Phase 3a - Arun&Erin > Neuro > Flashcards

Neuro Flashcards

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1
Q

What is a tension headache?

A

Typically cause a mild ache or pressure in a band-like pattern around the head.

Develops and resolves gradually

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2
Q

What 5 things are tension headaches associated with?

A
  • Stress
  • Depression
  • Alcohol
  • Skipping meals
  • Dehydration
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3
Q

What is the management of tension headaches?

A
  • Simple analgesia (ibuprofen/paracetamol)
  • Amitriptyline: 1st line for chronic/frequent tension headaches
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4
Q

What are 5 red flags associated with headache? (10)

A
  • Fever, photophobia, neck stiffness (meningitis, encephalitis)
  • New neurological symptoms (haemorrhage/tumours)
  • Visual disturbance (GCA, glaucoma, tumours)
  • Sudden onset occipital headache (SAH)
  • Worse on coughing or straining (raised intracranial pressure)
  • Postural, worse on standing, lying, bending over (raised IP)
  • Vomiting (raised IP, carbon monoxide poisoning)
  • Hx of trauma (intracranial haemorrhage)
  • Hx of cancer (brain metastasis)
  • Pregnancy (pre-eclampsia)
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5
Q

What is trigeminal neuralgia?

A

Causes sudden intense shooting facial pain along distribution of trigeminal nerve
May be triggered by touch, eating, talking, shaving, cold

  • ophthalmic
  • maxillary
  • mandibular

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6
Q

What is the management of trigeminal neuralgia?

A

Carbamazepine: 1st line

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7
Q

What is a cluster headache?

A

Severe unilateral headaches, usually orbital, that come in clusters of attacks then disappear for extended periods

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8
Q

Describe a typical patient who suffers from cluster headaches and their triggers

A

30-50 year old male smoker
Triggers: alcohol, strong smells, exercise

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9
Q

Name 5 symptoms of cluster headaches

A
  • Red, swollen, watering eye
  • Miosis (pupil constriction)
  • Ptosis (eyelid drooping)
  • Nasal discharge
  • Facial sweating

Typically unilateral

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10
Q

What is the management of an acute attack of cluster headaches?

A

Triptans (subcutaneous/intranasal sumatriptan)
High flow 100% oxygen

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11
Q

What is the prophylaxis management of cluster headaches?

A

Verapamil
Other options: Occipital nerve block, Prednisolone, Lithium

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12
Q

What is temporal arteritis?

A

Giant Cell Arteritis is a type of systemic vasculitis affecting medium and large arteries

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13
Q

What is the key complication of temporal arteritis?

A

Vision loss

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14
Q

Name 4 presenting symptoms of temporal arteritis

A
  • Unilateral headache: severe around temple/forehead
  • Scalp tenderness
  • Jaw claudication
  • Blurred/double vision (If left untreated - vision loss)
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15
Q

Name 3 associated features of temporal arteritis

A
  • Polymyalgia rheumatica Sx: shoulder/pelvic girdle pain & stiffness
  • Systemic Sx: wt loss, fatigue, fever
  • Carpel tunnel syndrome
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16
Q

Name 4 things a diagnosis of temporal arteritis is based on

A
  • Clinical presentation
  • Raised inflammatory markers (ESR)
  • Temporal artery biopsy (shows multinucleated giant cells)
  • Duplex ultrasound (shows hypoechoic ‘halo’ sign and stenosis of temporal artery)
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17
Q

What is the first line treatment of temporal arteritis?

A

Steroids started immediately to reduce the risk of vision loss:
* Prednisolone with no visual Sx or jaw claudication
* Methylprednisolone with visual Sx or jaw claudication

Once diagnosis confirmed, steroid dose is slowly weaned over 1-2 years

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18
Q

Name 3 other medications used to manage temporal arteritis

A
  • Aspirin: decreases vision loss/strokes
  • PPI (omeprazole): gastroprotection while on steroids
  • Bisphosphonates & calcium/vit D: bone protection while on steroids
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19
Q

What are the four main types of migraine?

A
  • Migraine without aura
  • Migraine with aura
  • Silent migraine
  • Hemiplegic migraine
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20
Q

What are the 5 stages of a migraine?

A
  1. Premonitory/Prodromal: can begin several days before the headache)
  2. Aura
  3. Headache
  4. Resolution
  5. Postdromal/Recovery
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21
Q

Name 8 typical features of a migraine headache

A
  • Usually unilateral, but can be bilateral
  • Moderate-severe intensity
  • Pounding/throbbing
  • Photophobia
  • Phonophobia
  • Osmophobia
  • Aura
  • N+V
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22
Q

What are the symptoms of an aura?

A

Can affect vision, sensation or language
* Visual Sx: sparks in vision, blurred, lines across vision, loss of visual fields
* Sensation: tingling, numbness
* Language: dysphasia

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23
Q

What are the main features of a hemiplegic migraine?

A
  • Hemiplegia (unilateral limb weakness)
  • Ataxia (loss of coordination)
  • Impaired consciousness

