism. Questions may give a history of a patient who has deteriorated following the introduction of an antipsychotic agent
161
what are the features of lewy body dementia?
```
Features
• Progressive cognitive impairment
• Parkinsonism
• Visual hallucinations (other features such as delusions and non-visual hallucinations may also
be seen)
```
162
what is essential tremor?
Essential tremor: (previously called benign essential tremor) is an autosomal dominant condition which usually affects both upper limbs.
163
what are the features of essential tremor?
Features
• Postural tremor: worse if arms outstretched (usually 6-8 Hz)
• Improved by alcohol and rest
• Most common cause of titubation (head tremor)
164
what is the management of essential tremor?
Management
• Propranolol is first-line
• Primidone (anticonvulsant ) is sometimes used when propanolol is contraindicated
165
CJD:
- what is the course of disease
- what are the clinical features?
Creutzfeldt - Jakob disease (CJD): Basics & Features:
• Rapidly progressive, severe and invariably fatal (usually within few months)
• Dementia
• Cerebellar ataxia
• Defuse myoclonic jerks
• Other neurological abnormalities.
Myoclonus is typical and progressive, even during the later stage when the patient is stuporous or comatose.
166
what investigations are used in CJD?
Investigation:
• The EEG patern is characteristic (diffuse non specific slowing periodic sharp wave complexes- PSWCs of 1 – 2 Hz), but diagnosis relies on either espiecalized tests for prion protein in CSF or direct brain biopsy.
• Pulvinar Sign on cranial MRI > 90% pathological in vCJD (Not Sporadic)
• Prion protien in tonsils
• 14-3-3 protien in CSF
167
in sporadic CJD:
- how old are people affected?
- what are early features vs later features?
- what is the course of disease?
Sporadic CJD predominantly affects late mid-aged individuals with mean age of death in the late 60s. Memory impairment and cerebellar ataxia are common early features, subsequently, rapidly progressive dementia and myoclonus. The median duration of illness is 4 months and about 65% of caeses die within 6 months.
168
in Alzheimers disease:
- are most cases sporadic or inherited?
- if it is inherited, how is this inherited? and what mutations are involved?
Genetics
• Most cases are sporadic
• 5% are inherited as an autosomal dominant trait
• Mutations in the amyloid precursor protein (chromosome 21), presenilin 1 (chromosome 14)
and presenilin 2 (chromosome 1) genes are thought to cause the inherited form
• Apoprotein E allele E4 - encodes a cholesterol transport protein
169
in alzheimers disease:
- what macroscopic pathological changes are seen?
- what microscopic pathological changes are seen?
- what biochemical pathological changes are seen?
Pathological changes
• Macroscopic = widespread cerebral atrophy, particularly involving the cortex and hippocampus
• Microscopic = intraneuronal neurofibrillary tangles, neuronal plaques, deficiency of neurons
• Biochemical = deposition of type A-β-amyloid protein in cortex, deficit of Ach from damage to
an ascending forebrain projection
170
what is a neurofibrillary tangle?
Neurofibrillary tangles
• Paired helical filaments are partly made from a protein called tau
• In Alzheimer: tau proteins are excessively phosphorylated
171
what is the management for alzheimers disease?
Management
• NICE now recommend the three acetylcholinesterase inhibitors (donepezil, galantamine and rivastigmine) as options for managing mild to moderate Alzheimer’s disease. Donepezil may cause bradycardia and atrioventricular node block.
• Memantine is reserved for patients with moderate - severe Alzheimer’s.
172
when does hemiballism occur?
Hemiballism occurs following damage to the subthalamic nucleus.
173
what are the features of hemiballism?
Features - Ballisic movements:
• Involuntary, sudden, jerking movements which occur contralateral to the side of the lesion
• Primarily affect the proximal limb musculature whilst the distal muscles may display more
choreiform-like movements.
• Symptoms may decrease during sleeping.
174
what is the cause of hemiballism?
• Etiology: stroke in elders, infection or inflammatory in young.
175
what is the treatment for hemiballism?
• Antidopaminergic agents (e.g. Haloperidol) are the mainstay of treatment. Tetrabenazine may
be considered when long-term therapy is required.
176
```
• Huntington's disease, Wilson's disease, ataxic telangiectasia
• SLE, anti-phospholipid syndrome
• Rheumatic fever: Sydenham's chorea
• Drugs: oral contraceptive pill, L-dopa,
antipsychotics
• Chorea gravidarum
• Thyrotoxicosis
• Polycythemia rubra vera
• Carbon monoxide poisoning
• Neuroacanthocytosis
• Cerebrovascular disease
```
are all causes of?
chorea
177
what does the facial nerve supply?
Supply - 'face, ear, taste, and tear'
• Face: muscles of facial expression
• Ear: nerve to stapedius
• Taste: supplies anterior two-thirds of tongue
• Tear: parasympathetic fibres to lacrimal glands, also salivary glands
178
if there is a facial palsy and squint - where is the lesion?
Facial Palsy + convergent squint - lesion is in Pons as VIth is encircled by VIIth
179
what are 3 causes of bilateral facial nerve palsy?
Causes of bilateral facial nerve palsy
• Sarcoidosis
• Guillain-Barre syndrome
• Polio, Lyme disease
180
name some lower motor neurone causes of facial nerve palsy?
| -will this spare the forehead
* Bell's palsy
* Ramsay-Hunt syndrome (due to herpes zoster)
* Acoustic neuroma
* Parotid tumours
* HIV
* Multiple Sclerosis
* Diabetes Mellitus
NO
181
what is a upper motor neurone cause of facial palsy?
| -will this spare the forehead?
stroke
| YES
182
what is Bell's Palsy?
Bell's palsy may be defined as an acute, unilateral, idiopathic, facial nerve paralysis. The aetiology is unknown although the role of the herpes simplex virus has been investigated previously.
183
Describe the features of Bell's Palsy
Features
• Lower motor neuron facial nerve palsy - forehead affected
• Patients may also notice post-auricular pain (may precede paralysis), altered taste, dry eyes
184
Describe the management of Bell's Palsy
Management
• In the past a variety of treatment options have been proposed including no treatment, prednisolone only and a combination of aciclovir and prednisolone
• Following a National Institute for Health randomised controlled trial it is now recommended that prednisolone 25mg BD for 10 days should be prescribed for patients within 72 hours of onset of Bell’s palsy. Adding in aciclovir gives no additional benefit
• Eye care is important - prescription of artificial tears and eye lubricants should be considered
185
what is the prognosis of Bell's Palsy?
Prognosis
| • If untreated around 15% of patients have permanent moderate to severe weakness
186
what is MS?
- what is this caused by?
- when is disease onset?
- males or females?
