Neurological Flashcards
1
Q
What is the pathophysiological of Alzheimer’s Disease?
A
- Macroscopic changes include widespread cerebral atrophy involving the cortex and hippocampus
- Microscopic changes include cortical plaques due to deposition of type A-Beta-amyloid protein and intraneuronal neurofibrillary tangles caused by aggergation of hyperphosphorylated tau protein
- Biochemical changes include deficit of acetylcholine
- RF include age, family history (presenilin mutation), caucasian ethnicity, Down’s syndrome
2
Q
What is the 1st line treatment for Alzheimer’s Disease?
A
Donepezil, galantamine, rivastigmine
These are all acetylcholinesterase inhibitors
3
Q
What is the 2nd line treatment for Alzheimer’s Disease and when is it used?
A
Memantine (NMDA receptor antagonist)
Uses:
- Moderate AD who are intolerant or have a contraindication to acetylcholinesterase inhibitors
- As an add on drug to acetylcholinesterase inhibitors for patients with moderate or severe AD
- Monotherapy in severe AD
4
Q
What are the contraindications for donepezil?
A
Patients with bradycardia eg. sick sinus syndrome
5
Q
How are non-cognitive symptoms managed in AD?
A
Antipsychotics are only used in patients who are at risk of harm to themselves or others, or when agitation is causing them severe distress
NICE do not recommend antidepressants for mild to moderate depression in patients with dementia
6
Q
What are the features of Lewy Body Dementia?
A
- Progressive cognitive impairment
- Typically occurs before parkinsonism
- Cognition may be fluctuating
- Early impairments in attention and executive function rather than just memory loss - Parkinsonism
- Visual hallucinations
7
Q
How is LBD diagnosed?
A
Usually clinical
SPECT aka DaTScan is increasingly used - this has a sensitivity of 90% and specificity of 100%
8
Q
How is LBD managed?
A
Mild/moderate:
1st line - Donepezil or rivastigmine
2nd line - Galantamine, memantine
Do not give neuroleptics (eg. antipsychotics) as patients may develop irreversible parkinsonism - give lorazepam if agitation
9
Q
What are the features of vascular dementia?
A
- Thought to account for 17% of dementia in UK (2nd most common)
- Tends to be step-wise deterioration
- History of strokes/vascular disease
- Subtypes include stroke-related, subcortical (small vessel disease), or mixed (VD + AD)
- Can be inherited as CADASIL
- Focal neurological abnormaliites seen
10
Q
What is the NINDS-AIREN criteria?
A
Criteria used for probable vascular dementia:
Presence of cognitive decline that interferes with activities of daily living, not due to secondary effects of the cerebrovascular event
- established using clinical examination and neuropsychological testing
Cerebrovascular disease
- defined by neurological signs and/or brain imaging
A relationship between the above two disorders inferred by:
- the onset of dementia within three months following a recognised stroke
- an abrupt deterioration in cognitive functions
- fluctuating, stepwise progression of cognitive deficits
11
Q
How is vascular dementia managed?
A
- No specific pharmacological treatment
- Only give AChE inhibitors/memantine if comorbid AD PDD or LBD
- Treatment tends to be symptomatic with aim to address cardiovascular risk factors
12
Q
What are the features of frontotemporal lobar degeneration?
A
- Onset before 65
- Insidious onset
- Relatively preserved memory and visuospaital skills
- Personality change and social conduct problems
13
Q
What are the three recognised types of FTLD?
A
- Frontotemporal dementia (Pick’s disease)
- Personality change and impaired social conduct
- Changes include focal gyral atrophy with knife-blade appearance , pick bodies, gliosis, neurofibrillary tangles and senile plaque - Progressive non fluent aphasea (chronic progressive aphasia)
- Non-fluent, agrammatic, short utterance with preserved comprehension - Semantic dementia
- Fluent, progressive aphasia
14
Q
How is frontotemporal lobar degeneration managed?
A
- No routine pharmacological treatment recommended
15
Q
What is the Addenbrookes Cognitive Exam? What would you see in each form of dementia?