Can mimic a stroke/TIA, so must exclude these

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24
Q

Name 8 triggers of a migraine

A
  • Stress
  • Bright lights
  • Strong smells
  • Foods: chocolate, cheese, caffeine
  • Dehydration
  • Menstruation
  • Disrupted sleep
  • Trauma
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25
Name 4 medications used to treat an acute attack of a migraine
* **NSAIDs** (ibuprofen / naproxen) * **Paracetamol** * **Triptans** (sumatriptan) * **Antiemetics** for vomiting (metoclopramide / prochlorperazine) ## Footnote Non-medical management: retreat to a dark, quiet room, sleeping
26
What is the role of triptans in the management of migraines?
Taken as soon as migraine headache starts, used to **halt attack** If it doesn't work, a second dose should **not** be taken for the same attack They bind to & stimulate **serotonin receptors** to: * inhibit transmission of pain signals * inhibit release of inflammatory neuropeptides * cause cranial vasoconstriction
27
Name 5 prophylactic medications used to treat a migraine?
* **Propanolol** * **Amitriptyline** * **Topiramate** (teratogenic) * **Pizotifen** * **Sodium valproate**
28
What is the management of menstrual migraines?
**Prophylactic triptans**: e.g. zolmitriptan/frovatriptan Sx tend to occur 2 days before until 3 days after start of menstruation
29
Name the 2 types of cerebrovascular accidents
* Ischaemic stroke * Haemorrhagic stroke
30
Name 4 things that can disrupt the blood supply to the brain
* Thrombus/embolus * Atherosclerosis * Shock * Vasculitis
31
What is a TIA?
Temporary (**< 24hrs**) neurological dysfunction caused by **ischaemia without infarction**
32
Name 6 symptoms of stroke
* Limb weakness * Facial weakness * Dysphasia * Visual field defects * Sensory loss * Ataxia
33
Name 5 risk factors for stroke
* Previous stroke or TIA * **Atrial Fibrillation** * **Carotid artery stenosis** * Hypertension * Diabetes * Raised cholesterol * Family Hx * Smoking * Obesity
34
What is the FAST tool for stroke?
**F**ace **A**rm **S**peech **T**ime
35
Tx: What is the management of a TIA?
* **Aspirin 300mg daily** * Referral for specialist assessment * **Diffusion-weighted MRI scan**
36
Tx: What is the immediate management of a stroke?
* **Immediate CT brain** (to exclude haemorrhage) * **Aspirin 300mg daily** for 2 weeks * Admission to a **specialist stroke centre**
37
Tx: What management is considered once haemorrhage is excluded?
**Thrombolysis with alteplase** * Alteplase is a **tissue plasminogen activator** that rapidly breaks down clots * It may be given within **4.5hrs** of symptom onset **Thrombectomy** * Considered in patients with a confirmed blockage of the **proximal anterior/posterior circulation** * offered if symtoms started within 6 hours ago * It may be considered within **24hrs** of symptom onset and alongside **IV thrombolysis** if there is visualised as able brain tissue
38
Ix: Name 2 things that are always investigated for in patients who have had a TIA or stroke
* **Carotid artery stenosis**: carotid imaging * **Atrial fibrillation**: ECG ## Footnote Anticoagulation is initiated for AF (after excluding haemorrhage and finishing 2 weeks of aspirin) Surgical interventions are considered for significant CAS (carotid endarterectomy or angioplasty and stenting)
39
Tx: What is the secondary prevention of a stroke?
* **Clopidogrel** 75mg once daily if contraindicated then aspirin 75mg * **Atorvastatin** 20-80mg (not started immediately, usually delayed by at least 48hrs) * **Blood pressure** and **diabetes** control * Addressing **modifiable risk factors** (smoking, obesity, exercise)
40
What is a seizure?
Transient episodes of **abnormal electrical activity** in the brain
41
Name 5 types of seizures
* Generalised **tonic-clonic** * **Partial** / **focal** * **Myoclonic** * **Tonic** * **Atonic**
42
What is a generalised tonic-clonic seizure?
* **Tonic** - muscle tensing, followed by **clonic** - muscle jerking * Associated with a **complete loss of consciousness** * Before seizure pt may experience **aura** * May be **tongue biting**, **incontinence**, groaning and irregular breathing * After seizure: **post-ictal period** - pt is confused, tired, irritable, low
43
What is a partial/focal seizure?
* Occurs in an isolated brain area, often in **temporal lobes** * Affect **hearing**, **speech**, **memory**, **emotions** * Pt remains **awake** * Simple: pt remains aware * Complex: pt loses awareness
44
Name 4 symptoms of a partial seizure
* Deja vu * Strange smells/tastes/sight/sound sensations * Unusual emotions * Abnormal behaviours
45
How do myoclonic seizures present?
* Sudden, brief muscle contractions * Pt remains awake
46
How do tonic seizures present?
* Sudden onset of **increased muscle tone** - entire body stiffens * Only last a few seconds - a few minutes
47
How do atonic seizures present?
* Cause 'drop attacks' * Involve sudden loss of muscle tone, often resulting in a fall * Pts usually aware during episodes * Often begin in childhood * May be indicative of **Lennox-Gastaut syndrome**
48
How do absence seizures present?
* Usually seen in **children** * Pt becomes **blank**, stares into space, then abruptly returns to normal * Unaware of surroundings and do not respond during episode * Typically last 10-20 secs
49
What is epilepsy?
Recurrent (**2 or more**) unprovoked epileptic seizures occuring **> 24hrs** apart
50
Name 2 main investigations for epilepsy
* **EEG** (electroencephalogram) * **MRI brain**: structural pathology
51
Name 4 additional investigations to rule out other causes of seizures
* ECG * Serum electrolytes (Na, K, Ca, Mg) * Blood glucose (hypoglycaemia, diabetes) * Blood & urine cultures, lumbar puncture (sepsis, encephalitis, meningitis)
52
What is the treatment for generalised tonic-clonic seizures?
**Lamotrigine**/ **Levetiracetam**
53
What is the treatment for focal seizures?
**Lamotrigine**/ **Levetiracetam** 2nd = carbamazepine
54
What is the treatment for myoclonic seizures?
**Levetiracetam** 2nd = lamotrigine
55
What is the treatment for tonic & atonic seizures?
**Lamotrigine** 2nd = clobazam/topiramate
56
What is the treatment for absence seizures?
**Ethosuximide** 2nd = lamotrigine/levetiracetam
57
How does sodium valproate work?
Increases activity of **GABA**: has a calming effect on the brain
58
Name 5 side effects of sodium valproate
* **Teratogenic** * Liver damage / Hepatitis * Hair loss * Tremor * Reduced fertility
59
What is Status Epilepticus?
Either: * A seizure lasting **more than 5 minutes** * Multiple seizures **without regaining consciousness** in between
60
What is the management of status epilepticus?
ABCDE approach: * Secure airway * Give high-conc oxygen * Check blood glucose levels * Gain IV access (insert a cannula)
61
What is the medical treatment of status epilepticus?
* 1st line: **Benzodiazepine** (repeated after 5-10 mins if seizure continues) * 2nd line (after 2 doses of benzos): **IV levetiracetam**, **phenytoin** or **sodium valproate** * 3rd line: **phenobarbital** or **general anaesthesia**
62
What is NEAD?
**Non-epileptic attack disorder** * Seizures without a physical cause * May be caused by brain dealing with **overwhelming stress** by 'shutting down' * Affects people with dissociative disorders
63
Name 4 features of a dissociative seizure (NEAD)?
* Convulsions of arms/legs/head/body (uni- or bilateral) * Lose control of bladder/bowels * Bite your tongue * Go blank/stare
64
What is narcolepsy?
Condition where brain is unable to **regulate sleeping and waking patterns** normally
65
Name 5 features of narcolepsy
* **Excessive daytime sleepiness** * **Sleep attacks**: falling asleep suddenly without warning * **Cataplexy**: temporary loss of muscle control resulting in weakness/possible collapse * **Sleep paralysis**: temporary inability to move/speak when waking up or falling asleep * **Excessive dreaming** / waking up in the night
66
What is the management of narcolepsy?
* Improve sleeping habits * Take frequent, brief naps in the day * Keep to a strict bedtime routine * Inform DVLA and don't drive
67
What is cataplexy?
A condition where **strong emotion** or **laughter** causes sudden brief **muscle weakness**, often resulting in **collapse**
68
What is the causative organism of shingles?
**Varicella zoster virus** (VZV)
69
What is the aetiology of shingles?
* **Primary infection** with **VZV** manifests as **chickenpox** (varicella) * Following resolution, virus establishes **latent infection** within **sensory nerve ganglions** * **Reactivation** of these dormant viruses results in **shingles** (herpes zoster) * Virus spreads through **affected sensory nerve**, causing **neuronal damage** and causing a skin **rash** to develop in the corresponding dermatone
70
What are the signs of shingles?
* **Tingling/ pain** in an area of skin * **Headache**/ feeling generally unwell * **Rash** appears a few days later anywhere on body
71
Describe the shingles rash