Multiple Sclerosis: (it is an UML) also known as disseminated sclerosis or encephalomyelitis disseminate, is an autoimmune disease in which the body's immune response attacks a person's central nervous system (brain and spinal cord), leading to demyelination. Disease onset usually occurs in young adults, and it is more common in ♀.
187
what is the course of relapsing-remitting MS?
| -is this common?
Relapsing-remitting: Characterized by unpredictable relapses followed by periods of months to years of relative quiet (remission) with no new signs of disease activity. Deficits suffered during attacks may either resolve or leave sequelae. This describes the initial course of 85–90% of individuals with MS. When deficits always resolve between attacks, this is sometimes referred to as benign MS.
188
Describe secondary progressive MS?
Secondary progressive: Describes those with initial relapsing-remitting MS, who then begin to have progressive neurologic decline between acute attacks without any definite periods of remission. Occasional relapses and minor remissions may appear. The median time between disease onset and conversion from relapsing-remitting to secondary progressive MS is 19 years.
189
Describe primary progressive MS
Primary progressive: Describes the approximately 10–15% of individuals who never have remission after their initial MS symptoms. It is characterized by progression of disability from onset, with no, or only occasional and minor, remissions and improvements. The age of onset for the primary progressive subtype is later than other subtypes.
190
what is progressive relapsing MS?
Progressive relapsing: Describes those individuals who, from onset, have a steady neurologic decline but also suffer clear superimposed attacks. This is the least common of all subtypes.
191
what are non-standard behaviors in MS?
Non-standard behaviors: Have also been described. Sometimes referred to as borderline forms of MS, these include Devic's disease, Balo concentric sclerosis, Schilder's diffuse sclerosis and Marburg MS. MS also behaves differently in children. There is debate whether these are atypical variants of MS or different diseases
192
What are the the visual features of MS?
Visual
• Optic neuritis: common presenting feature
• Optic atrophy
• Uhthoff's phenomenon: worsening of
vision following rise in body temperature
(hot bath)
• Internuclear ophthalmoplegia
193
Describe the sensory features of MS
```
Sensory
• Pins/needles
• Numbness
• Trigeminal neuralgia
• Lhermitte's syndrome: paraesthesiae in
limbs on neck flexion
```
194
describe the motor features of MS
Motor
| • Spastic weakness
195
describe the cerebellar features of MS
Cerebellar
| • Ataxia, Tremor
196
describe the urinary features of MS
Urinary incontinence
197
describe the sexual features of MS
Sexual dysfunction
198
describe the cognition features of MS
Intellectual deterioration
199
what MS features carry a good prognosis?
```
Good prognosis features
• ♀sex
• Young age of onset
• Relapsing-remitting disease
• Sensory symptoms
• long interval between first two relapses
```
TYPICAL PRESENTATION
200
what is the diagnosis of MS?
```
Diagnosis requires demonstration of lesions disseminated in time and space
MRI
• High signal T2 lesions
• Periventricular plaques
CSF
• Oligoclonal bands (and not in serum)
• ↑ intrathecal synthesis of IgG
Visual evoked potentials
• Delayed, but well preserved wave form
```
201
What is the treatment of relapses in MS?
Treatment in multiple sclerosis is focused at reducing the frequency and duration of relapses. There is no cure. High dose steroids (e.g. IV methylprednisolone) may be given for 3-5 days to shorten the length of an acute relapse. Baclofen is helpful in controlling spasticity. Hallucinations are occasionally seen on the withdrawal of baclofen
202
what are the disease modifying drugs in MS?
Beta-interferon has been shown to reduce the relapse rate by up to 30%. Certain criteria have to be met before it is used:
relapsing-remitting disease + 2 relapses in past 2 years + able to walk 100m unaided
secondary progressive disease + 2 relapses in past 2 years + able to walk 10m (aided or unaided)
reduces number of relapses and MRI changes, however doesn't reduce overall disability
Other drugs used in the management of multiple sclerosis include:
glatiramer acetate: immunomodulating drug - acts as an 'immune decoy'
natalizumab: a recombinant monoclonal antibody that antagonises Alpha-4 Beta-1-integrin found on the surface of leucocytes, thus inhibiting migration of leucocytes across the endothelium across the blood-brain barrier
fingolimod: sphingosine 1-phosphate receptor modulator, prevents lymphocytes from leaving lymph nodes. An oral formulation is available
203
what can be used for fatigue in MS?
Fatigue
once other problems (e.g. anaemia, thyroid or depression) have been excluded NICE recommend a trial of amantadine
other options include mindfulness training and CBT
204
what can be used for spasticity in MS?
baclofen and gabapentin are first-line. Other options include diazepam, dantrolene and tizanidine
physiotherapy is important
cannabis and botox are undergoing evalulation
205
what can be done for bladder dysfunction in MS?
may take the form of urgency, incontinence, overflow etc
guidelines stress the importance of getting an ultrasound first to assess bladder emptying - anticholinergics may worsen symptoms in some patients
if significant residual volume → intermittent self-catheterisation
if no significant residual volume → anticholinergics may improve urinary frequency
206
what can be done for oscillopsia in MS?
Oscillopsia (visual fields apper to oscillate)
gabapentin is first-line
207
Ptosis + dilated pupil = ? nerve palsy
Ptosis + dilated pupil = third nerve palsy
208
Ptosis + constricted pupil = ? syndrome
Horner's syndrome
209
what are 3 drug causes of miosis (constricted pupil)?
* Opiates
* Parasympathomimetics: pilocarpine
* Organophosphate toxicity
210
what happens to the pupil when accommodating?
constricts
211
what is an argyll-robertson pupil?
| -what are the causes for this?
Argyll-Robertson: small irregular pupils that do not react to light but react to accommodation.
Causes: Multiple Sclerosis, Sarcoidoisis, DM
Referred to as the "Whore's Eye" because of the association with tertiary syphilis and because of the
convenient mnemonic that, like a prostitute, they “accommodate but do not react”
212
how can you work out the site of the lesion in Horner's syndrome?
-what is this caused by?
Horner's syndrome - anhydrosis determines site of lesion:
• Head, arm, trunk = central lesion: stroke, syringomyelia
• Just face = pre-ganglionic lesion: Pancoast's, cervical rib
• Absent = post-ganglionic lesion: carotid artery
It is caused by an interruption of the sympathetic pupillomotor fibres
213
where is lesion in Horner's syndrome with Anhydrosis of the face, arm and trunk?
What could cause this?
central: STEMS
```
stroke
tumour
encephalitis
multiple sclerosis
syringomyelia
```
214
Where is the lesion in Horner's syndrome with Anhydrosis of the face
pre-ganglionic: PCT
pancoast tumour
cervical rib
trauma
thyroidectomy
215
Where is the lesion in Horner's syndrome with no anhydrosis?