A
A validated tool for detection of dementia - a score of 82 or less strongly suggests dementia and a score of 82-88 suggests MCI
Alzheimers - global deficit
Frontotemporal - deficits in fluency and language
Vascular - no consistent pattern
16
Q
What is the MMSE?
A
Mini mental state examination - an assessment tool used to detect dementia. A score of <24 out of 30 suggests dementia
17
Q
Peripheral neuropathy may be divided into conditions which cause a motor or sensory loss. Give examples of each.
A
Predominately motor loss:
- Porphyria
- Lead poisoning
- Hereditary sensorimotor neuropathies (Charcot-Marie-Tooth)
- Chronic inflammatory demyelinating polyneuropathy
- Diphthreia
Predominately sensory loss:
- Diabetes
- Uraemia
- Lepropsy
- Alcoholism
- Vitamin B12 deficiency
- Amyloidosos
18
Q
What can excess vitamin B6 lead to (pyridoxine)?
A
Peripheral neuropathy
19
Q
What is the typical sensory change with peripheral neuropathy?
A
Symmetrical glove and stocking
20
Q
Which conditions tend to cause painful neuropathies?
A
Alcohol, nutritional deficiencies
Diabetes
Hereditary sensory and autonomic neuropathy
Arsenic
Cryoglobulinaemia
Lyme disease
Paraneoplastic sensory neuropathy
Vasculitis neuropathies
21
Q
What is Guillain-Barre syndrome?
A
Immune mediated demyelination of the peripheral nervous system causing progressive symmetrical ASCENDING weakness of all the limbs
Pathogenesis:
- Usually triggered by campylobacter jejuni
- Cross reaction of antibodies with gangliosides in the PNS
- Anti-GBM 1 antibodies found in 25% of patients
22
Q
What are the examination findings in GBS?
A
- Progressive, symmetrical ascending weakness
- Reflexes reduced or absent
- Mild sensory symptoms
- Resp muscle weakness
- CN involvement (diplopia, bilateral facial nerve palsy, oropharyngeal weakness)
- Autonomic (urinary retention, diarrhoea)
- Papilloedema
23
Q
How is GBS investigated?
A
Lumbar puncture - high protein, normal WCC
NCS - decreased motor conduction, prolonged motor/F wave latency
24
Q
How is GBS managed?
A
IV immunoglobulin (or plasma exchange if IgA deficiency/renal failure) + supportive treatment
25
What are the features of MS?
Diagnose on the basis of two or more relapses and two or more lesions:
Visual:
- Optic neuritis or atrophy
- Uthoff's phenomenen (worsening of vision following rise in body temperature)
- Internuclear ophthalmoplegia
Sensory:
- Parasthesia/numbness
- Trigeminal neuralgia
- Lhermitte's syndrome (parasthesiae in limbs on neck flexion)
Motor:
- Spastic weakness most commonly in legs
Cerebellar:
- Ataxia (usually relapse rather than acute)
- Tremor
Others:
- Urinary incontinence
- Sexual dysfunction
- Intellectual deterioration
26
How is MS investigated?
Disseminated lesions in time and space
MRI:
- High signal T2 lesions
- Periventricular plaques
- Dawsons fingers in FLAIR images
CSF:
- Oligoclonal bands
- Increased intrathecal synthesis of IgG
Visual evoked potentials
- Delayed but well preserved waveform
27
How is MS treated?
Acute:
- High dose steroids for 5d
DMD - start if relapssing-remitting or secondary progressive:
1st line - IV natalizumab or ocrelizumab (monoclonal anitbodies)
2nd line - fingolimid, beta-interferon or glatiramer acetate
Spasticity:
1st line - baclofen, gabapentin
2nd line - diazepam, dantrolene, tiazindine
Future - cannabis, botox
Bladder dysfunction:
- USS first, if residual volume for intermittitent self-cath
- If no residual, anticholinergics
Oscillopsia (oscillating visual fields):
- Gabapentin
28
What are the features of MND?
- Upper and lower motor neurone signs
- Most common patterns are ALS, progressive muscular atrophy and bulbar pulsy
- DOES NOT AFFECT EXTERNAL OCULAR MUSCLES OR CEREBELLAR
- poor prognosis (50% of pts die within 3 years)
29
How is MND managed?
