1
Q
define dementia
A
Dementia is a syndrome, usually of chronic or progressive nature, which involves impairment of multiple higher cortical functions, such as memory, thinking, orientation, comprehension and language.
2
Q
what is the incidence and prevalence of dementia?
A
- incidence 200 per 100,000
- prevalence 1500 per 100,000
3
Q
what proportion of people over 80 have dementia?
A
1/6
4
Q
what are late onset (65+ y/o) causes of dementia?
A
- Alzheimer’s disease (55%)
- Vascular dementia (20%)
- Lewy body (20%)
- Others (5%)
5
Q
what are causes of early onset (<65 y/o) dementia?
A
- Alzheimer’s disease (33%)
- vascular dementia (15%)
- frontotemporal dementia (15%)
- other (33%): toxic (alcohol), genetic (Huntington’s), infection (HIV, CJD), inflammatory (MS)
6
Q
what investigations are performed when for suspected dementia?
A
- routine: bloods, CT/MRI
- cognitive assessments
- others: CSF, EEG, functional imaging, genetics
7
Q
what is the speed of dementia progression in Creutzfeldt-Jakob disease (CJD)?
A
rapid progression
8
Q
what is the speed of dementia progression in vascular dementia?
A
stepwise progression
9
Q
what is the most common neurodegenerative condition?
A
Alzheimer’s disease
10
Q
what is the mean age of onset of AD?
A
70 years old (25% < 65y/o)
11
Q
what is the pathophysiology
of AD?
A
Hallmarks:
- beta-amyloid plaque accumulation.
- Tau tangles: abnormal tau protein accumulation formes neurofibrillary tangles.
12
Q
risk factors Alzheimer’s disease
A
- advanced age
- genetic predisposition (APOE, trisomy 21, APP, PSEN1, PSEN2)
- family history
- CV risk factors: hypertension, diabetes, obesity, hypercholesterolemia
- lifestyle factors: physical inactivity, smoking, diet high in saturated fats
- traumatic brain injury
13
Q
what are clinical features of temporo-parietal dementia?
A
- early memory disturbance
- language and visuospatial problems
- personality preserved until later
14
Q
what are clinical features of frontotemporal dementia?
A
- early change in personality/behaviour
- often change in eating habits
- early dysphasia
- memory/visuospatial relatively preserved
15
Q
what is the pathology of dementia with Lewy bodies?
A
abnormal accumulation of alpha-synuclein protein > Lewy bodies
16
Q
what are clinical features of dementia with Lewy bodies?
A
- early visuospatial and executive dysfunction
- prominent fluctuation
- Parkinsonism and visual hallucinations common
17
Q
what is the medical treatment for Alzheimer’s (+/- Lewy body dementia)?
A
cholinesterase inhibitors (treat cholinergic deficit):
- donepezil, rivastigmine, galantamine
NMDA antagonist (memantine)
18
Q
what is Parkinsonism?
A
A clinical syndrome with 2 or more of:
- bradykinesia (slowness of movement)
- rigidity (stiffness)
- tremor (shakiness)
- postural instability (unsteadiness/falls)
19
Q
what are causes of Parkinsonism?
A
- idioptathic Parkinson’s disease > dementia with lewy bodies.
- drug-induced (e.g. dopamine antagonists).
- vascular parkinsonism (lower-half)
- multiple system atrophy (MSA)
- cortico-basal degeneration
- progressive supranuclear palsu (PSP)
20
Q
risk factors of PD
A
- genetic (LRRK2, Parkin, GBA)
- environmental (pesticides, smoking, caffeine)
21
Q
clinical diagnosis of PD
A
- bradykinesia + 1 or more of: tremor, rigidity, postural instability
- no other cause/atypical features
- slowly progressive (>5-10 years)
- good response to dopamine replacement treatment
22
Q
what are the cardinal signs of parkinsonism?
A
- tremor: unilateral and at rest.
- bradykinesia > progressive
- postural instability
23
Q
what are some non-motor signs of PD?
A
- olfactory loss (anosmia)
- autonomic dysfunction (constipation, urinary and bowel dysfunction, erectile dysfunction)
- REM sleep behaviour disorder
- psychiatric features: depression, anxiety, hallucinations
24
Q
what functional imaging can be used to grade PD?
A
dopamine transporter SPECT
25
what is the early medical treatment of PD?
- L-dopa
- dopamine agonists: ropinirole, rotigotine, apomorphine, pramipexole
- MAO-B inhibitors: selegiline, rasagiline, safinamide
- COMT inhibitors: entacapone, tolcapone, opicapone
26
what are drug-induced complications in PD?