Post-ganglionic: all the C's
Carotid artery dissection
Carotid aneurysm
Cavernous sinus thrombosis
Cluster headache
216
what are four causes of bilateral ptosis?
* Myotonic dystrophy
* Myasthenia gravis*
* Syphilis
* Congenital
*ptosis is much less common in Lambert-Eaton syndrome than myasthenia gravis
217
what are 2 causes of unilateral ptosis
* Third nerve palsy
| * Horner's
218
what should a painful third nerve palsy indicate?
Painful third nerve palsy = posterior communicating artery aneurysm
219
what are the features of third nerve palsy?
* Eye is deviated 'down and out'
* Ptosis
* Pupil may be dilated (sometimes called a 'surgical' third nerve palsy) → PCA aneurysm
220
what are the causes of third nerve palsy?
• Diabetes Mellitus
• Vasculitis e.g. Temporal arteritis, SLE
• False localizing sign due to uncal herniation through tentorium
if raised ICP
• Posterior communicating artery aneurysm (pupil dilated)
• Cavernous sinus thrombosis
• Weber's syndrome: ipsilateral third nerve palsy with
contralateral hemiplegia -caused by midbrain strokes
(cerebral peduncle)
• Other possible causes: amyloid, multiple sclerosis
221
what is myaesthenia gravis?
| -what antibodies are most commonly seen?
Myasthenia Gravis: Myasthenia gravis is an autoimmune disorder resulting in insufficient functioning acetylcholine receptors. Antibodies to acetylcholine receptors are seen in 90% of cases (antibodies are less commonly seen in disease limited to the ocular muscles).
222
is myaesthenia more common in men or women?
| -what is the pathophysiology?
Myasthenia is more common in women (2:1). It is a neuromuscular disease leading to fluctuating muscle weakness and fatiguability, in which weakness is caused by circulating antibodies that block acetylcholine receptors at the post-synaptic neuromuscular junction.
223
is myaesthenia common and what is the hallmark feature?
At 200–400 cases per million it is one of the less common autoimmune disorders. The most common exacerbating factor is exertion resulting in fatigability, which is the hallmark feature of myasthenia gravis. Symptoms become more marked during the day
224
describe the features of myaesthenia gravis?
```
Features
• Extraocular muscle weakness: diplopia
• Proximal muscle weakness: face, neck, limb girdle
• Ptosis
• Dysphagia
```
225
what conditions are assoc with myaesthenia gravis?
Associations
• Thymomas in 15%
• Autoimmune disorders: pernicious anemia, hypothyroidism, rheumatoid, SLE
• Thymic hyperplasia in 50-70%
226
what is the management of myaesthenia gravis?
Management
• Long-acting anticholinesterase e.g. Pyridostigmine
• Immunosuppression: prednisolone initially (although sometimes it can worsen the symptoms)
• Thymectomy
227
what is the management of myaesthenic crisis in myaesthenia gravis?
Management
• Long-acting anticholinesterase e.g. Pyridostigmine
• Immunosuppression: prednisolone initially (although sometimes it can worsen the symptoms)
• Thymectomy
228
• β-blockers (theoretical worsening based on propensity to
cause side effects of fatigue and weakness)
• Gentamicin, Aminoglycosides and Tetracyclin
• Lithium
• Magnesium
• Penicillamine
• Phenytoin
• Quinidine, procainamide, verapamil, contrast agents.
• Chloroquine
• Prednisolone
who can not recieve the above drugs?
myaesthenia gravis patients - may worsen symptoms
229
what is lambert-eaton myaesthenic syndrome?
- what is assoc with this?
- what is it caused by?
Lambert-Eaton Myasthenic Syndrome is seen in association with small cell lung cancer, and to a lesser extent breast and ovarian cancer. It may also occur independently as an autoimmune disorder. Lambert-Eaton myasthenic syndrome is caused by an antibody directed against pre-synaptic voltage gated calcium channel in the peripheral nervous system
230
what are the features of lambert-eaton myaesthenic syndrome?
Features
• Repeated muscle contractions lead to ↑ muscle strength* (in contrast to myasthenia gravis)
• Limb girdle weakness (affects lower limbs first). Proximal muscles more commonly affected.
• Hyporeflexia
• Autonomic symptoms: dry mouth, impotence, difficultly micturating
• Ophthalmoplegia and ptosis not commonly a feature (unlike in myasthenia gravis)
*in reality this is seen in only 50% of patients and following prolonged muscle use muscle strength will eventually ↓
231
what is the investigation for lambert-eaton syndrome?
EMG
| • Incremental response to repetitive electrical stimulation
232
what is the management for lambert-eaton?
Management
-treatment of underlying cancer
-immunosuppression, for example with prednisolone and/or azathioprine
-3,4-diaminopyridine is currently being trialled
works by blocking potassium channel efflux in the nerve terminal so that the action potential duration is increased. Calcium channels can then be open for a longer time and allow greater acetylcholine release to the stimulate muscle at the end plate
intravenous immunoglobulin therapy and plasma exchange may be beneficial
233
* Associated with small cell lung carcinoma and neuroblastomas
* Sensory neuropathy - may be painful
* Cerebellar syndrome
* Encephalomyelitis
what is the paraneoplastic syndrome?
Anti-Hu (imagine H sticks as 2 lungs or 2 brain hemispheres)
234
* Associated with ovarian and breast cancer
* Cerebellar syndrome
what is the paraneoplastic syndrome?
Anti-Yo (imagine Y as lady’s private organ
235
* Associated with breast, colorectal and small cell lung carcinoma
* Stiff person’s syndrome or diffuse hypertonia
what is the paraneoplastic syndrome?
Anti-GAD antibody
236
* Associated with breast and small cell lung carcinoma
* Ocular opsoclonus-myoclonus
what is the paraneoplastic syndrome?
Anti-Ri
237
what is paraneoplastic cerebellar syndrome?
- what is this believed to be caused by?
- how does this typically present?
It is believed to be due to an autoimmune reaction targeted against components of the central nervous system (specifically Purkinje cells and large brain stem nuclei). It is thought to be caused by an anti-neuronal Antibody known as anti-Yo.
It typically presents as a subacute progressive cerebellar ataxia, both truncal and appendicular.
238
what is an acoustic neuroma?
Acoustic Neuromas account for approximately 5% of intracranial tumours and 90% of cerebellopontine angle. AKA Vestibular schwannoma, it is a benign primary intracranial tumor of the myelin-forming cells of the vestibulocochlear nerve (CN VIII). The term "acoustic" is a misnomer, as the tumor rarely arises from the acoustic (or cochlear) division of the vestibulocochlear nerve
239
in acoustic neuroma - where is the lesion:
- hearing loss/vertigo/tinnitus
- absent corneal reflex
- facial palsy
Features can be predicted by the affected cranial nerves
• Cranial nerve VIII: hearing loss, vertigo, tinnitus
• Cranial nerve V: absent corneal reflex
• Cranial nerve VII: facial palsy
240
which condition are bilateral vestibular schwannomas seen in?