Riluzole
- prevents stimulation of glutamate receptors in ALS (as MND thought to be due to overactivity of glutamate causing nerve damage)
- prolongs life by 3m
Respiratory care
- BIPAP at night
- prolongs life by 7m
Nutrition
- PEG
30
What are the features of degenerative cervical myelopathy? How is it diagnosed and managed?
Features:
- RF include smoking, genetics and occupation
- Subtle symptoms incl pain, loos of motor/sensroy/autonomic function
- Positive Hoffman's sign (gently flick one finger on a patients hand - a positive test results in reflex twitching of the other fingers on the same hand in response)
Diagnosis:
- MRI cervical spine; shows disc degeneration and ligament hypertrophy
Management:
- Urgent NS referral
- Early treatment (<6 months) with decompressive surgery
- PHYSIO SHOULD ONLY BE INITIATED BY SPECIALISTS
If symptoms recur - refer urgently to NS
31
What are the features of porphyric neuropathy?
- Porphyria is a group of liver disorders in chich prophyrins build up in the body due to overproduction of haem
- Porphyric attacks involve brain fog, anxiety and restlessness followed by severe abdominal pain, peripheral neuropathy and PURPLE URINE
- Motor-predominant axonal neuropathy
- Begins symmetrically in the upper extremities followed by lower extremity involvement
- Can progress rapidly to quadriparesis and resp involvement if untreated
32
What is subacute combined degeneration of the spinal cord?
- Impariment of dorsal columns, lateral corticospinal tract and spinocerebellar tracts
- Due to B12 deficiency
Dorsal column:
- Symmetrical parasthesia/numbness in the legs
- Impaired proprioception and vibration sense
Lateral corticospinal tract:
- Weakness, hyperreflexia, spasticity
- UMN signs
- Brisk knee, absent ankle
- Extensor plantars
Spinocerebellar tract:
- Sensory ataxia
- Positive Rombergs
33
What are the features and management of trigeminal neuralgia?
Features:
- Severe unilateral electric-shock type pain
- Evoked by light touch
Management:
- Carbamazepine
- Failure to respond or atypical features> urgent neuro referral
34
How is epilepsy classified?
Focal:
- aka partial
- aware or not aware
- motor or non-motor
Generalised:
- no awareness
- motor or non-motor
- tonic-clonic/absence
Focal to bilateral:
- starts on one side of brain before spreading to both lobes
- aka secondary generalised seizure
35
How is epilepsy diagnosed and managed?
Diagnosis:
- Brain imaging/EEG
Treatment is typically started after 2nd seizure UNLESS:
- persistent neurological deficit
- structural abnormality on brain imaging
- EEG shows unequivocal epileptic activity
- patient/carer choice
Generalised tonic-clonic/myoclonic or tonic/atonic:
1st line - sodium valproate in males or lamotrigine/levetiracetam in females
Focal:
1st line - lamotrigine/leveutracetam
2nd line - carbamazepine
Absence:
1st line - ethosuxamide
36
What are the DVLA rules for epilepsy?
Epilepsy with LOC:
- Inform DVLA
- Can drive if seizure free for 1 year UNLESS epilepsy with recent medication change (6 monhths)
First seizure with LOC:
- Inform DVLA
- Can drive if seizure free for 6 months and low risk of recurrence (normal imaging/EEG)
Seizures whilst asleep:
- Inform DVLA
- May still qualify for licence if >12m since last seizure
Seizures with no impairment in consciousness:
- Inform DVLA
- Can qualify for licence if first seizure must have been over 12m ago
37
How are acute seizures managed?
Basics:
- Check airway +/- oxygen
- Recovery position
- Give rectal diazepam if prolonged seizure, and repeat after 10-15 minutes if necessary
- Midazolam solution is alternative
38
What is status epilepticus and how is it managed?