* **Blotches** on skin, on **one** side of the body * Blotches become **itchy blisters** that **ooze fluid** - blisters **dry out and scab** a few days later * Rash may be around eye - **ophthalmic** division of trigeminal nerve
72
What is the treatment of shingles?
Mild rash - Tx not always needed **Antiviral tablets** within 72 hours if: * Weakened immune system * Rash/pain is moderate/severe * Rash is affecting other areas of your body apart from chest, tummy, back
73
Name an antiviral that can be used to treat shingles
**Acyclovir**
74
How long does shingles last?
Can take up to 4 weeks for rash to heal
75
What is Wernicke-Korsakoff syndrome?
**Thiamine** deficiency due to excessive alcohol consumption. * thiamine is poorly absorbed in the presence of alcohol
76
What are the 2 stages of Wernicke-Korsakoff syndrome?
* **Wernicke's encephalopathy**: brain inflammation * **Korsakoff's syndrome**: long-term condition due to untreated Wernicke's encephalopathy
77
Name 3 symptoms of Wernicke's encephalopathy
* **Confusion** * **opthalmoplegia** * **Ataxia**: difficulty with coordinated movements
78
Name 3 symptoms of Korsakoff syndrome
* **Memory impairment**: confabulation - invented memory * Difficulty understanding **new info**/ learning **new skills** * **Personality changes**: apathetic, very talkative etc.. * Problems with **concentration**, **planning**, **making decisions**, **solving problems**
79
What is the treatment of Wernicke-Korsakoff syndrome?
* **IV thiamine** * Stop drinking alcohol
80
What is Huntington's disease?
* **Autosomal dominant** condition that causes **progressive neurological dysfunction**
81
What is the genetic mutation in Huntington's disease?
* **Trinucleotide repeat disorder** * Genetic mutation in the **HTT gene** on **chromosome 4** which codes for the **Huntingtin** (**HTT**) protein
82
What age do Huntington's symptoms begin?
**30 - 50** years old
83
Definition: Genetic anticipation
Successive generations have more **repeats** in the gene resulting in: * **Earlier age of onset** * **Increased severity of disease** ## Footnote Feature of **trinucleotide repeat disorders**
84
What is the presentation of Huntington's disease?
**Cognitive**/**psychiatric**/**mood problems**, followed by movement disorders: * **Chorea** (involuntary random abnormal body movements) * **Dystonia** (abnormal tone) * **Rigidity** (increased resistance to passive joint movement) * **Eye movement disorders** * **Dysarthria** (speech difficulties) * **Dysphagia** (swallowing difficulties)
85
How is a diagnosis of Huntinton's disease made?
**Genetic testing**
86
What is the management of Huntington's disease?
No cure * **Physio** * **Speech & language therapy** * **Tetrabenazine** (for chorea Sx) * **Antidepressants** * End of life care * Advanced directives
87
What is the prognosis of Huntington's disease?
* Life expectency is ~ **10-20** years after onset of Sx * As disease progresses, Pt becomes more frail/ susceptible to illnesses * Death often due to **aspiration pneumonia** or suicide
88
Definition: Parkinson's disease
Condition where there is a **progressive reduction** in **dopamine** in the **basal ganglia** leading to disorders of movement Sx are **asymmetrical**
89
What is the classic triad of Parkinson's disease features?
* **Resting tremor**: worse at rest * **Rigidity**: resisting passive movement * **Bradykinesia**: slow movement
90
Name 5 clinical features of Parkinson's disease (8)
* **Pin-rolling tremor** * **Cogwheel rigidity** * Bradykinesia: **shuffling gait**, micrographia, festinating gait, difficulty initiating movement, difficulty turning around, reduced facial movements/expressions * **Depression** * **Insomnia** * **Anosmia** * Postural instability * Cognitive impairment/ memory problems
91
Tx: What are the 4 treatment options for Parkinson's disease?
* **Levodopa + peripheral decarboxylase inhibitor** (e.g. carbidopa, benserazide) * COMT inhibitors * Dopamine agonists * Monoamine oxidase-B inhibitors
92
What is Levodopa?
* **Synthetic dopamine** * Combined with a **peripheral decarboxylase inhibitor** which stops it from being **metabolised** before it reaches the brain
93
Name 2 combination drugs to treat Parkinson's
* **Co-beneldopa** (levodopa + benserazide) * **Co-careldopa** (levodopa + carbidopa)
94
What is the main SE of Levodopa and it's Tx?
SE: **Dyskinesia** Tx: **Amantadine** (glutamate antagonist)
95
Definition: Dyskinesia
Abnormal movements associated with excessive motor activity: * **Dystonia**: excessive muscle contraction leads to abnormal postures/exaggerated movements * **Chorea**: abnormal involuntary movements, jerking * **Athetosis**: involuntary twisting/writhing movements, usually in fingers/hands/feet
96
What are **COMT inhibitors**?
e.g. entacapone * Inhibits **catechol-o-methyltransferase** which metabolises levodopa * Taken with Levodopa to slow breakdown in the brain
97
What are **dopamine agonists**?
* Mimic action of **dopamine** in the **basal ganglia**, stimulating **dopamine receptors**
98
Name 3 dopamine agonists
* **Bromocriptine** * **Cabergoline** * **Pergolide**
99
What is the main SE of prolonged dopamine agonist use?
**Pulmonary fibrosis**
100
What are **monoamine oxidase-B inhibitors**?
* **Block** the action of **monoamine oxidase-B enzymes** which break down the neurotransmitter **dopamine** ## Footnote Monoamine oxidase enzymes break down other neurotransmitters such as serotonin and adrenaline, but monoamine oxidase-B is specific to dopamine
101
Name 2 examples of monoamine oxidase-B inhibitors
* **Selegiline** * **Rasagiline**
102
What are the 4 types of dementias from most prevalent to least?
* **Alzheimer's disease** * **Vascular dementia** * **Dementia with Lewy bodies** (DLB) * **Fronto-temporal dementia**
103
Name 5 **irreversible** causes of dementia
* **Neurodegenerative**: Alzheimer's, F-T dementia, DLB, Parkinson's, Huntington's * **Infections**: HIV, encephalitis, syphilis * **Toxins**: alcohol, barbiturates, benzodiazepines * **Vascular**: vascular dementia, CVD * **Head trauma**
104
Name 3 **reversible** causes of dementia
* **Neurological**: normal pressure hydrocephalus, intracranial tumours, CSH * **Vitamin deficiencies**: B12, folic acid, thiamine, nicotinic acid * **Endocrine**: Cushing's, hypothyroidism
105
What is the pathophysiology of Alzheimer's disease?
* Degeneration of **cholinergic neurons** in the **nucleus basalis of Meynert** leading to **acetylcholine deficiency** due to accumulation of beta amyloid plaques
106
What are 2 **microscopic** physiological changes seen in Alzheimer's disease?
* **Neurofibrillary tangles** (intracellularly) * **Beta-amyloid plaque formation** (extracellularly) ## Footnote These are pathological lesions progressively distributed around the brain
107
What are 3 **macroscopic** physiological changes seen in Alzheimer's disease?
* **Cortical atrophy** (commonly hippocampus) * **Widened sulci** * **Enlarged ventricles**
108
Name 7 risk factors for Alzheimer's disease
* Advancing age * Family Hx * Genetics * Down's syndrome * Low IQ * CVD * Vascular RFs: stroke/MI, smoking, HTN, DM, high cholesterol
109
Clinical features: Name 3 symptoms in **early stages** of AD
* Memory lapses * Difficulty finding words * Forgetting names of people/places
110
Clinical features: Name 4 symptoms during **disease progression** of AD
* Apraxia * Agnosia * Confusion * Language problems * Impairment of executive functions
111
Clinical features: Name 7 symptoms in **later stages** of AD
* Disorientation to time/place * Wandering * Apathy * Incontinence * Eating problems * Depression * Agitation
112
Aetiology: What is the cause of **vascular dementia**?
**Cerebrovascular disease** due to: * stroke * multi-infarcts * chronic changes in small vessels (arteriosclerosis)
113
Name 6 clinical features of vascular dementia
* **Stepwise** rather than continuous deterioration * **Memory loss** * **Emotional** and **personality changes** * **Confusion** * **Neurological signs/Sx** * On examination --> **focal neurology** (UMN signs) and signs of **CVD**
114
Ix: Name 10 investigations for dementia
**Blood tests**: FBC,CRP,U&E,calcium,LFT,glucose,vit B12 & folate,TFT Non-routine Ix: * Urine dipstick * Chest Xray * Syphilis serology & HIV testing * CT/ MRI/ SPECT (to differentiate between AD, VascD and F-TD) * ECG * EEG * Lumbar puncture * Genetic tests * Cognitive assessment
115
DDx: Name 5 differentials for dementia (9)
* Normal **ageing**/ mild cognitive impairment * **Delirium** * **Trauma**: stroke, hypoxic, brain injury * **Depression**: poor concentration/impaired memory common in depression in the elderly * Late onset **schizophrenia** * **Amnesic syndrome**: severe disruption in memory with minimal deterioration in cognitive function * **Learning disability** * **Substance misuse** * **Drug side effects**: opiate, benzodiazepine
116
After a diagnosis of dementia, what are patients legally obliged to do?
**Contact DVLA**
117
Tx: What are 5 non-pharmacological managements of dementia?