Bilateral vestibular schwannomas are seen in neurofibromatosis type 2.
241
how should suspected vestibular schwannoma be managed?
Patients with a suspected vestibular schwannoma should be referred urgently to ENT. It should be noted though that the tumours are often slow growing, benign and often observed initially.
242
what are the investigations for suspected vestibular schwannoma?
MRI of the cerebellopontine angle is the investigation of choice. Audiometry is also important as only 5% of patients will have a normal audiogram.
243
what is the management of vestibular schwannoma?
Management is with either surgery, radiotherapy or observation.
244
what are glomus jugulare tumours?
| -where do they arise?
Glomus Jugulare Tumours are slow-growing, highly vascular tumours derived from neural tissue and arising within the jugular foramen of the temporal bone.
245
what is the female:male ratio of glomus jugulare tumours?
| -when do these present?
♀:♂ ratio between 3 and 6:1 - Annual incidence is around 1 in 1.3 million.
Tend to present between 40 and 70 years of age.
246
how does glomus jugulare tumours present?
Presents with deafness and pulsatile tinnitus
| Rarely causes hearing loss (4%) with vertigo (if extends to middle ear)
247
which cranial nerves are affected by glomus jugulare tumours?
Cranial nerves IX to XI pass through the jugular foramen, and so are commonly involved.
Less commonly, affect cranial nerves VII and XII.
248
what does otoscopic examination show in glomus jugulare tumours?
Otoscopic examination may reveal a characteristic pulsatile reddish-blue mass behind the
tympanic membrane.
249
what is the treatment for glomus jugulare tumours?
Surgical resection is the treatment of choice, embolisation and radiotherapy have been used.
250
The below peripheral neuropathies have what kind of pathology?
```
• Guillain-Barre syndrome
• Chronic inflammatory demyelinating
polyneuropathy (CIDP)
• Amiodarone
• Hereditary sensorimotor neuropathies (HSMN)
type I
• Paraprotein neuropathy
```
Demyelinating pathology
251
The below peripheral neuropathies have what kind of pathology?
• Alcohol
• Diabetes mellitus (±demyelinating picture)
• Vasculitis
• Vit. B12 deficiency (±demyelinating picture)
• Hereditary sensorimotor neuropathies (HSMN)
type II
axonal pathology
252
The below peripheral neuropathies have motor or sensory loss?
* Guillain-Barre syndrome
* Porphyria
* Lead poisoning
* HSMN - Charcot-Marie-Tooth
* CIDP
* Diphtheria
Predominately motor loss
253
The below peripheral neuropathies have motor or sensory loss?
* Diabetes
* Uremia
* Leprosy
* Alcoholism
* Vitamin B12 deficiency
* Amyloidosis
sensory
254
in alcoholic neuropathy:
- what is this secondary to?
- what symptoms present first?
* Secondary to both direct toxic effects and ↓ absorption of B vitamins
* Sensory symptoms typically present prior to motor symptoms
255
what does B12 deficiency lead to? what symptoms present first?
* Subacute combined degeneration of spinal cord
| * Dorsal column usually affected first (joint position, vibration) prior to distal paraesthesia
256
what 5 drugs causes peripheral neuropathy?
* Antibiotics: nitrofurantoin, metronidazole
* Amiodarone
* Isoniazid
* Vincristine and most of the anti-cancer chemotherapy
* Tricyclic antidepressants
257
what is found on nerve conduction studies in demyelinating illness?
Demyelinating
• Reduced conduction velocity
• Normal amplitude
258
what is found on nerve conduction studies in axonal illness?
Axonal
• Normal conduction velocity
• Reduced amplitude
259
what are the features of autonomic neuropathy?
Features
• Impotence, inability to sweat
• Postural hypotension e.g. Drop of 30/15 mmHg
• Loss of ↓ in heart rate following deep breathing
• Pupils: dilates following adrenaline instillation
260
* Diabetes
* Guillain-Barre syndrome
* Multisystem atrophy (MSA), Shy-Drager
* Parkinson's
* Infections: HIV, Chagas', neurosyphilis
* Drugs: antihypertensives, tricyclics
* Craniopharyngioma
all cause what?
autonomic neuropathy
261
what is guillain-barre?
Guillain-Barre Syndrome describes an immune mediated demyelination of the peripheral nervous system often triggered by an infection (classically Campylobacter jejuni). It is usually a subacute neuropathy.
262
describe the pathogenesis of guillain-barre
Pathogenesis
• Cross reaction of antibodies with gangliosides in the peripheral nervous system
• Correlation between anti-ganglioside antibody (e.g. Anti-GM1) and clinical features has been
demonstrated
• Anti-GM1 antibodies in 25% of patients
263
what are the precipitating organisms of guillain-barre?
* Campylobacter jejuni
* Chlamydia
* HBV
* Mycoplasma Pneumoniae
* CMV, EBV, HZV, HIV
264
how does guillain-barre usually present?
• Usually presents subacutely (over days to weeks) as an ascending paralysis noted by weakness in the legs that spreads to the upper limbs and the face along with complete loss of deep tendon reflexes. It may involve respiratory muscles leading to their paralysis.
265
What is the management of guillain-barre?
* Plasma exchange
* IV immunoglobulins
* Steroids and immunosuppressants have not been shown to be beneficial
* FVC regularly to monitor respiratory function
266
what is the prognosis of Guillain-barre?
Prognosis
| • 20% suffer permanent disability, 5% die
267
what are poor prognostic factors for guillain-barre?
* Age > 40 years
* Poor upper extremity muscle strength
* Rapid symptoms progression.
* Previous history of a diarrhoeal illness (specifically campylobacter jejuni)
* High anti-GM1 antibody titre
* Need for ventilatory support
There is currently contradictory evidence as to whether a gradual or rapid onset of GBS is associated with a poor outcome
268
what is miller-fisher syndrome?
| -what antibodies are involved?
Miller-Fisher syndrome
• Variant of Guillain-Barre syndrome
• Associated with areflexia, ataxia, ophthalmoplegia
• Usually presents as a descending paralysis rather than ascending as seen in other forms of
Guillain-Barre syndrome
• Anti-GQ1b antibodies are present in 90% of cases
269
which lobe does herpes simplex encephalitis affect?