Definition:
- Single seizure lasting >5 minutes or >2 seizures within 5 minutes without the person returning to normal between them
Management:
1. ABC
2. Benzo - IV lorazpem in hospital, PR diazepam or buccal midazolam OOH. Repeat once after 5-10 minutes
3. If ongoing start levetiracetam, phenytoin or sodium valproate
4. If ongoing within 45 minutes, general anaesthesia or phenobarbital
39
How can a pseudoseizure and an epileptic seizure be differentiated?
Pseudoseizure:
- Normal bloods incl prolactin
- Hip thrusting
- Absence of tongue biting
- Only when people watching
- Family member with epilepsy
- Gradual onset
40
How does carbamazepine work and what are the side effects?
MOA:
- Binds to sodium channels to increase their refractory period
SE:
- P450 enzyme inducer
- Dizziness/ataxia/drowsiness
- Headache and diplopia
- SJS
- Leucopenia and agranulocytosis
- Hyponatremia secondary to SIADH
41
How does phenytoin work and what are the side effects?
MOA:
- Binds to sodium channels increasing their refractory period
SE:
- Dizziness/diplopia/nystagmus/ataxia
- P450 enzyme inducer
- Megaloblastic anaemia
- Cushings type sx
- Peripheral neuropathy
- Duputryens
- Lupus
- Rashes
- Teratogenic
MONITOR TROUGH LEVELS PRE DOSE
42
How does sodium valproate work and what are the side effects?
MOA:
- Increases GABA activity
SE:
- Teratogenic (neural tube defects)
- P450 inhibitor
- Alopecia
- Ataxia/tremor
- Hepatotoxicity and pancreatitis
- Hyponatremia
- Encephlatophy secondary to high ammonia; treat with L-carnitine
- Weight gain
43
What are the red flag features for headache?
- Compromised immunity
- History of malignancy
- Vomiting
- Worse with fever
- Thunderclap
- New neurological deficit or cognitive fysfunction
- Recent head trauma
- ICP signs
- Symptoms suggestive of GCA or glaucoma
- Substantial change in characteristics
44
What are the features, diagnosis and management of cluster headache?
Features:
- Intense stabbing ocular pain
- Occurs in clusters lasting 4-12 weeks
- Assoc with redness, lacrimation, swelling and stuffiness
Diagnosis:
- MRI with gadolinium to r/o brain lesion
Management:
ACUTE - 100% oxygen + SV triptan
PROPHYLAXIS - verapamil
45
What are the features, diagnosis and management of migraine?
Features:
- Recurrent, severe headache
- May be assoc with aura, nausea and photosensitvity
Diagnosis:
- Clinical
Management:
ACUTE
1st line - Triptan (5-HT agonist)+ NSAID/paracetamol
2nd line - Metoclopramide/prochorperazine (non-oral)
PROPHYLAXIS (if prolonged/severe or >1 weekly):
- Propranolol in women
- Topiramate in men
- Amityptylline
- Acupuncture 2nd line
- Frovatriptan/zolmitriptan for menstrual irregularities
DON'T USE TRIPTAN IF HX OF IHD/CEREBROVASCULAR DISEASE
46
What are the features and management of medication overuse headache?
Features:
- Present for 15d or more monthly
- Common with opioids/triptans
Mangement:
- DISCONTINUE!
47
What are the features of ischaemic stroke?
Cerebral hemisphere infarct:
- Contralateral hemiplegia (initially flaccid then spastic)
- Contralateral sensory loss
- Homonymous hemianopia
- Dysphasia
Brainstem infarction:
- MORE SEVERE!
- Quadriplegia and lock-in syndrome
Lacunar infarction:
- Small infarcts around basal ganglia, internal capsule, thalamus and pons
- Pure motor, pure sensory, mixed motor and sensory signs or ataxia
- Strong association with hypertension
48
What is the Bamford classification of ischaemic stroke aka Oxford?