* **Social support** * Increasing assistance with **day-to-day activities** * **Education** * **Community** dementia teams & services * **Home nursing** and **personal care**
118
What are the aims of dementia treatment?
* **Promote** **independence** * **Maintain function** * **Treat** **symptoms**
119
Tx: What is the pharmacological management of dementia?
* **Acetylcholinesterase inhibitors** (mild/moderate AD) * **N-methyl-D-aspartate receptor antagonist** (moderate AD in those who are intolerant/contraindication to AChE inhibitors / severe AD) * **Antipsychotic** for challenging behaviour (risperidone) * **Antidepressant** for low mood (sertraline)
120
Tx: Name 3 AChE inhibitors
* **Donepezil** * **Galantamine** * **Rivastigmine**
121
Tx: Name an NMDA receptor antagonist
**Memantine**
122
What is normal pressure hydrocephalus?
**CSF** (cerebrospinal fluid) building up abnormally within **brain** and **spinal cord**
123
What is the triad of symptoms in normal pressure hydrocephalus?
* Dementia * Gait disturbance * Urinary incontinence
124
What are the 2 causes of hydrocephalus?
* **Over-production** of CSF * Problem with **draining**/ **absorbing** CSF
125
What is normal CSF physiology?
* **Four ventricles**: two lateral, 3rd ventricle, 4th ventricle * Ventricles contain **CSF**: provides cushion for brain tissue * CSF created in four **choroid plexuses** (one in each ventricle) and by walls of the ventricles * CSF absorbed into venous system by **arachnoid granulations**
126
What is the most common cause of hydrocephalus?
**Aqueductal stenosis** * **Cerebral aqueduct** connecting 3rd + 4th ventricle is **stenosed** * Blocks normal CSF flow * CSF **build up** in lateral & 3rd ventricles * Insufficient CSF drainage
127
Name 4 causes of hydrocephalus
* **Aqueductal stenosis** * **Arachnoid cysts**: can block outflow of CSF * **Arnold-Chiari malformation**: cerebellum herniates down through foramen magnum, blocking outflow of CSF * **Chromosomal abnormalities** / **congenital malformations**
128
Name 5 signs of hydrocephalus in children
* **Enlarged head circumference**: outward pressure on cranial bones (babies) * **Bulging** anterior fontanelle * Poor **feeding** & vomiting * Poor **tone** * **Sleepiness**
129
What is the treatment of hydrocephalus?
**Ventriculoperitoneal Shunt** * Drains CSF from ventricles into peritoneal cavity (space & easily reabsorbed) * Valve can regulate amount of CSF that is drained
130
Name 5 complications of a VP shunt
* Infection * Blockage * Excessive drainage * Intraventricular haemorrhage during shunt related surgery * Outgrowing them (need replacing every 2 years as child grows)
131
Name 4 causes of increased pressure in the intracranial space
* **Brain tumours** * **Intracranial haemorrhage** * **Idiopathic intracranial hypertension** * **Abscess / infection** * **meningitis/encephalitis**
132
What concerning features indicate **intracranial hypertension** in patients with a headache?
* **Constant** headache * **Nocturnal** * Worse on **waking** * Worse on **coughing**, **straining**, **bending forward** * **Vomiting** * **Papilloedema** on fundoscopy
133
What are 5 other presenting features of **raised intracranial hypertension** (not regarding headache)?
* Altered **mental state** * **Visual field** defects * **Seizures** * Unilateral **ptosis** * **3rd** & **6th** nerve palsies
134
What are benign and malignant brain tumours called?
* Benign - **meningiomas** * Malignant - **glioblastomas**
135
What is the presentation of brain tumours?
**Progressive** focal neurological Sx (depending on location of lesion) Sx of **raised ICP**: * Headache * N+V * Papilloedema * Coma
136
What is papilloedema?
Swelling of the **optic disc** secondary to **raised intracranial pressure**
137
What are gliomas?
Tumours of the **glial cells** in the brain/spinal cord * Glial cells surround **neurones** * Include **astrocytes**, **oligodendrocytes**, **ependymal cells**
138
What are the 3 main types of glioma? (from most to least malignant)
* **Astrocytoma** (most common form is glioblastoma) * **Oligodendroglioma** * **Ependymoma**
139
What are meningiomas?
Tumours growing from the cells of the **meninges** * Usually benign, but take up space * **Mass effect** can lead to raised intracranial pressure & neurological Sx
140
Name 4 cancers that metastasise to the brain
* Lung * Breast * Renal cell carcinoma * Menaloma
141
What is the defect caused by pituitary tumours?
Can press on the **optic chiasm** causing **bitemporal hemianopia** (loss of outer half of visual fields in both eyes)
142
Name 4 hormone related diseases caused by pituitary tumours
* **Acromegaly** (excessive GH) * **Hyperprolactinaemia** (excessive prolactin) * **Cushing's disease** (excessive ACTH & cortisol) * **Thyrotoxicosis** (excessive TSH & thyroid hormone)
143
What are 4 ways to manage pituitary tumours?
* **Trans-sphenoidal surgery** * **Radiotherapy** * **Bromocriptine** (to block excess prolactin) * **Somatostatin analogues** (to block excess GH): e.g. octreotide
144
What are acoustic neuromas?
Benign tumours of the **Schwann cells** that surround the **auditory nerve** * Occur at the **cerebellopontine angle** * Usually **unilateral**
145
Name 5 symptoms of acoustic neuroma ## Footnote Gradual onset
* Unilateral sensorineural **hearing loss** * Unilateral **tinnitus** * **Dizziness** / imbalance * Sensation of **fullness** in ear * **Facial nerve palsy**
146
What is the management of acoustic neuroma?
* **Conservative** + monitoring * **Surgery** to remove tumour * **Radiotherapy** to reduce growth
147
Name 2 investigations for brain tumours
* **MRI scan** - 1st line * **Biopsy** - gives definitive histological diagnosis
148
What are the 4 main options for treating a brain tumour?
* Surgery * Chemotherapy * Radiotherapy * Palliative care
149
What is multiple sclerosis?
* Chronic, progressive **autoimmune** condition * Involves **demyelination** in **CNS** * **Immune system** attacks **myelin sheath** of the neurones
150
Who typically presents with MS?
* Younger adults * Women
151
Describe the pathophysiology of MS
* **Inflammation** and immune cell infiltration cause damage to the **myelin** * This affects the **electrical signals** moving along neurones * In early disease, **re-myelination** can occur = Sx resolve * In later stages, re-myelination is incomplete = Sx gradually become more permanent
152
How must the lesions in MS present to be diagnosed with MS
**Disseminated in time and space** * Vary in location = affected sites & Sx change over time
153
Name 5 possible causes of MS
* Genetics * EBV * Low vit D * Smoking * Obesity
154
Describe the onset and duration of MS Sx
* Sx progress over** > 24hrs** * Sx last **days - weeks** at first presentation then improve
155
What is optic neuritis?
* Most common presentation of MS * **Demyelination** of **optic nerve** * Presents with **unilateral reduced vision** developing over hours - days
156
What are 4 key features of optic neuritis?
* **Central scotoma** - enlarged central blind spot * **Pain** with eye movement * Impaired **colour vision** * **Relative afferent pupillary defect** - pupil in affected eye constricts **more** when shining a light in the **contralateral** eye
157
Name 6 causes of optic neuritis other than MS
* Sarcoidosis * SLE * Syphilis * Measles/mumps * Neuromyelitis optica * Lyme disease
158
How is optic neuritis treated?
* High dose steroids * Iv IG
159
Name 3 ophthalmic Sx of MS
* Optic neuritis * Nystagmus * Diplopia
160
Name 4 Sx of focal **weakness** in MS
* Incontinence * Horner syndrome * Facial nerve palsy * Limb paralysis
161
Name 5 focal **sensory** Sx of MS
* Trigeminal neuralgia * Numbness * Paraesthesia * Lhermitte's sign - electric shock sensation travelling down spine when flexing neck * Sensory ataxia - due to loss of proprioception
162
Name 4 cerebellar Sx of MS
* Loss of balance / cerebellar ataxia * nystagmus * Dysarthria * Tremor
163
What are the 4 main classifications of MS?
1. **Clinically Isolated Syndrome** - unexplained first episode of neurological dysfunction 2. **Relapsing-remitting MS** - unpredictable attacks of neurological dysfunction followed by recovery 3. **Secondary progressive MS** - used to be RRMS but now there is progressive worsening of Sx with incomplete remissions 4. **Primary progressive MS** - steady, progressive worsening of disease severity without remission
164
How is a diagnosis of MS made?
* Clinical picture & Sx * **MRI scan with contrast** - shows lesions disseminated in time and space * **Lumbar puncture** - detects **oligoclonal bands** in the CSF
165
What is the treatment of an acute attack of MS?
**High dose steroids** - methylprednisolone
166
What is the long term treatment of MS?
* **Disease-modifying** agents - **beta-IFN** (decrease relapses) * **Manage Sx**: exercise, amitriptyline/gabapentin for neuropathic pain, SSRIs for depression etc..
167
What is Guillain-Barre syndrome?
**Acute paralytic polyneuropathy** that affects the **PNS**. Causes acute, symmetrical **ascending** weakness (can cause sensory Sx)
168