Herpes Simplex (HSV) Encephalitis is a common topic in the MRCP. The virus characteristically affects the temporal lobes - questions may give the result of imaging or describe temporal lobe signs e.g. aphasia
270
describe the features of herpes simplex encephalitis
Features
• Fever, headache, psychiatric symptoms, seizures, vomiting
• Focal features e.g. Aphasia
• Peripheral lesions (e.g. Cold sores) have no relation
to presence of HSV encephalitis
271
describe the features of herpes simplex encephalitis
Pathophysiology
• HSV-1 responsible for 95% of cases in adults
• Typically affects temporal and inferior frontal lobes
272
```
describe what is seen on:
-CSF
-PCR
-CT/MRI
-EEG
for herpes simplex encephalitis?
```
Investigation
• CSF: lymphocytosis, elevated protein
• PCR for HSV
• CT: medial temporal and inferior frontal changes
(e.g. Petechial hemorrhages) - normal in one-third of
patients
• MRI is better
• EEG pattern: lateralised periodic discharges (LPD,
not LAPD☺) at 2 Hz
273
What is the treatment for herpes simplex encephalitis?
Treatment
| • IV aciclovir
274
what is the prognosis of herpes simplex encephalitis?
The prognosis is dependent on whether aciclovir is commenced early. If treatment is started promptly
the mortality is 10-20%. Left untreated the mortality approaches 80%
275
what is sub-acute sclerosis panencephalitis?
| -what is the disease progression?
Sub-acute Sclerosing Panencephalitis: It is recognized to be the result of chronic measles infection. It is now an uncommon disease after the widespread use of measles vaccination. There is usually a history of measles very early in life followed by 8 years of asymptomatic period.
276
describe the stages of evolution of sub-acute sclerosis panencephailitis?
• Evolves in several stages.
" Initially: decline in proficiency in school.
" Followed by diffuse myoclonic jerks in association with focal and generalised seizures and visual deterioration due to choiroidoretinitis.
" Followed by pyramidal signs, rigidity and progressive unresponsiveness.
" Finally the patient lies decorticated followed by death.`
The course of the illness is 1 to 3 years. No effective treatment is available
277
describe the encephalitis seen in HIV infection
1. Encephalitis
• May be due to CMV or HIV itself
• HSV encephalitis but is relatively rare in the context of HIV
• CT: edematous brain
278
describe cryptococcus CNS infection in HIV?
- common?
- what are the features?
- what is seen in CSF
- what is seen on CT
- what may this cause?
2. Cryptococcus
• Most common fungal infection of CNS
• Headache, fever, malaise, nausea/vomiting, seizures, focal
neurological deficit
• CSF: high opening pressure, India ink test positive
• CT: meningeal enhancement, cerebral edema →
• Meningitis is typical presentation but may occasionally cause a space occupying lesion
279
HIV neurocomplications:
- what is PML?
- what is this caused by?
- what are the symptoms?
- what is seen on CT scan?
- what is seen on MRI scan?
- which is better - CT or MRI?
3. Progressive multifocal leukoencephalopathy (PML)
• Widespread demyelination
• Due to infection of oligodendrocytes by human papovirus (jc virus)
• Symptoms, subacute onset : behavioural changes, speech, motor,
visual impairment
• CT: single or multiple lesions, no mass effect, don't usually enhance, Low attenuation diffusely
• MRI is better - high-signal demyelinating white matter lesions are seen in advanced HIV.
280
what is AIDS dementia complex?
- what is this caused by?
- what are the symptoms?
- what is seen on CT scan?
4. AIDS dementia complex
• Caused by HIV virus itself
• Symptoms: behavioural changes, motor impairment
• CT: cortical and subcortical atrophy
281
Focal neurological lesions in HIV
what is toxoplasmosis:
- what proportion of cerebral lesions does this count for in patients with HIV?
- what symptoms are seen?
- what is seen on CT scan?
- what is the management of toxoplasmosis?
1. Toxoplasmosis
• Accounts for around 50% of cerebral lesions in patients with HIV
• Constitutional symptoms, headache, confusion, drowsiness
• CT: usually single or multiple ring enhancing lesions, mass
effect may be seen
• Management: sulfadiazine and pyrimethamine
282
Focal neurological lesions in HIV
What condition is primary CNS lymphoma assoc with?
What proportion of cerebral lesions does this account for in HIV patients?
what is seen on CT scan?
2. Primary CNS lymphoma
• Accounts for around 30% of cerebral lesions
• Associated with the Epstein-Barr virus
• CT: single or multiple ring enhancing lesions
283
Focal neurological lesions in HIV: TB
- more common or less common than other focal neurological lesions?
- what is seen on CT scan?
3. Tuberculosis
• Much less common than toxoplasmosis or primary lymphoma
• CT: Single enhancing lesion
284
```
what do:
• Distal weakness initially
• autosomal Dominant
• Diabetes
• Dysarthria
all link with?
```
```
Myotonic dystrophy (also called dystrophia myotonica)
DM1
```
285
what is myotonic dystrophy?
- when do features develop?
- what does this affect?
- what are the two main types?
Myotonic dystrophy (also called dystrophia myotonica) is an inherited myopathy with features developing at around 20-30 years old. It affects skeletal, cardiac and smooth muscle. There are two main types of myotonic dystrophy, DM1 and DM2.
286
Myotonic Dystrophy:
- how is this inherited
- which gene is DM1 caused by?
- which gene is DM2 caused by?
Genetics
• Autosomal dominant
• A trinucleotide repeat disorder
• DM1 is caused by a CTG repeat at the end of the DMPK (Dystrophia Myotonica-Protein
Kinase) gene on chromosome 19
• DM2 is caused by a repeat expansion of the ZNF9 gene on chromosome 3
287
- DMPK gene on chromosome 19
- Distal weakness more prominent
= DM1 or 2
DM1
288
- ZNF9 gene on chromosome 3
- Proximal weakness more prominent
- Severe congenital form is not seen
=DM1 or 2?
DM2
289
what are the general features of myotonic dystrophy?
| -what may be noticed on initial hand shake?
```
General features (typically in the above figure)
• Myotonic facies (long, 'haggard'
appearance)
• Frontal balding
• Bilateral ptosis
• Cataracts
• Dysarthria
```
Slow-relaxing grip may be noticed on initial hand-shake with the patient and is typical of myotonic dystrophy. Dysarthric speech is secondary to myotonia of the tongue and pharynx
290
What are the less common features of myotonic dystrophy;
- muscular features
- congnitive features
- endocrine complications
- testicular complications
- cardiac involvement
- swallow issues
Myotonia (tonic spasm of muscle)
Weakness of arms and legs (distal initially)
Mild mental impairment
Diabetes mellitus
Testicular atrophy
Cardiac involvement: heart block, cardiomyopathy
Dysphagia
291
```
All of the below are causes of what?