The following criteria should be assessed:
1. unilateral hemiparesis and/or hemisensory loss of the face, arm & leg
2. homonymous hemianopia
3. higher cognitive dysfunction e.g. dysphasia
Total anterior circulation infarcts (TACI 15%):
- Involves middle and cerebral arteries
- all 3 of above
Partial anterior circulation infarcts (PACI 25%):
- Involves smaller arteries of anterior circulation
- 2 of above
Lacunar infarcts (LACI 25%):
- Involves perforating arteries around internal capsule, thalamus and basal ganglia:
- 1 of unilateral weakness, pure sensory or ataxic hemiparesis
Posterior circulation infarcts (POCI 25%):
- Involves vertebrobasilar arteries:
- 1 of cerebellar/brainstem features, LOC or isolated HH
- Locked in syndrome if basilar artery
49
What are the common features of haemmorhagic stroke?
- Decreased consciousness
- Headache
- Nausea and vomiting
- Seizures
50
How is a suspected stroke managed?
Urgent non-contrast CT head to differentiate between ischaemic and haemorrhagic
ISCHAEMIC
- 300mg aspirin for 2 weeks
<4.5h - thrombolysis
<6h - thrombectomy
HAEMMORHAGIC
- Urgent NS opinion
- Stop anticoag/antiplats and lower BP
51
How is a TIA managed?
Immediate aspirin 300mg unless CI
If crescendo TIA or suspected cardioembolic source/carotid stenosis:
- Discuss urgently with stroke specialist ?for admission
Suspected TIA in last 7 days:
- Urgent stroke assessment within 24h
Suspected TIA >7d ago:
- Urgent stroke assessment within 7d
If they are on a blood thinner they need to go to ED straight away!
52
What are the absolute contraindications to thrombolysis?
- 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
53
When is thrombectomy offered in management of ischaemic stroke?
Within 6h:
Confirmed occlusion of proximal anterior circulation on CTA
(give alongside thrombolysis if within 4.5h)
Within 24h and known to be well previously if:
Confirmed occlusion of proximal anterior circulation on CTA and potential to salvage brain tissue
54
What secondary prevention is given after a stroke/TIA?
1st line - clopidogrel
2nd line - aspirin + MR dipyridamole life long
3rd line - MRI dipyridamole
55
When is carotid artery endarterectomy offered post stroke/TIA?
- Stroke in carotid territoy and not severely disabled
- If carotid stenosis >70%/50% depending on criteria
56
What is lateral medullary syndrome?
aka Wallenbergs syndrome
- Stroke to posterior inferior cerebellar artery
- Causes ipsilateral ataxia/nystagmus/dysphagia/numbness/CN palsy
- Causes contralateral limb sensory loss
57
What is Webers syndrome?
- Ipsilateral III CN palsy
- Contralateral weakness
58
What are the features of subdural haemmorhage?
- Collection of blood deep to the dural layer of the meninges (not actually within the brain itself)
- Typical lucid interval followed by gradual decline in consciousness/fluctuating consciousness
- May have papilloedema or unilateral dilated pupil (due to 3rd CN compression)
- CT often shows hyperdense crescentic lesion not limited by sututre lines
- Often bridging veins affected
- Elderly and alcohol pts at risk
- Management is conservative if small or chronic. May need decompressive craniectomy
59
What are the features of intracranial venous thrombosis?
Features:
- Headache, N&V, reduced consciousness
Investigation:
- MRI venography
- Often non-contrast CT head is normal
- Elevated d-dimer
Management:
- Anticoag (acutely with LMWH, warfarin long term)
60
What are the specific features of:
a) sagittal sinus thrombosis?
b) cavernous sinus thrombosis?
c) lateral sinus thrombosis
a) seizures, hemiplegia. Empty delta sign on venography
b) periorbital odema, ophthalmoplegia, trigem nerve
involvement, central retinal vein thrombosis
c) 6th and 7th CN palsies
61
What are the features of Bell's palsy? How is it managed?
Features:
- Unilateral facial nerve paralysis
- Due to lower motor neuron facial nerve palsy WITH FOREHEAD AFFECTED
- May have post-auricular pain, altered taste, dry eyes or hyperacusis
Management:
- Oral pred within 72h
- Consider adding antivirals if severe
- Artificial tars and eye lubricants to prevent exposure keratopathy
Follow-up:
- If ongoing after 3w > urgent ENT referral
- If ongoing after months > plastic surgery
62
What are the types of aphasia?