What organism are GBS particularly associated with?
* **Campylobacter jejuni** * **CMV** (cytomegalovirus) * **EBV** (Epstein-Barr virus)
169
What is the pathophysiology of GBS?
**Molecular mimicry** * **B cells** create antibodies against the **antigens** on the triggering **pathogen** * These antibodies also match proteins on the **peripheral neurones** * May target proteins on **myelin sheath** or **nerve axon** itself
170
Describe the symptom timeline and presentation of GBS
* Sx start: within **4 weeks** of triggering infection * Begin in **feet** and progress **upward** * Sx peak: **2-4 weeks** * Recovery period: months to years
171
Name 4 symptoms of GBS
* **Symmetrical ascending** weakness * **Reduced** reflexes * **Peripheral** loss of sensation * **Neuropathic pain** Autonomic dysfunction --> urinary retention, ileus, arrhythmias May progress to cranial nerves --> facial weakness
172
Name 2 investigations for GBS
* **Nerve conduction studies**: reduced signal through nerves * **Lumbar puncture**: CSF shows raised protein & normal cell count
173
What is the management of GBS?
* **Supportive care**: IV fluids, HR control * **VTE prophylaxis**: PE leading cause of death * **IV IG** - 1st line * **Plasmapheresis** (plasma exchange) Severe cases with **resp failure**: intubation/ventilation
174
What is motor neuron disease?
Progressive neurodegenerative disease where **motor** neurones stop working (sensory neurons aren't affected = no sensory Sx)
175
What are the 4 types of motor neuron disease?
1. **Amyotrophic lateral sclerosis**- MC 2. **Progressive bulbar palsy** 3. Progressive muscular atrophy 4. Primary lateral sclerosis
176
What is the typical presentation of MND?
* **Progressive weakness** of muscles throughout body: limbs, trunk, face, speech * **Upper limb weakness** noticed first: hand weakness/loss of dexterity * **Increased fatigue** when exercising * **Clumsiness**, tripping over, dropping things * **Dysarthria**: slurred speech * **mix of LMN and UMN signs**
177
What are UMN signs? (5)
* Hyp**er**tonia * Hyp**er**reflexia * **Spastic** gait * Ankle **clonus** * Jaw **clenching** / exaggerated jaw jerk * +ve babinski sign**
178
What are LMN signs? (4)
* Hyp**o**tonia * Hyp**o**reflexia * **Fasciculations** * Muscle **atrophy** --> weakness * negative babinski sign
179
Name 2 investigations for MND
* Nerve conduction studies - normal motor conduction * electromyograph - decreased number of action potentials * MRI
180
What is the management of MND?
No cure! * **Riluzole**: slows disease progression * **Non-invasive ventilation** (NIV): when resp muscles weaken * **PEG feeding** * End of life care
181
What is the main cause of death in MND?
**Respiratory failure** (muscles of respiration affected)
182
What is the prognosis of MND?
Survival of **3-5** years after Sx onset
183
What is cerebral palsy?
Group of **permanent, non-progressive** conditions that affect **movement & coordination** resulting from damage to the brain around birth
184
Name 2 antenatal causes of cerebral palsy?
* Maternal infections * Trauma during pregnancy
185
Name 2 perinatal causes of cerebral palsy?
* Birth asphyxia / hypoxic-ischaemic injury * Pre-term birth
186
Name 3 postnatal causes of cerebral palsy?
* Meningitis * Head injury * Severe neonatal jaundice / Kernicterus
187
What are the 4 types of cerebral palsy?
* **Spastic**: UMN damage = hypertonia & reduced function * **Dyskinetic** basal ganglia damage = problems controlling muscle tone (hypertonia & hypotonia), athetoid movements, oro-motor problems * **Ataxic**: cerebellum damage = problems with coordinated movement * **Mixed**
188
Name 6 signs of cerebral palsy
* Failure to meet **milestones** * Increased/decreased **tone** (generally or in specific limbs) * Early development of **hand preference** * Problems with **coordination**, **speech** or **walking** * **Feeding**/**swallowing** problems * **Learning difficulties**
189
What does a **hemiplegic**/ **diplegic** gait indicate?
**UMN lesion**
190
What does a **broad based**/ **ataxic** gait indicate?
**Cerebellar lesion**
191
What does a **high stepping** gait indicate?
**Foot drop** or **LMN lesion**
192
What does a **waddling** gait indicate?
Pelvic muscle weakness due to **myopathy**
193
What does an **antalgic** gait indicate?
limp = **localised pain**
194
Name 4 symptoms of cerebral palsy
* **Hemiplegic/diplegic gait**: increased muscle tone & spasticity in legs, extended leg & plantar flexion of feet/toes causes leg to swing around * **UMN lesion signs** (good muscle bulk, increased tone, brisk reflexes, slightly reduced power) * **Athetoid movements**: indicate extrapyramidal (basal ganglia) involvement * **Coordination** problems: cerebellar involvement
195
What is the general management of cerebral palsy?
**MDT approach** * **Physio**: prevent muscle contractures * **OT**: help with ADLs * **SLT**:
196
What are possible medical treatments for cerebral palsy?
* **Baclofen**: muscle relaxant to manage high muscle tone/spasticity * **Botox**: relieve spasticity by blocking nerve signals that cause muscle contraction - not permanent, wears off after 3-6 months
197
Definition: Neurofibromatosis
Genetic condition that causes **neuromas** to develop throughout the NS ## Footnote 2 types: NF1 & NF2
198
What is a neuroma?
Benign nerve tumour
199
What chromosome is **neurofibromatosis type 1 gene** found on?
**chromosome 17** * codes for a **tumour suppressor protein** called **neurofibromin**
200
What is the genetic inheritance pattern of neurofibromatosis?
autosomal dominant
201
What are the features of neurofibromatosis? ## Footnote **CRABBING**
* **C**afe-au-lait spots * **R**elative with NF1 * **A**xillary / inguinal freckling * **B**ony displaysia e.g. **B**owing of a long bone / sphenoid wing dysplasia * **I**ris hamartomas (**Lisch nodules**) * **N**eurofibromas * **G**lioma of optic pathway
202
What is a neurofibroma?
* Skin-coloured * Raised nodules/papules * With smooth, regular surface ## Footnote 2 or more = significant
203
What is a plexiform neurofibroma?
Larger, irregular, complex neurofibroma containing multiple cell types that cover and protect the nerves ## Footnote a single plexiform neurofibroma is significant
204
What is the management of NF1?
Treat complications
205
What are some complications of NF1?
* **Malignant peripheral nerve sheath tumours** (MPNST) * **Gastrointestinal stromal tumour** (GIST) * migraine * epilepsy * renal artery stenosis --> HTN * learning disability * scoliosis * vision loss (secondary to optic nerve glioma) * brain tumour * spinal cord tumour
206
What chromosome is **neurofibromatosis type 2 gene** found on?
**chromosome 22** * codes for **tumour suppressor protein** called **merlin** * important in **Schwann cells** * mutations lead to **Schwannomas**
207
What is the main feature of NF2?
**acoustic neuroma** (tumour of the auditory nerve) ## Footnote surgery can be used to resect the tumours - but risk of permanent nerve damage
208
Definition: Meningitis
Inflammation of the **meninges** due to infection
209
Where is CSF contained?
In the **subarachnoid space** within the meninges
210
What are the 5 causes of bacterial meningitis?
* **Neisseria meningitidis** (meningococcus) * **Strep pneumoniae** (pneumococcus) * **H influenzae** * **Group B strep** (particularly in neonates) * **Listeria monocytogenes** (particularly in neonates)
211
What is meningococcal septicaemia?
when **meningococcus** bacterial infection is in the **bloodstream** * can cause **non-blanching rash**
212
What are the 3 most common causes of viral meningitis?
* **Enteroviruses** e.g. coxsackievirus * **Herpes simplex virus** (HSV) * **Varicella zoster virus** (VZV) ## Footnote * **Viral PCR testing** can be done on a CSF sample * **Aciclovir** is used to treat HSV & VZV
213
What are some symptoms of meningitis?
* **Fever** * **Neck stiffness** * **N+V** * **Headache** * Photophobia * Altered consciousness * Seizures Children: Non-blanching rash - **meningococcal septicaemia** Neonates: Non-specific signs
214
Name 2 special tests to look for meningeal irritation
* **Kernig's** * **Brudzinski's**
215
Describe Kernig's test
* Pt lies on back flexing one hip & knee to **90 degrees** * Slowly **straightens knee** whilst keeping hip flexed * Creates **stretch** in meninges * If meningitis - **spinal pain** or **resistance to movement**
216
Describe Brudzinski's test
* Pt lies on back * You gently lift their head & neck off bed, flexing **chin to chest** * If meningitis - this causes Pt to **involuntarily flex** hips & knees
217
Describe the **appearance** of a CSF sample in meningitis
* Bacterial - **cloudy** * Viral - **clear**
218
Describe the **protein** levels of a CSF sample in meningitis
Bacterial - **high** Viral - **mildly raised/normal**
219
Describe the **glucose** of a CSF sample in meningitis
Bacterial - **low** Viral - **normal**
220
Describe the **white cell count** of a CSF sample in meningitis
Bacterial - **high** (neutrophils) Viral - **high** (lymphocytes)