- motor fluctuations - levodopa wears off
- dyskinesias (involuntary movements)
- psychiatric - hallucinations, lack of impulse control
27
what are complications of PD?
- balance/falls/fractures
- dementia (50% after 10 years)
- speech, swallow
- gait freezing
28
what is the late treament for PD?
Drugs:
- prolong levodopa half life: MAO-B inhibitors, COMT inhibitor, slow release levodopa.
- add oral dopamine agonist.
- continous infusion (apomorphine, duodopa)
- Functional neurosurgery (deep brain stimulation)
- allied health professional +/- care package
29
WHO definition of stroke
- focal neurological deficit (loss of function affecting a specific region of the CNS) due to disruption of blood supply.
30
In practise, what are the three main causes of localised interrupted blood supply?
1. atheroma + thrombosis of artery causing ischaemia (e.g. internal carotid artery thrombosis).
2. thromboembolism (for example , from left atrium) causing ischaemia.
3. ruptured aneurysm of a cerebral vessel causing haemorrhage (=/- dostal ischaemia due to spasm of artery).
31
what is a localised area of brain death called?
regional cerebral infarct
32
what are 2 common sites of ruptured vessels causing haemorrhagic stroke?
- basal ganglia - microaneurysms form in hypertensive patients.
- Circle of Willis - Berry anaeurysm forms in hypertensive patients.
33
what are causes of generalised interrupted blood supply or hypoxia to the brain?
1. low oxygen in blood (hypoxia with intact circulation of blood) e.g. CO2 poisoning, near drowning, respiratory arrest.
2. inadequate supply of blood (flow of blood not occurring) e.g. cardiac arrest, hypotension, brain swelling (trauma).
3. rarely: inability to use oxygen e.g. cyanide poisoning.
34
What are 'Watershed' infarcts?
WATERSHED INFARCTS are ischemic lesions which are situated along the border zones between the territories of two major arteries, for example the anterior and middle or the middle and posterior cerebral arteries.
35
what can cause Watershed infarcts?
prolonged hypotension with oxygenated blood
36
what are three examples of brain injury caused by generalised interrupted blood supply?
1. hypotension > watershed infarction
2. cardiac arrest > cortical infarction
3. complex case > complex pattern
37
what are causes of raised intracranial pressure?
localised lesions:
- haemorrhage (haematoma)
- tumour
- abscess
generalised pathology:
- oedema post-trauma
38
what are the effects of intracranial space occupying lesions?
- raised ICP
- internal shift (herniation): right-left or left-right (under falx), cerebrum moves inferiorly over edge of tentorium (uncal herniation), cerebellum moves inferiorly into foramen magnum (coning)
39
symptoms and signs of raised ICP
- squeeze on cortex and brainstem > morning headaches and sickness
- squeeze on optic nerve > papilloedema
- pupillary dilation > squeeze and stretch on CN III
- falling GCS
- brain stem death due to cerebellar tonsil herniation
40
what cancers can commonly metastasize to the brain?
breast
lung
kidney
colon
melanoma
41
what is a tumour of embryonic neural cells called?
medulloblastoma
42
what are tumours of the nerve sheath cell called?
schwannoma
neurofibroma
43
are the majority of brain tumours in adults above or below the tentorium?
above
44
are the majority of brain tumours in children above or below the tentorium?
below
45
do gliomas metastasise outside the CNS?
NO
46
what are the edges of a glioma like?
diffuse edges > NOT encapsulated
47
describe a low grade astrocytoma
- cancer of astrocytes.
- bland cells on microscopy (similar to normal astrocytes), small cells with single nucleus.
- grow very slowly.
48
describe a glioblastoma
- most malignant form of astrocytoma.
- cellular, atypical tumour with necrosis under microscope, lare cell with multiple or irregular nucleus.
- grow quickly > often present as large tumours.
49
what area of the brain are medulloblastomas present in?
posterior fossa, especially the brainstem
50
del
del
51
what is an acoustic neuroma?
- CN VIII vestibulocochlear nerve schwannoma at angle between pons and cerebellum.
- causes unilateral deafness.
- benign lesion but removal technically difficult.
52
features of a CNS lymphoma
- high grade neoplasm
- usually diffuse large B-cell lymphoma
- often deep and central site in brain > difficult to biopsy
- generally do not spread outside of CNS
53
describe a hemagioblastoma
- tumour of blood vessels
- space occupying
- may bleed
- most often in cerebellum
54
describe osmotherapy related to treating ICP and what is used
- TEMPORARY attempt to use autoregulation to reduce ICP.
- Mannitol: osmotic diuretic, will cause hypovolaemia. Easy to give > 0.5-2g/kg dose.