• Demyelination e.g. Multiple sclerosis
• Cord compression: trauma, tumour
• Parasagittal meningioma
• Tropical spastic paraparesis
• Hereditary spastic paraplegia
• Transverse myelitis e.g. HIV
• Syringomyelia
• Osteoarthritis of the cervical spine
```
Spastic Paraparesis: describes a upper motor neuron pattern of weakness in the lower limbs
292
what is trigeminal neuralgia?
Trigeminal neuralgia is a pain syndrome characterized by severe unilateral pain. The vast majority of cases are idiopathic but compression of the trigeminal roots by tumours or vascular problems may occur. The International Headache Society defines trigeminal neuralgia as:
• A unilateral disorder characterized by brief electric shock-like pains, abrupt in onset and termination, limited to one or more divisions of the trigeminal nerve
• The pain is commonly evoked by light touch, including washing, shaving, smoking, talking, and brushing the teeth (trigger factors), and frequently occurs spontaneously
• Small areas in the nasolabial fold or chin may be particularly susceptible to the precipitation of pain (trigger areas)
• The pains usually remit for variable periods
293
what is the management of trigeminal neuralgia?
Management
carbamazepine is first-line
failure to respond to treatment or atypical features (e.g. < 50 years old) should prompt referral to neurology
294
what are 'red flags' symptoms and signs in trigeminal neuralgia that suggest serious underlying cause?
Sensory changes
Deafness or other ear problems
History of skin or oral lesions that could spread perineurally
Pain only in the ophthalmic division of the trigeminal nerve (eye socket, forehead, and nose), or bilaterally
Optic neuritis
A family history of multiple sclerosis
Age of onset before 40 years
295
what are the causes for optic neuritis?
Causes
multiple sclerosis
diabetes
syphilis
296
what are the features of optic neuritis?
unilateral decrease in visual acuity over hours or days
poor discrimination of colours, 'red desaturation'
pain worse on eye movement
relative afferent pupillary defect
central scotoma
297
what is the management of optic neuritis?
Management
high-dose steroids
recovery usually takes 4-6 weeks
298
what is the prognosis of optic neuritis?
Prognosis
MRI: if > 3 white-matter lesions, 5-year risk of developing multiple sclerosis is c. 50%
299
what is the management for neuropathic pain:
- first line
- is this a monotherapy or an add on?
- what may be used as a rescue therapy?
- what may be used for localised neuropathic pain?
NICE updated their guidance on the management of neuropathic pain in 2013:
first-line treatment: amitriptyline, duloxetine, gabapentin or pregabalin
if the first-line drug treatment does not work try one of the other 3 drugs
in contrast to standard analgesics, drugs for neuropathic pain are typically used as monotherapy, i.e. if not working then drugs should be switched, not added
tramadol may be used as 'rescue therapy' for exacerbations of neuropathic pain
topical capsaicin may be used for localised neuropathic pain (e.g. post-herpetic neuralgia)
pain management clinics may be useful in patients with resistant problems
300
Episodic eye pain, lacrimation, nasal stuffiness occurring daily - ? headache
cluster
301
what are 7 features of cluster headache?
Features
• Pain typical occurs once or twice a day, each episode lasting 15 mins - 2 hours
• Clusters typically last 4-12 weeks
• Intense pain around one eye (recurrent attacks 'always'
affect same side)
• Patient is restless during an attack
• Accompanied by redness, lacrimation, lid swelling
• Nasal stuffiness
• Miosis and ptosis in a minority
302
what is the management of cluster headache?
Management
• Acute: 100% oxygen, subcutaneous sumatriptan (5-HT1D receptor agonist), nasal lidocaine
• Prophylaxis: verapamil, prednisolone
• Consider specialist referral
303
what are the features of Chronic Paroxysmal Hemicrania (CPH)?
• Unilateral pain which is generally oculofrontotemporal in location.
• Can occur at any time, and patients often describe a throbbing, boring, pulsating or claw-like
pain
• Frequency of attacks is usually 10–20 per day. Episodes usually last 2–25 min, but may last as
long as 60 min
• Patients may also complain of ipsilateral conjunctival injection, ptosis and lid swelling,
lacrimation and rhinorrhoea, occasionally photophobia
304
what is the treatment of CPH?
• The most effective treatment is with indomethacin
305
what type of headache is seen in SUNCT
short-lasting unilateral neuralgiform headache with conjunctival injection and tearing
306
CH or CPH:
- male preponderance
- higher frequency of attacks
- shorter duration of headache
CH has male preponderance, unlike CPH, which is more common in females.
In CPH, frequency of attacks is higher, usually more than 15 in 24 hours, whereas CH has an attack frequency of 1-4 (maximum 8) in 24 hours.
The duration of headaches is shorter in CPH (2-25 min) than in CH (15-60 min).
307
what is the diagnostic criteria for migraine?
A At least 5 attacks fulfilling criteria B-D
B Headache attacks lasting 4-72 hours* (untreated or unsuccessfully treated)
C Headache has at least two of the following characteristics:
1. unilateral location
2. pulsating quality (i.e., varying with the heartbeat)
3. moderate or severe pain intensity
4. aggravation by or causing avoidance of routine physical activity (e.g., walking or climbing stairs)
D During headache at least one of the following:
1. nausea and/or vomiting
2. photophobia and phonophobia
E Not attributed to another disorder (history and examination do not suggest a secondary headache disorder or, if they do, it is ruled out by appropriate investigations or headache attacks do not occur for the first time in close temporal relation to the other disorder)
308
what is different about migraines that happen in children?
In children, attacks may be shorter-lasting, headache is more commonly bilateral, and gastrointestinal disturbance is more prominent
309
what is the treatment for acute migraine?
Standard analgesia, Triptans & Ergotamine.
Standard analgesia?
• First-line therapy
• E.g. Paracetamol, ibuprofen, aspirin, may be poorly
absorbed, often combined with anti-emetic e.g. Metoclopramide (to relieve associate nausea) → caution should be exercised with young patients as acute dystonic reactions may develop
Avoid aspirin in children < 16 years as risk of Reye's syndrome
Triptans
• Second-line therapy
• Specific 5-HT1 agonists - opposes Vasodilation
Ergotamine
• α-blocker and a partial 5-HT1 agonist
• Now rarely used due to high incidence of adverse effects (e.g. Nausea and vomiting)
• Listed in the BNF as 'less suitable for prescribing'
310
What is the prophylaxis for migraine?
| -when should this be given
Prophylaxis should be given if patients are experiencing ≥ 2 attacks/month. Treatment is effective in about 60% of patients
First-line
• β-blockers: propranolol 80-240mg OD
```
Also recommended in the SIGN guidelines
• Sodium valproate
• Topiramate (CKS recommend this is used under
specialist supervision)
• Gabapentin
• Amitriptyline
• Venlafaxine
```
The SIGN guidelines also suggest that stress management and acupuncture may be useful
5-HT2 antagonists
• Pizotifen: used less commonly now due to adverse effects (weight gain and drowsiness)
• Methysergide: very rarely used as associated with retroperitoneal fibrosis
311
what is used to treat migraine during pregnancy?