221
Describe the **bacterial culture** of a CSF sample in meningitis
Bacterial - shows **bacteria** Viral - **negative**
222
What is the management of bacterial meningitis?
Medical emergency!! * Children w/ suspected meningitis **and** non-blanching rash = urgent dose of **benzylpenicillin** * Under 3 months - **cefotaxime** + **amoxicillin** * Above 3 months - **ceftriaxone** * **Steroids** - dexamethasone to reduce freq & severity of hearing loss/neurological complications **only in patients over 3 months**
223
What should be added if there is a risk of **penicillin-resistant pneumococcal infection**? ## Footnote e.g. recent foreign travel / prolonged ABx exposure
**Vancomycin**
224
What is the management of viral meningitis?
**Acyclovir** ## Footnote (mainly HSV)
225
What is the post-exposure prophylaxis for meningitis?
**Ciprofloxacin** - single dose
226
Name 5 complications of meningitis
* **Hearing loss** * Seizures & epilepsy * Cognitive impairment & LD * Memory loss * Focal neurological deficits - limb weakness / spasticity
227
What is a brain abscess?
Intracerebreal collection of pus
228
Name 3 causes of brain abscess
* **Cranial infections**: mastoiditis, sinusitis, osteomyelitis * Penetrating **head wounds** * **Haematogenous spread**: bacterial endocarditis, IV drug use
229
What are some symptoms of brain abscess?
Sx result from **raised ICP**, **mass effect** & **focal brain injury** * Headache * N+V * Lethargy * Seizures * Personality changes * Focal neurological deficits
230
What investigation is done for a brain abscess?
**Contrast-enhanced MRI** with DWI ## Footnote Contrast-enhanced CT if MRI unavailable
231
What is the treatment of a brain abscess?
* **ABx**: cefotaxime/ceftriaxone + metronidazole * **CT-guided aspiration** / **surgical drainage** * Corticosteroids for raised ICP Sx (dexamethasone)
232
Definition: coma
State of **unconsciousness** where a person is **unresponsive** & **cannot be woken**
233
What can cause a coma?
* Severe head injury * Stroke * Severe alcohol poisoning * Encephalitis * Hypoglycaemia / Hyperglycaemia
234
What is the management of someone in a coma?
Supporting body functions (breathing, BP etc) whilst treating underlying cause
235
What is the Glasgow Coma Scale?
Used to assess the level of consciousness in a patient
236
What is included in the Glasgow Coma Scale?
**Eye opening** * Spontaneously - 4 * To sound - 3 * To pain - 2 * No response - 1 **Verbal response** * Orientated - 5 * Confused conversation - 4 * Innapropriate words - 3 * Incomprehensible sounds - 2 * No response - 1 **Motor response** * Obeys commands - 6 * Localises to pain - 5 * Withdraws to pain - 4 * Abnormal flexion response to pain - 3 * Abnormal extension response to pain - 2 * No response - 1
237
Definition: Encephalitis
Inflammation of **brain parenchyma** associated w/ **neurological dysfunction**
238
What are some features of encephalitis?
* Altered mental state * Fever * Headache * Seizures * Focal neurological signs
239
Name some investigations of encephalitis
* Bloods * Lumbar puncture * CT scan
240
What is the management of encephalitis?
* ** IV Acyclovir** - clears virus * Anti-inflammatory drugs - if fever/pain * Anticonvulsants - if seizures
241
What lobe does Herpes Simplex Encephalitis characteristically affect?
**Temporal lobes** ## Footnote Question may mention changes on **CT scan** (e.g. petechial haemorrhages/ 'asymmetrical low-density areas') or describe **temporal lobe signs** (e.g. aphasia)
242
What is the triad of Horner syndrome?
* **Ptosis** * **Miosis** * **Anhidrosis**
243
What is the pathology of Horner syndrome?
Damage to **sympathetic nervous system**
244
What are the causes of Horner syndrome? ## Footnote **4 Ss, 4 Ts, 4 Cs** - **S**entral, **T**orso, **C**ervical
Central lesions: * **S**troke * Multiple **S**clerosis * **S**welling (tumours) * **S**yringomyelia (spinal cord cyst) Pre-ganglionic lesions: * **T**umour (Pancoast tumour) * **T**rauma * **T**hyroidectomy * **T**op rib (extra rib above clavicle) Post-ganglionic lesions: * **C**arotid aneurysm * **C**arotid artery dissection * **C**avernous sinus thrombosis * **C**luster headache
245
How do you test for Horner syndrome?
**Cocaine eye drops** * stops noradrenalin re-uptake at neuromuscular junction * causes normal eye to dilate (noradrenalin stimulates dilator muscles) * In Horner, nerves aren't releasing noradrenalin, so blocking re-uptake makes no difference = no pupil reaction
246
What are the 5 branches of the facial nerve?
1. Temporal 2. Zygomatic 3. Buccal 4. Mandibular 5. Cervical
247
What is the motor function of the facial nerve?
* **Facial expression** * **Stapedius** in the inner ear * Posterior **digastric**, **stylohyoid** and **platysma** muscles
248
What is the sensory function of the facial nerve?
**Taste** from the **anterior 2/3** of the tongue
249
What is the difference between upper vs lower motor neurone facial nerve palsy?
* UMN lesion: **Forehead sparing** * LMN lesion: patient cannot move their forehead on the affected side
250
What are 2 causes of **unilateral** UMN lesions?
* **CVA** (strokes) * **Tumours**
251
What are 2 causes of **bilateral** UMN lesions? ## Footnote These are rare
* **Pseudobulbar palsies** * **MND**
252
Definition: Bell's Palsy
**Unilateral**, **idiopathic** LMN facial nerve palsy
253
What are the clinical features of Bell's Palsy?
* **Rapid onset** < 72 hours * **Unilateral** facial weakness - forehead affected * Incomplete eye closure - dry eyes * Eyebrow, eyelid & mouth corner **drooping** * Loss of **nasolabial fold** * **hyperaucusis** due to stapedius muscle being impaired
254
What are some differentials for Bell's Palsy?
* **Inner ear disease** - otitis media, cholesteatoma * **Parotid disease** * **GBS** * **Stroke** * **Tumours** * **Meningitis** * **MS** * Sarcoidosis
255
What is the management of Bell's Palsy?
* Generally supportive * **Prednisolone** - within **72 hours** * **Eye care** - lubricating drops, eye taping at night * **for severe facial nerve palsy** then add Aciclovir
256
Definition: Bulbar Palsy
**LMN** lesion of cranial nerves **IX**, **X** and **XII**
257
Definition: Pseudobulbar palsy
**UMN** lesion of cranial nerves **IX**, **X** and **XII**
258
What is the main difference in presentation between bulbar and pseudobulbar palsy?
Pseudonbulbar - **Emotional lability** exaggerated mood changes Bulbar - emotions not affected
259
What are some causes of bulbar palsy?
* **Brainstem strokes & tumours** * **ALS** (amyotrophic lateral sclerosis) * **GBS**
260
What are some symptoms of bulbar palsy?
* **Dysphagia** * **Absent gag reflex** * Tongue wasting * Slurred speech * Difficulty chewing * Aspiration of secretions * Emotions **unaffected**
261
What is the management of bulbar palsy?
Symptom management * Difficulty swallowing - **feeding tube** * **Speech & Language therapy**
262
Definition: Myasthenia Gravis
**Autoimmune** condition affecting **neuromuscular junction** * causes **muscle weakness**
263
What is the pattern of muscle weakness in myasthenia gravis?
* **Progressive worsening** with **activity** * **Improves** with **rest**
264
What ages does MG affect men and women?
Women - **under 40 years** Men - **over 60 years**
265
What is strongly linked to Myasthenia Gravis?
**Thymomas** ## Footnote 10-20% pts with MG
266
What is the normal function of NMJ?
**Motor neurones** communicate with the **muscles** via **NMJ** * Axons release **Acetylcholine** from presynaptic memb. * Travels across **synapse** * Attaches to **receptors** on post-synaptic memb. * **Stimulates muscle contraction**
267
What is the pathophysiology of myasthenia gravis?
* **Acetylcholine receptor antibodies** (AchR) bind to post-synaptic ACh receptors = **block them** * **Prevents stimulation** by **ACh** * AChR antibodies also activate **complement system** within NMJ --> cell damage at post-synaptic memb. * **MuSK** & **LRP4** antibodies cause ACh receptor destruction
268
What antibodies are involved in myasthenia gravis?
* **Acetylcholine receptor** antibodies (AChR) * **Muscle-specific kinase** antibodies (MuSK) * **Low-density lipoprotein receptor-related protein 4** antibodies (LRP4)
269
Describe how MG is worsened during activity and better during rest
* The more the receptors are used during muscle activity = more they become blocked (i.e. less effective stimulation of muscle w/ increased activity) * With rest - receptors are cleared = Sx improve
270
What muscles are mainly affected in myasthenia gravis?
* **Proximal muscles of limbs** * **Small muscles of head & neck**
271
What are some symptoms of myasthenia gravis?
* **Difficulty climbing stairs**, standing from a seat, raising hands above head * Extraocular muscle weakness = **diplopia** * Eyelid weakness = **ptosis** * **Facial movement** weakness * **Swallowing difficulty** * **Jaw fatigue** when chewing * **Slurred speech**
272
What investigations should you do for myasthenia gravis?