1. Wernicke's (receptive) aphasia
- lesion in superior temporal gyrus supplied by inferior left MCA
- word salad
- comprehension impaired
2. Broca's (expressive) aphasia
- lesion in inferior frontal gyrus
- supplied by superior left MCA
- non-fluent speech
- comprehension normal
3. Conduction aphasia
- stroke in arcuate fasiculus (connection between Wernicke's and Broca's)
- Speech and comprehension fluent
- Repetition!!! and errors speaking words
4. Global aphasia
- lesion in all 3 areas
- can communicate in gestures sometimes
63
What cause an anopia (monocular visual loss?
Lesion on ipsilateral optic nerve
64
What causes bitemporal hemianopia?
Lesion of optic chiasm (often due to a pituitary tumour)
65
What causes homonymous hemianopia?
1. Lesion of contralateral optic tract (incongrous)
2. Lesion of optic radiation or occipital cortex (congruous)
66
What causes homonymous quadrantanopia?
Superior: lesion of the inferior optic radiation in temporal lobe
Inferior: lesion of the superior optic radiation in the parietal lobe
PITS (parietal-inferior, temporal-superior)
67
What causes central scotoma?
Macular degeneration (unable to see in small spot in centre of vision)
68
What causes macular sparring (tunnel vision)?
Glaucoma
Retinitis pigmentosa
69
What are the features and management of Wernicke's encephalopathy?
Features:
- Triad of opthalmoplegia/nystagmus, ataxia and encephalopathy
- Secondary to thiamine (B1) deficiency in alcoholism/persistent vomiting/stomach cancer
- Decreased red cell transketolase
- Diagnose with MRI
Management:
- Urgent thiamine replacement
NOTE - INFUSION OF GLUCOSE WITHOUT THIAMINE IN A PT WITH CHRONIC THIAMINE DEFICIENCY CAN PRECIPITATE WERENICKES
70
What are the features and management of Korsakoff's syndrome?
Features:
- Develops as a progression of Wernicke's
- Addition of entero and retrograde amnesia and confabulation
Management:
- High dose thiamine
- Dementia care
71
What are the DVLA rules for syncope?
1. Simple faint: no restriction
2. Single episode, explained and treated: 4 weeks off
3. Single episode, unexplained: 6 months off
4. Two or more episodes: 12 months off
72
What are the DVLA rules for stroke/TIA?
1. Stroke/TIA - 1 month off driving, don't need to inform DVLA unless residual neurological deficit
2. Multiple TIAs over short period - inform DVLA, 3 months off
73
What are the DVLA rules for craniotomy?
1. For pituitary tumour - 6 months
2. For meningioma - 1 year (6m if benign and no seizures)
74
What is cataplexy and what are the DVLA rules?
Fainting triggered by emotional stimulus
Stop driving on dx, can restart once satisfactory symptom control achieved
75
What are the features and management of restless leg syndrome?
Features:
- Uncontrollable urge to move legs (akathisia) +/- parasthesia
- Associated with IDA, uraemia, DM, pregnancy
Management:
- Treat cause
- Dopamine agonists (pramipexole, ropinirole)
- Consider benzo or gabapentin if refractory
76
What are the features of Parkinson's disease?
- Degeneration of dopaminergic neurons in the substantia nigra
- Triad of bradykinesia, RESTING tremor and rigidity
- Symptoms are asymmetrical
- Clinical diagnosis +/- SPECT
77
What is the treatment for PD?
1st line:
1. Significant motor symptoms - Levodopa
2. Not significant - Dopamine agonist, levodopa or MAO-B inhibitor
2nd line - add in dopamine agonist, MAO-B or COMT inhibitor
78
What are the considerations with levodopa?