Migraine during pregnancy
• Paracetamol 1g is first-line
• Aspirin 300mg or ibuprofen 400mg can be used second-line in the first and second trimester
312
what drug is contraindicated in patients with migraine?
Migraine and the combined oral contraceptive (COC) pill
• If patients have migraine with aura then the COC is absolutely contraindicated due to an increased risk of stroke (relative risk 8.72)
313
what can be used if migraines increase in severity and frequency around the time of menstruation?
Migraine and menstruation
• Many women find that the frequency and severity of migraines increase around the time of menstruation
• SIGN recommends that women are treated with mefanamic acid or a combination of aspirin,
paracetamol and caffeine. Triptans are also recommended in the acute situation
314
can HRT be used with migraine?
Migraine and hormone replacement therapy (HRT)
| • Safe to prescribe HRT for patients with a history of migraine but it may make migraines worse
315
```
Normal values of cerebrospinal fluid (CSF) are?
• Pressure =
• Protein =
• Glucose =
• Cells:
```
Normal values of cerebrospinal fluid (CSF) are as follows:
• Pressure = 60-150 mm (patient recumbent)
• Protein = 0.2-0.4 g/l
• Glucose = > 2/3 blood glucose
• Cells: red cells = 0, white cells < 5/mm3
316
what conditions are associated with a raised lymphocyte count in the CSF?
* Viral meningitis/encephalitis
* TB meningitis
* Partially treated bacterial meningitis
* Lyme disease
* Behcet's, SLE
* Lymphoma, leukemia
317
what conditions are associated with a raised protein level in the CSF?
* Tuberculous, fungal and bacterial meningitis
* Viral encephalitis
* Guillain-Barre syndrome
* Spinal block (Froin's syndrome)
318
who are post-LP headaches more common in?
| -what are the typical features?
Post-LP headaches are more common in young ♀ with a low BMI.
Typical features
• Usually develops within 24-48 hours following LP but may occur up to one week later
• May last several days
• Worsens with upright position
• Improves with recumbent position
319
what factors may contribute to post-LP headache?
↑ needle size
Direction of bevel
Not replacing the stylet
↑ number of LP attempts
320
what factors do not contribute to post-LP headache?
```
↑ volume of CSF removed
Bed rest following procedure
↑ fluid intake post procedure
Opening pressure of CSF
Position of patient
```
321
what treatments can be given for post-LP headache?
• Supportive initially (analgesia, rest)
• If pain continues for more than 72 hours then specific treatment is indicated, to prevent subdural
hematoma
• Treatment options include: blood patch, epidural saline and intravenous caffeine
322
what is oppenheim's sign and what does this indicate?
Oppenheim's sign is seen when scratching of the inner side of leg leads to extension of the toes. It is a sign of cerebral irritation
323
what is seen on the EMG in polymyositis?
• Polymyositis: reduced amplitude and duration of motor unit
324
what is seen on EMG in MND?
MND: fibrillation due to denervation
325
what is seen on the EMG in myaesthenia gravis?
Myasthenia Gravis: diminished responses to repitative stimulations
326
what is seen on the EMG in lambert eaton myaesthenia?
Lambert Eaton Myasthenia: enhanced responses to repitative stimulations.
327
what is seen on the EMG in myotonic dystrophy?
Myotonia Dystophia: High frequency action potentioals, Kamikaze discharge or dive bombers
frequency which is heard
328
what is an arnold-chiari malformation?
Arnold-Chiari malformation describes the downward displacement, or herniation, of the cerebellar tonsils through the foramen magnum. Malformations may be congenital or acquired through trauma.
329
what are the features of arnold-chiari malformation?
Features
non-communicating hydrocephalus may develop as a result of obstruction of cerebrospinal fluid (CSF) outflow
headache
syringomyelia
330
what is unicrentric castlemans disease?
Unicentric Castleman's disease is a lymphoproliferative disorder associated in a subset of cases with HIV and HHV-8.
331
what is the presentation of unicentric castleman's disease?
Patient's with unicentric Castleman's disease tend to be asymptomatic and lymphadenopathy is constrained to one lymph node group.
332
what are the most common sites for castleman's disease?
The most common sites of the disease being the chest (24 percent), neck (20 percent), abdomen (18 percent), and retroperitoneum (14 percent).
333
what does lymph node biopsy show in castleman's disease?
Biopsy of the lymph node commonly shows regressed germinal centres surrounded by prominent mantle zones.
334
what are possible causes of a foot drop?
Possible causes include:
common peroneal nerve lesion - the most common cause
L5 radiculopathy
sciatic nerve lesion
superficial or deep peroneal nerve lesion
other possible includes central nerve lesions (e.g. stroke) but other features are usually present
335
what is the most common cause of a foot drop?
A common peroneal nerve lesion is the most common cause . This is often secondary to compression at the neck of the fibula. This may be caused by certain positions such as leg crossing, squatting or kneeling. Prolonged confinement, recent weight loss, Baker's cysts and plaster casts to the lower leg are also known to be precipitating factors.
336
what is seen on examination of a foot drop?
Examination
if the patient has an isolated peroneal neuropathy there will be weakness of foot dorsiflexion and eversion. Reflexes will be normal
weakness of hip abduction is suggestive of a L5 radiculopathy
337
what symptoms in a foot drop would prompt specialist referral?
Bilateral symptoms, fasiculations or other abnormal neurological findings (e.g. hyperreflexia) are indications for specialist referral.
338
what is the management for common peroneal neuropathy?
If the examination suggests a peroneal neuropathy then conservative management is appropriate. Leg crossing, squatting and kneeling should be avoided. Symptoms typically improve over 2-3 months
339
internuclear opthalmoplegia:
- what is this due to?
- where is this located?
a cause of horizontal disconjugate eye movement
due to a lesion in the medial longitudinal fasciculus (MLF)
controls horizontal eye movements by interconnecting the IIIrd, IVth and VIth cranial nuclei
located in the paramedian area of the midbrain and pons
340
what are the features of internuclear opthalmoplegia?
Features
impaired adduction of the eye on the same side as the lesion
horizontal nystagmus of the abducting eye on the contralateral side
341
what are the causes of internuclear opthalmoplegia?
Causes
multiple sclerosis
vascular disease
342
what is the role of Supraoptic nucleus of the hypothalamus
This area of the hypothalamus along with the paraventricular nucleus of the hypothalamus is responsible for the synthesis of antidiuretic hormone and oxytocin, which are transported to the posterior hypothalamus for storage and release.