* **Antibody tests** - AChR, MuSK, LRP4 * **CT**/**MRI** of the **thymus** (look for thymoma) * **Edrophonium test**
273
Describe the edrophonium test
* Pt given **IV edrophonium chloride** (or **neostigmine**) * Normally - **cholinesterase enzymes** in NMJ break down ACh * Edrophonium **blocks** cholinesterase enzymes = **reduces breakdown of ACh** * Results = **rise in ACh** at NMJ = temporarily **relieving weakness**
274
What is the management of myasthenia gravis?
* **Pyridostigmine** = cholinesterase inhibitor (prolongs action of ACh - improves Sx) * **Immunosuppression** = prednisolone / azathioprine (suppresses production of antibodies) * **Thymectomy** = improves Sx (even w/o thymoma) * **Rituximab** if other Tx fails
275
What is myasthenic crisis?
* **Acute worsening of Sx** often triggered by another illness * Resp muscle weakness --> **resp failure** * May require **non-invasive ventilation** (BiPAP) or **mechanical ventilation**
276
What is the treatment of myasthenic crisis?
* **IV immunoglobulins** * **Plasmapheresis**
277
Name some causes of mechanical back pain
* **Muscle**/ **ligament** sprain * **Sacroiliac joint** dysfunction * **Herniated disc** * **Scoliosis** * **Arthritis**
278
Name some red flag causes of back pain
* **Spinal fracture** - major trauma * **Cauda equina** - saddle anaethesia, urinary retention, incontinence, **bilateral sciatica** * **Spinal stenosis** * **Ankylosing spondylitis** - < 40y, gradual onset, morning stiffness, night-time pain * **Spinal infection** - fever, Hx of IVDU
279
Definition: Sciatica
Sx associated with irritation of **sciatic nerve**
280
What is the route of the sciatic nerve?
* **L4 - S3** join together = sciatic nerve * Exits posterior part of pelvis through **greater sciatic foramen** * Travels down back of leg, at knee divides into **tibial nerve** and **common peroneal nerve**
281
What is the function of the sciatic nerve?
* Sensation to **lateral lower leg & foot** * Motor function to **posterior thigh, lower leg & foot**
282
How does sciatica present?
* **Unilateral radiating pain**: butt --> back of thigh --> below knee/foot * "**electric / shooting**" * Paraesthesia * Numbness * Motor weakness
283
Name 3 causes of sciatica
Lumbosacral nerve root compression: * **Herniated disc** * **Spinal stenosis** * Spondylolisthesis
284
What investigation should you do for sciatica?
**Sciatic stretch test** * Pt lies on back with leg straight * You lift one leg from the ankle w/ knee extended until hip flexion limit (80-90 degrees) * You **dorsiflex** pt's ankle * Sx improve w/ knee flexion
285
What investigations should you do for back pain?
* Spinal fractures: **XR** / **CT** * Cauda equina: **Emergency MRI** * Ankylosing spondylitis: **CRP/ESR**, **XR spine** (bamboo spine), **MRI spine** (bone marrow oedema)
286
What is the management of lower back pain?
* Analgesia - **NSAIDs** (alt: codeine), **benzos** for muscle spasm * Staying active * Physiotherapy & CBT (chronic back pain)
287
What medications should be avoided in lower back pain? (5)
* **Opioids** * **Antidepressants** * **Amitriptyline** * **Gabapentin** * **Pregabalin**
288
What is the management of sciatica?
Mostly same as low back pain Tx * **DON'T USE**: gabapentin, pregabalin, diazepam, corticosteroids, opioids * **DO USE**: neuropathic meds = **amitriptyline, duloxetine** * Specialist Mx: epidural corticosteroid injections, local anaesthetic injections, radiofrequency denervation, spinal decompression
289
Definition: Spinal stenosis
**Narrowing** of part of the **spinal canal** = compression of **spinal cord** / **nerve roots** * usually affects cervical or lumbar (MC) spine
290
What are the 3 types of spinal stenosis?
* **Central** (central spinal canal) * **Lateral** (nerve root canal) * **Foramina** (intervertebral foramina)
291
What are some causes of spinal stenosis?
* **Congenital** * **Degenerative changes** * **Herniated discs** * Spinal fractures * Tumours
292
How does spinal stenosis present?
* Gradual onset * Sx absent at rest, occur when walking/standing * **Intermittent neurogenic claudication** (pseudoclaudication) * lower back pain * butt & leg pain * leg weakness
293
Definition: radiculopathy
**Compression** of the nerve roots as they exit the spinal cord and spinal column - leads to motor & sensory Sx
294
What investigation should you do for spinal stenosis?
**MRI** * exclude **PAD** (e.g. ABPI & CT angiogram) where intermittent claudication is present
295
What is the management of spinal stenosis?
* Exercise * Analgesia * Physio * Decompression surgery * Laminectomy
296
Definition: Charcot-Marie-Tooth Disease
Inherited disease that causes **motor & sensory neuropathy**
297
What is the epidemiology of CMT?
* Autosomal dominant * Sx usually appear **before 10 years old**
298
What are some features of CMT?
* **High foot arches** (pes cavus) * **Distal muscle wasting** * **Lower leg weakness** - loss of ankle dorsiflexion = foot drop / high stepping gait * **Hand weakness** * Reduced tendon reflexes * Reduced muscle tone * Peripheral sensory loss
299
Definition: stocking-glove distribution
Reduced sensory & motor function in peripheral nerves, typically affecting **feet and hands**
300
What are 5 causes of peripheral neuropathy? ## Footnote **ABCDE**
* **A**lcohol * **B**12 deficiency * **C**ancer (e.g. **myeloma**) & **C**KD * **D**iabetes & **D**rugs * **E**very vasculitis
301
Name 4 drugs that can cause peripheral neuropathy
* Isoniazid * Amiodarone * Leflunomide * Cisplatin
302
What is the management of CMT?
Supportive Mx with MDT * Physios * OTs * Podiatrist **Analgesia** for neuropathic pain - amitriptyline
303
Definition: Mononeuropathy
Only affects a **single nerve** e.g. meralgia paraesthetica/ carpel tunnel
304
What is the cause of Subarachnoid Haemorrhage?
**Ruptured cerebral aneurysm**
305
What are some risk factors for SAH?
* Age 45 - 70 * F > M * Black ethnic origin * HTN * Smoking * Excessive alcohol * Family Hx * Cocaine use * Sickle cell anaemia * Neurofibromatosis
306
How does SAH present?
* **Thunderclap headache** - sudden onset occipital headache * Neck stiffness * Photophobia * Vomiting * Neurological Sx
307
What investigations should you do for SAH?
* **CT head** (1st line): hyper-attenuation in subarachnoid space * **Lumbar puncture** if CT normal (at least **12 hours** after Sx start): **raised red cell count, xanthochromia** (yelloow CSF due to bilirubin) * **CT angiography** - confirms location of bleed
308
What is the management for SAH?
* **Surgical intervention** - aneurysms (endovascular coiling/neurosurgical clipping) * **Nimodipine** = CCB to prevent **vasospasm** (usually results in brain ischaemia)
309
What is a pseudo subarachnoid sign? And when do you see it?
SAH appearance on CT - due to loss of grey/white matter differentiation. Due to severe hypoxic injury to the brain
310
How do you treat hydrocephalus?
* Lumbar puncture * External ventricular drain * VP shunt
311
What is the triad caused by Cushing's reflex?
Cushing's reflex is a physiological response to raised ICP which attempts to improve perfusion. It leads to: * **HTN** * **Bradycardia** * **Irregular breathing pattern**
312
What is herniation in reference to the brain?
The movement of brain structures from one cranial compartment to another
313
What is primary vs secondary brain injury?
**Primary** = initial injury * skull fracture * blood vessel injury * haematoma **Secondary** = indirect damage commonly caused by inadequate perfusion * cerebral hypoxia * acidosis * hypoglycaemia * cerebral oedema
314
Where does a extradural haemorrhage occur?
Between the **skull** and **dura mater**
315
Where does a subdural haemorrhage occur?
Between the **dura mater** and **arachnoid mater**
316
What are the risk factors for intracranial bleeds?
* Head injuries * HTN * Aneurysms * Brain tumours * Bleeding disorders * Anticoagulants (e.g. DOACs / warfarin)
317
What is the cause of **extradural haemorrhage**?
Rupture of the **middle meningeal artery** in the **temporoparietal region**
318
Extradural haemorrhage is associated with which fracture?
fracture of the **temporal bone**
319
What are the characteristics of an extradural haemorrhage on a CT scan?
* **lentiform shape** (lemon) * Limited by the **cranial sutures**
320
What is the typical history for an extradural haemorrhage?
* **Young** patient * **Traumatic head injury** * **Ongoing headache** * Period of improved neurological Sx & consciousness followed by **rapid decline over hours** as haematoma gets large enough to compress intracranial contents
321
What is the cause of a subdural haemorrhage?
Rupture of **bridging veins** in outermost meningeal layer
322
What are the characteristics of a subdural haemorrhage on a CT scan?
* **Crescent shape** * **NOT** limited by the **cranial sutures** (they can cross over the sutures) * Instead limited by venous sinuses
323
Who is most likely to get a subdural haemorrhage? And why?
* **Elderly** * **Alcoholics** Because they have more **atrophy** in their brains = vessels more prone to rupture
324