- Hypertonic saline: harder to give but less side effects.
55
Discuss the treatment of ICP
- ABCDE
- sit up at 30-40 degree angle
- reduce ICP with hypertonic saline
- Definitive management of the underlying cause should then be sought with CNS imaging and neurosurgical input as appropriate.
56
what are the main blood vessels in the brain?
- right and left internal carotid arteries
- right and left vertebral arteries
- basilar artery
- right and left anterior cerebral arteries
- right and left middle cerebral arteries
- right and left posterior cerebral arteries
- anterior communicating artery
- right and left posterior communicating arteries
57
what is the treatment of a ruptured intracranial aneurysm?
- endovascular coil embolisation
- surgical clipping
- treating complications such as hydrocephalus, vasospastic infarcts and disability
58
The right middle cerebral artery supplies areas of the brain that allow for which functions?
- left body strength and sensation
59
the left middle cerebral artery supplies areas of the brain that allow for which functions?
- right body strength and sensation
- language
60
the right posterior cerebral artery supplies areas of the brain that allow for which functions?
- perception of the left visual field
61
the left posterior cerebral artery supplies areas of the brain that allow for which functions?
- perception of right visual field
62
the cerebellum is supplied by which arteries?
- posterior inferior, anterior inferior and superior cerebellar arteries.
63
how are the lateral ventricles connected to the third ventricle?
foramen of Munro, or interventricular foramen
64
how is the third and fourth ventricle connected?
cerebral aqueduct of Slyvius or the mesencephalic duct
65
the fourth ventricle empties into the subarachnoid space by which foramen?
- median aperture > foramen of Magendie
- lateral apertures > foramen of Luschka
66
rehabilitation definition
- the active participation of a disabled person and others to reduce the impact of disease and disability on daily life.
67
what are the aims of rehabilitation?
- **enabling** and supporting you
- **adjusting** to your new situation
- **achieving** your best possible potential
- **living** life as fully and **actively** as possible
- becoming as **independent** as possible
68
what is considered a severe head injury? use GCS and PTA
- GCS 3-8
- post-traumatic amnesia (PTA) 1-7 days
69
what is considered a moderate head injury? use GCS and PTA
- GCS 9-12
- PTA 1-24 hours
70
what is considered a mild head injury?
- GCS 13-15
- PTA < 1 hour
71
physical impairments from neurological conditions
- weakness (hemiparesis/paraparesis)
- loss of/abnormal sensation
- increased or decreased tone/spasticity
- autonomic dysfunction: bladder instability, bowel disturbance, difficulty in bowel and bladder sensation and recognition
- swallowing and communication difficulties
- pain syndrome > somatic, neuropathic
- seizures
- neuroendocrine disturbance
- physical fatigue
72
cognitive ('thinking') impairments after brain surgery
- post-traumatic amnesia (PTA)
- confusion/disorientation: time, place, person
- severe memory problems
- poor concentration/attention
- slowed thinking and mental fatigue
- poor executive function, planning, reacting to changing events
- impaired reasoning and problem solving
73
psychiatric/behavioural impairments after brain injury
- depression
- anxiety
- personality change
- irritability
- 'childishness, selfishness, laziness'
- behavioural problems: aggression, disinhibition, apathy
- anhedonia
74
secondary complications of long-term neurological conditions
- pressure sores
- infections: urine, chest
- falls and other secondary injury
- DVT
- malnutrition
- constipation
- pain and spasticity
- contractures
- low morale and depression
75
evidence for the benefit of rehabilitation
- stroke units vs general medical ward
- more likely to be discharged home
- reduced carer dependency
- also evidence for efficacy in traumatic brain injury
- cost effectiveness: care costs, economic productivity
76
what are SMART goals used in the rehabilitation process?
goals should be:
Specific
Measurable
Achievable
Realistic
Timely
77
what layer of the meninges contains blood vessels?
subarachnoid space
78
what is the classic clinical triad associated with normal pressure hydrocephalus?
| wet, wacky and wobbly
- Dementia: Often manifests as global cognitive impairment, with attention and memory disturbances.
- Magnetic gait: Characterized by difficulty in lifting the feet off the floor, appearing as if they are "stuck."
- Incontinence: Primarily urinary incontinence, but fecal incontinence can also occur.
79
label the dermatomes of the upper limb
80
label the dermatomes of the lower limb
81
what are contraindications to performing a lumbar puncture?
a. Focal neurological deficit
b. Bleeding disorder
c. Head trauma
d. Papilloedema
e. Localised infection over skin site
f. Recent Intracranial bleed
g. Raised ICP
Neurology Systems
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