- Nearly always combined with carbidopa (reduces side effects)
- SE: dry mouth and psychosis
- May have end of dose wearing off, and dyskinesias at peak dose
- Do not acutely stop!! If unable to take tablets, given dopamine agonist patch to prevent acute dystonia
79
What are the considerations of dopamine receptor agonists?
eg. bromocriptine, ropinirole, cabergoline
- SE: pulmonary, retroperitoneal, cardiac fibrosis
- Must do CXR, ESR, eco, creatining before starting
- Risk of impulse control disorder and daytime sleepiness
- Likely to cause hallucinations
80
What are the considerations of MAO-B inhibitors?
eg. selegiline
- Inhibits breakdown of dopamine
81
What are the considerations of COMT inhibitors?
eg. entacapone, tolcapone
- Adjunct therapy to levodopa in patients with established PD
82
What can be used to control excessive daytime somnolence in medicated PD patients?
Modafinil
83
What can be used to prevent orthostatic hypotension in medicated PD patients?
Midodrine (increases arterial resistance)
84
What medication is used in drug induced PD?
Antimuscarinics (eg. procyclidine, benzotropine, benzhexol)
- Block cholinergic receptors
- Help tremor and rigidity
85
What can cause parkinsonism?
- PD
- Drugs (antipsychotics, metoclopramide)
- PSP
- MSA
- Wilson's disease
- Post-encephalitis
- Dementia puglistica (secondary to chronic head trauma)
- Toxins (carbon monoxide)
86
What is progressive supranuclear palsy?
Parkinsons plus syndrome
aka Steele-Rihcardson--Olszewski syndrome
- Postural instability and falls with stiff broad-based gait
- Impairment of vertical gaze (unable to read/descend stairs)
- Frontal lobe dysfunction
- Poor response to L-dopa
87
What is multiple system atrophy?
Parkinsons plus syndrome, two types:
1) MSA-P - predominantly parkinsonian
2) MSA-C - predominantly cerebrellar
Causes parkinsonism, autonomic (ED, OH, atonic bladder) and cerebellar signs
88
What is the most appropriate antiemetic to prescribe to PD patients?
Domperidone
89
What are the features and management of essential tremor?
Features:
- Postural tremor which is worse when arms outstretched
- Improved by alcohol and rest
- Causes head tremor (titubation)
Management:
1st line - propranolol
2nd line - primidone
90
What is idiopathic intracranial hypertension and how is it managed?
aka pseudotumour cerebri
Features:
- Classically young, overwheight female
- Can be due to COCP, steroids, tetracyclines, retinoids, lithium
- Headache, blurred vision, papilloedema, sixth nerve palsy
Management:
- Weight loss incl with semaglutide, topiramate, azetazolamide
- Repeated LP
- Optic nerve sheath decompression to prevent optic nerve damage
- Lumboperitoneal shunt to reduce ICP
91
What are the features and management of encephalitis?
Features:
- Fevers, headache, psychiatric symptoms, vomiting
- Due to HSV in 95% of patients
- Typically affects temporal and inferior frontal lobes
Investigation:
- CSF; lymphocytosis, high protein, PCR
- MRI
- EEG; lateralising periodic discharges at 2Hz
Management:
- IV aciclovir
92
What are the featurs and management of a brain abscess?
Features:
- Presents with dull headache, fever, focal neurology, raised ICP
- Due to sepsis, trauma, embolic events from IE
Management:
- Craniotomy + abscess debridement (risk of reformation as head is closed following drainage)
- IV abx: 3rd gen cephalosporin (ceftriaxone) + metronidazole
- ICP management with dex
93
What are the features of a common peroneal nerve lesion?
aka common femoral nerve
- Foot drop
- Injury at neck of fibula
- Weakness of extensor hallucis longus
- Muscle wasting
94
What are the mnemonics for remembering spinal innervation?
1, 2 buckle my shoes - S1 S2 ankle jerk
3, 4 kick the door - L3 L4 knee jerk
5, 6 pick up sticks - C5, C6 biceps and brachioradialis
7, 8 shut the gate - C7 C8 triceps
C5 C6 C7 raise your arms to heaven
C3 C4 C5 keeps the diaphragm alive
S2 S3S4 keeps the pee/poo/penis off the floor
95
What is capgras syndrome?
A disorder in which a person has a fixed delusion that their partner/family has been replaced by an imposter
96
What is ataxia telangectasia?