What are the initial investigations you should do for an intracranial bleed?
* Immediate **CT head** - to establish diagnosis * FBC (**platelets**) & **coag screen**
325
What is the management of intracranial bleeds?
* Correct any **clotting abnormality** - platelet transfusion or vit K for warfarin * Correct **severe hypertension** - avoid hypotension * Admit to **specialist stroke centre** * Small bleeds = managed **conservatively** (close monitoring & repeat imaging) * Consider surgery
326
What are 2 **surgical** options for treating an extradural or subdural haematoma?
* **Craniotomy** - open surgery, removing section of skull * **Burr holes** - small holes drilled into skull to drain blood
327
What is anterior cord syndrome?
Front of the spinal cord is damaged, but posterior part is spared
328
Which spinal tracts are affected in anterior cord syndrome?
**Spinothalamic** and **Corticospinal**
329
What causes anterior cord syndrome?
**Ischaemia** within the **anterior spinal artery** * Can cause damage to the anterior part of the spinal cord * Analogous to a **stroke** within the spinal cord Can also be caused by: * **External compression** * **Flexion** injuries
330
What are the features of anterior cord syndrome?
* Spinothalamic tracts: **bilateral loss of pain & temperature** * Corticospinal: **bilateral spastic paralysis** & **UMN** signs Dorsal columns = posterior = won't be affected: fine touch/proprioception/vibration will be **preserved**
331
What is the management of anterior cord syndrome?
* **Immobilisation** of neck/back - reducce further damage * **Steroids** - reduce inflammation/swelling * **Surgery** - decompress spinal cord / stabilise spine * **Rehab** - PT / OT
332
What is cauda equina?
Surgical **emergency** where cauda equina nerve roots are **compressed** leading to neurological compromise
333
What do the cauda equina nerves supply?
* **Sensation** to the LL, perinuem, bladder & rectum * **Motor** innervation to the LL & anal/urethral sphincters * **Parasympathetic** innervation of bladder & rectum
334
Name 5 causes of compression in cauda equina syndrome
* **Herniated disc** (MC) * **Tumours** (metastasis) * **Spondylolisthesis** (anterior disaplacement of a vertebra out of line w/ one below * **Abscess** (infection) * **Trauma**
335
What are the red flags to look out for in cauda equina syndrome?
* **Saddle anaesthesia** * **Loss of sensation** in bladder/rectum * **Urinary/faecal incontinence** / retention * **Bilateral sciatica** * Bilateral / severe **motor weakness in legs** * Reduced anal tone
336
What investigations should you do for cauda equina syndrome?
* **MRI lumbar spine** * Straight leg raise (**+ve**)
337
What is the management of cauda equina syndrome?
**Lumbar decompression surgery** * laminectomy & discectomy
338
What is a dermatome?
**Area of skin** supplied by a **single spinal nerve**
339
What is a myotome?
**Group of muscles** innervated by a **single spinal nerve**
340
What are myopathies?
Class of diseases that attack the **skeletal muscles**
341
What are the types of myopathy?
* Inflammatory * Steroids * Metabolic * Myotonic * Statin * Muscular dystrophy * Polio
342
What are the symptoms of myopathy?
* **Muscle weakness** * Muscle cramps / stiffness * Muscle spasms * Fatigue
343
What is muscular dystrophy?
Group of conditions that affect the function of the muscles - cause progressive muscle weakness / degeneration
344
What are the symptoms of myotonic dystrophy?
* **Weakness of smaller muscles** first (face, jaw, neck) * muscle stiffness * Cataracts * dysphagia * slow, irregular heartbeat
345
What is the management of muscular dystrophy?
* No cure, so treat Sx & complications (e.g. PT, OT) * **Steroids** for Duchenne MD
346
What is steroid myopathy?
* Most common drug-induced myopathy, can be acute or chronic. Characterised by muscle weakness after starting steroids * **Corticosteroid myopathy**: after an endocrine cause e.g. cushings
347
What investigation should you do for myopathy?
**Muscle biopsy**
348
What can cause inflammatory myopathy?
**Infections** e.g. HIV, influenza, EBV
349
What are metabolic myopathies?
Rare genetic diseases that affect metabolism and primarily affect muscle
350
What is statin-induced myopathy?
Side effect of taking statins. Leads to muscle Sx e.g. weakness, stiffness, pain, fatigue
351
What is poliomyelitis?
**Viral infection** causing nerve injury leading to **paralysis**
352
What is bovine papular stomatitis?
Virus that can spread from cattle to farmers/vets. Causes **skin lesions** typically on **hands**/ forearms
353
What is Uhthoff’s phenomenon?
Worsening of Sx on exercise/ in warm environment e.g. shower
354
Dx of normal pressure hydrocephalus
CT- enlarged ventricles LP- CSF pressure will be normal
355
What is foot drop most commonly caused by(3)
Common peroneal nerve lesion L5 radiculopathy Sciatic nerve lesion
356
Most common brain tumour in adults
Glioblastoma multiforme
357
What gene is associated narcolepsy
HLA DR2
358
Most common cancers causing metastatic cord compression
Breast Prostate lung cancer Myeloma
359
Most common cancers causing metastatic cord compression
Breast Lung Prostate Myeloma
360
Treatment of metastatic cord compression
Analgesia Steroids - dexamethasone Surgery - to decompress Physiotherapy
361
Investigation for suspected metastatic cord compression
MRI whole spine - within 24 hours If CI then CT whole spine
362
Most common cancers that cause hypercalcaemia of malignancy (5)
Breast Prostate Lung Thyroid Renal cancer
363
How does hypercalcemia of malignancy occur
-PTH related peptide - secreted from tumour cells -acts on same receptors as PTH increasing serum calcium -increased bone resorption -increased calcium resorption
364
Immediate Mx for hypercalcemia of malignancy
IV fluids - 0.9% NaCl 3-4L in first 24 hrs IV **bisphosphonates after rehydration Monitor calcium Tx of malignancy
365
What’s the tx of hypercalcaemia of malignancy if its refractory
More aggressive tx -denosumab- RANKL inhibitor -Calcitonin -Dialysis if there’s renal failure
366
Causes of raised ICP
idiopathic intracranial hypertension traumatic head injuries meningitis tumours hydrocephalus
367
Features of raised ICP
-headache -vomiting -reduced levels of consciousness -papilloedema -Cushing's triad widening pulse pressure bradycardia irregular breathing
368
Ix for ICP
-neuroimaging (CT/MRI) is key to investigate the underlying cause -invasive ICP monitoring- usually via catheter placed into lateral ventricles to monitor pressure -CSF samples
369
Mx of raised ICP (4)
-Head elevation to 30 degrees -IV mannitol -controlled hyperventilation - to reduce PaCO2 -removal of CSF - ventricular peritoneal shunt/ repeat LP/ drain from catheter
370
Why does Bell’s palsy affect the forehead
It is a paralysis of the facial nerve so all of that nerve is affected ipsilaterally including the frontalis division of the facial nerve
371
What is syringomyelia
a collection of cerebrospinal fluid within the spinal cord.
372
Most common cause of syringomyelia
Chiari I malformation Spina bifida Ependymoma/astroctyoma
373
What is chiari I malformation
downward herniation of the cerebellar tonsils through the foramen magnum impedes CSF circulation
374
Clinical features of syringomyelia
**cape-like' (neck and arms) loss of sensation to temperature and pain** **pain worse on valsalva manoevures -spastic weakness -paraesthesia -neuropathic pain -scoliosis - if not treated
375
Ix for syringomyelia
**MRI of entire spine and brain- gold standard**
376
Management of syringomyelia
*Address underlying cause * surgical - If chiari malformation then decompression If tumour then resection of tumour
377
What do you see on head CT for a meningioma
Dural tail - thickened strip of dura mater next to the tumour
378
Dose of methylprednisolone given to MS patients
500mg
379
What antibody is responsible for neuromyelitis optica
Aquaporin 4 antibodies
380
What artery do you treat with thrombectomy if occluded
Middle cerebral artery
381
What underlying cause is suggestive of painful horners syndrome
**internal carotid artery dissection**
382
What is Waterhouse friedrechsen syndrome
Adrenal haemorrhage caused by nisseria meningitidis
383
What are the Parkinson plus syndromes (3)
Progressive supranuclear palsy multiple system atrophy Corticosteroids basal degeneration
384
What is bilateral acoustic neuroma seen in
Neurofibromatosis type 2
385
Investigation of choice in suspected acoustic neuroma
MRI of cerebellopontine angle Audiometry
386
Migraine tx in pregnant people
1st line - paracetemol 1g 2nd line - NSAIDS
387
Oculomotor nerve palsy features (3)
*down and out pupil *ptosis (lavator palpebrae) *mydriasis (pupil dilation)
388
Trochlear nerve palsy features
*Vertical diplopia - double vision when looking inferiorly *Head tilt to compensate
389
Abducens nerve palsy features
*unopposed abduction - pupil is drawn in medially *horizontal diplopia (wore when looking at affected side)
Phase 3a - Arun&Erin
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