343
what is the role of the posterior hypothalamus?
The posterior hypothalamus is responsible for heat generation to maintain core body temperature.
344
what is the role of the anterior hypothalamus?
3: The anterior hypothalamus is responsible for heat dissipation to cool down the body to prevent a rise in temperature which would be detrimental to body's internal environment.
345
what is the role of the ventromedial area of the hypothalamus and what is this often invaded by?
4: The ventromedial area of the hypothalamus is often invaded by craniopharyngiomas. This area of the thalamus controls the satiety center and it is removed during surgery, the patient can have uninhibited hunger leading to significant weight gain.
346
what is the role of the paraventricular nucleus of the hypothalamus?
5: This area of the hypothalamus along with the supraoptic nucleus of the hypothalamus is responsible for the synthesis of antidiuretic hormone and oxytocin, which are transported to the posterior hypothalamus for storage and release.
347
what is menieres disease?
Meniere's disease is a disorder of the inner ear of unknown cause. It is characterized by excessive pressure and progressive dilation of the endolymphatic system. It is more common in middle- aged adults but may be seen at any age. Meniere's disease has a similar prevalence in both men and women
348
what are the 4 features of menieres disease?
Features
• Recurrent episodes of vertigo, tinnitus and hearing loss (sensorineural). Vertigo is usually the prominent symptom
• A sensation of aural fullness or pressure is now recognised as being common
• Other features include nystagmus and a positive Romberg test (patient can’t stand-alone when
eyes closed and feet together)
• Episodes last minutes to hours
349
what is the natural history of disease of menieres disease?
Natural history
• Symptoms resolve in the majority of patients after 5-10 years
• Some patients may be left with hearing loss
• Psychological distress is common
350
what is the management of menieres disease?
Management
• ENT assessment is required to confirm the diagnosis
• Patients should inform the DVLA. The current advice is to cease driving until satisfactory
control of symptoms is achieved
• Acute attacks: buccal or intramuscular prochlorperazine. Admission is sometimes required
• Prevention: betahistine may be of benefit
351
describe rinnes test
Rinne's test
• Tuning fork is placed over mastoid process, followed by repositioning just over external acoustic meatus
• Air conduction (AC) is normally better than bone conduction (BC)
• If BC > AC then conductive deafness
352
describe webers test
Weber's test
• Tuning fork is placed over middle of forehead, patient is asked which side is loudest
• In unilateral sensorineural deafness, sound is localised to the 'good' side
• In unilateral conductive deafness, sound is localised to the 'bad' side
353
```
what is the condition: tinnitus with
Onset is usually at 20-40 years
Conductive deafness
Tinnitus
Normal tympanic membrane* Positive family history
```
otosclerosis
354
what is the condition: tinnitus with
Hearing loss, vertigo, tinnitus
Absent corneal reflex is important sign
Associated with neurofibromatosis type 2
acoustic neuroma
355
what drugs can cause tinnitus?
Aspirin
Aminoglycosides
Loop diuretics
Quinine
356
what is ramsay hunt syndrome?
Ramsay Hunt syndrome: (herpes zoster oticus) is caused by the reactivation of the varicella zoster virus in the geniculate ganglion of the seventh cranial nerve.
357
what are the features of ramsay hunt syndrome?
Features
• Auricular pain is often the first feature
• Facial nerve palsy
• Vesicular rash around the ear
• Other features include vertigo and tinnitus
358
what is the management of ramsay hunt syndrome?
Management
| • oral aciclovir and corticosteroids are usually given
359
what are management options for hyperhydrosis?
Management options include
• Topical aluminium chloride preparations are first-line. Main side effect is skin irritation
• Iontophoresis: particularly useful for patients with palmar, plantar and axillary hyperhidrosis
• Botulinum toxin: currently licensed for axillary symptoms
• Surgery: e.g. endoscopic transthoracic sympathectomy. Patients should be made aware of the
risk of compensatory sweating
360
describe the features of BPPV?
Features
• Vertigo triggered by change in head position (e.g. Rolling over in bed or gazing upwards)
• May be associated with nausea
• Each episode typically lasts 10-20 seconds
• Positive halpike manoeuvre
361
what is the management of BPPV?
BPPV has a good prognosis and usually resolves spontaneously after a few weeks to months. Symptomatic relief may be gained by:
• Epley maneuvre (successful in around 80% of cases)
362
what are 7 indications for plasma exchange?
* ANCA positive vasculitis e.g. Wegener's, Churg-Strauss
* Cryoglobulinemia
* Goodpasture's syndrome
* Guillain-Barre syndrome
* Hyperviscosity syndrome e.g. Secondary to myeloma
* Myasthenia gravis
* TTP/HUS
363
what are causes of reflex syncope?
```
Reflex syncope (neurally mediated)
• Vasovagal: triggeres; emotion, pain, stress or orthostatic factors. Often referred to as 'fainting'
• Situational: cough, micturition, gastrointestinal
• Carotid sinus syncope
```
364
what is carotid sinus hypersensitivity??
Carotid sinus hypersensitivity (CSH): diagnosis is only made after ischemic heart disease or rhythm disturbance have been excluded. CSH may be predominantly cardioinhibitory (bradycardia), vasodilatory (hypotension), or a mixture of the two. Cardioinhibitory is usually managed with insertion of a dual-chamber pacemaker, and vasodilator is managed with support stockings, fludrocortisone and midodrine.
365
what are causes of orthostatic syncope?
Orthostatic syncope
• Primary autonomic failure: Parkinson's disease, Lewy body dementia
• Secondary autonomic failure: e.g. Diabetic neuropathy, amyloidosis, uraemia
• Drug-induced: diuretics, alcohol, vasodilators
• Volume depletion: hemorrhage, diarrhoea
366
what are causes of cardiac syncope?
Cardiac syncope
• Arrhythmias: bradycardias (sinus node dysfunction, AV conduction disorders) or tachycardias (supraventricular, ventricular)
• Structural: valvular, myocardial infarction, hypertrophic obstructive cardimyopathy
• Others: pulmonary embolism
367
what is Post Cranial Irradiation Somnolence Syndrome?
Features:
• Excessive somnolence
• Lethargy and clumsiness
• Tends to occur around 11-21 or 31-35 days after high dose cranial radiotherapy.
• No focal cause is identified and it is postulated that the condition may occur due to post
irradiation demyelination.
• No specific therapy is required, some case reports suggest that corticosteroids may be of use but
the evidence is not firmly established.
368
how to differentiate post cranial irradiation somnolence syndrome with cerebral mets or cerebral oedema?
To differentiate it from cerebral mets or cerebral edema
| There should be no new focal neurological signs in post irradiation SS.
MRCP part 1
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