- Autosomal recessive disorder causing defect in ATM gene which encodes for DNA repair enzymes
- Presents in childhood with cerebellar ataxia and telangectasia
- IgA deficiency causing recurrent chest infection
- Risk of malignancy
97
What is Friedreich's ataxia?
- Most common early onset hereditary ataxia
- Autosomal recessive, trinucelotide repeat disorder
- GAA repeat in X25 gene on chromosome (frataxin) but does not demonstrate anticipation
- Age of on set 10-15y old
- Presents with gait ataxia and kyphoscoliosis
- Associated with HCOM, DM and high arched palate
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What is Charcot-Marie-Tooth disease?
- Most common hereditary peripheral nueropthay
- Predoinantly motor (frequently sprained ankles, foot drop, high arched feet, muscle weakness, hammer toes, stork leg deformity)
- Management is OT/PT
99
What are the features of the dystrodinopathies?
- X-linked recessive
- Mutation in dystrophin gene on Xp21
Duchenne muscular dystrophy:
- Frameshift mutation leading to a severe form
- Progressive muscle weakness from 5yo
- Calf pseudohypetrophy
- Gowers sign (child uses arms to stand up from a squatted position)
- Intellectual impairment
Becker muscular dystrophy:
- Non-frameshift insertion leading to a milder form
- Develops after 10yo
- Intellectual impairment less common
100
What are the features and management of Lambert-Eaton syndrome?
Features:
- Antibody against calcium channel in PNS
- Associated with SCLC, may be independent autoimmune disorder
- Causes repeated muscle contraction and increased strength which eventually decreases
- Autonomic symptoms WITHOUT involving respiratory muscles or facial muscles
Management:
- Treat underlying cancer
- Immunosuppresion with steroids, azathioprine
- IVIG and plasma therapy
101
What are the features and management of myasthenia gravis?
Features:
- Chronic AI neuromuscular disease of the post synaptic membrane causing weakness in the voluntary muscles
- Muscle weakness the increases with exercise
- Drooping eyelids, double vision, SOB
Investigations:
- Elevated AChR or MuSK
- Repetitive nerve stimulation and EMG
Management:
- Pyridostigmine to reduce muscle weakness
- Steroids and immunosuppressants once disease becomes generalised
- Thymectomy
- IVIG/plasma if myasthenic crisis
102
What are the features and management of normal pressure hydrocephalus?
Features:
- Triad of urinary incontinence, dementia and gait abnormality
- Due to reduced CSF absorption at arachnoid villi (head injury, SAH, meningitis)
Investigations:
- CT shows hydrocephalus with ventriculomegaly
Management:
- Ventriculoperitoneal shunting
- Risk of seizures, infection and ICH with shunt
103
What are the features and management of syringomyelia?
Features:
- Collection of CSF within spinal cord
- Due to chiari malformation/trauma/tumour
- Loss of temperature sensation (classically will burn their hands without realising)
- Spastic weakness and neuropathic pain
Investigation:
- Full spine + brain MRI
Management:
- Treat cause
- Shunt in recurrent
104
What is tuberous sclerosis?
- Autosomal dominant
- Causes neurocutaneous features
- Ash leaf spots, epilepsy (incl lennox-gastaut syndrome), retinal hemartomas
105
What is neurofibromatosis?
- Autosomal dominant
- Neurocutaneous disorder
- Cafe au lait spots, phaeochromocytomas, acoustic neuromas
106
What is hemiballismus?
Involuntary flinging motions of the extremities due to infarction/haemorrhage in the ceontralateral subthalamic nucleus
107
What is chorea?
Involuntary, irregular, random and flowing movements which flit from one part of the body to another
108
What are the features of facioscapulohumeral muscular dystrophy?
- Autosomal dominance inheritance
- Usually presents in 1st/2nd decade of life
- Muscle involvement starts in face (unable to purse lips/drink through straw) then progresses to shoulder, humerus, abdomen and legs
- Winging of scapula is a characteristic sign
109
What is Ogilvie syndrome?
Acute colonic pseudo-obstruction in the absence of mechanical obstruction
Typilcally in critically ill patients and characterised by massive dilatation of the caecum
