Neuro 7.5 Flashcards
(368 cards)
1
Q
Causes of raised ICP
A
Vascular (aneurysm, AV malformation, SDH, IVH, sinus thrombosis)
Infective (abscess, meningitis, encephalitis)
Traumatic
Iatrogenic (some meds, post-surgical)
Idiopathic intracranial HTN
Neoplasia/Tumour
Degenerative
2
Q
Examples & adverse effect of 5-HT3 antagonists?
A
- ondansetron & granisetron
- antiemetics usually used in chemo-related nausea
- mainly act in chemoreceptor trigger zone in medulla oblongata
- constipation is common
3
Q
Features of absence seizures?
EEG?
Rx?
A
- form of generalised epilepsy mostly seen in childrenm typical onset 3-10yrs girls 2x boys
- absences last a few seconds, ass with a quick recovery
- may be provoked by hyperventilation/stress
- child usually unaware of the seizure, may occur many times/day
EEG: bilateral, symmetrical 3Hz spike & wave pattern
Rx: sodium valproate & ethosuximide 1st line
- good prognosis
4
Q
Features & Rx of acute confusional state?
A
- fluctuating
- memory disturbances, loss of short>long term
- disorientation
- agitated/withdrawn
- mood change
- visual hallucinations
- disturbed sleep cycle
- poor attention
Rx underlying cause, modify environment
- 1st line sedative is haloperidol
5
Q
What is acute disseminated encephalomyelitis?
What are the features
What might MRI show?
Rx?
A
ADEM: autoimmune demyelinating disease of CNS
- aka post-infectious encephalomyelitis
- e.g. measles, mumps, rubella, varicella, small pox
- acute onset of multifocal neuro Sx with rapid deterioration after a lag time of few days - 2months
- headache, fever, nausea, vomiting
- motor & sensory deficits
- amy also be brainstem involvement inc oculomotor defects
- MRI: may show areas of supra & infra-tentorial demyelination
- Rx: IV glucocorticoids, consider IV Ig when this fails
6
Q
Genetics of Alzheimer’s disease?
A
- most sporadic, 5% autosomal dominant
- mutations can occur in the amyloid precursor protein (chr 21), presenilin 1 (chr 14) & presenilin 2 (chr 1) in inherited form
- apoprotein E allele E4 - encodes a cholesterol transport protein
7
Q
Pathological changes in Alzheimer’s disease:
- macroscopic?
- microscopic?
- biochemical?
A
Macro: widespread cerebral atrophy esp involving the cortex & hippocampus
Micro: cortical plaques due to deposition of type A-beta-amyloid protein & intraneuronal neurofibrillary tangle caused by abnormal aggregation of the tau protein
Biochemical: Acetylcholine deficit from damage to an ascending forebrain projection
8
Q
What are neurofibrillary tangles in Alzheimer’s disease?
A
- paired helical filaments that are partly made from a tau protein - abnormal aggregation of tau protein as they are excessively phosphorylated
9
Q
Drug Rx in Alzheimer’s disease:
if mild-moderate?
A
Acetylcholinesterase inhibitors
e.g. donepezil, galantamine, rivastigmine
10
Q
Drug Rx in Alzheimer’s disease:
2nd line if 1st line intolerant or a C/I or as an add-on if moderate-severe or as mono therapy in severe disease?
A
NMDA receptor antagonist: Memantine
11
Q
What is Anti-NMDA receptor encephalitis? How does it present? What tumours are detected in nearly half of female adult patients? What may be shown on MRI? What may be shown in the CSF? How can it be treated?
A
A paraneoplastic syndrome, presenting as prominent psychiatric features e.g.: agitation, hallucinations, delusions & disordered thinking, seizures, insomnia, dyskinesias & autonomic instability
Ovarian teratomas in females, esp Afro-C
MRI head normal or: abnormalities can be visualised on FLAIR sequences in the deep subcortical limbic structures
CSF: pleiocytosis (high lymphocytes), but can be normal initially
Rx: immunosuppression with IV steroids, Ig, rituximab, cyclophosphamide or plasma exchange
Resection of teratoma can also be therapeutic
12
Q
Anti-MuSK is an autoAb specific to what?
A
specific to muscle kinase in myasthenia graves with no evidence of a thymoma and without Ab to acetylcholine receptors
13
Q
Anti-GM1 is an autoAb specific to what?
A
AIDP: acute inflammatory demyelinating polyneuropathy, a variant of Guillain-Barre
14
Q
Embryologically, what does the facial VIIth nerve supply?
efferents?
afferents?
What does it supply?
face ear taste tear
A
- the structures of the 2nd embryonic branchial arch
- efferent to muscles of facial expression, digastric muscle & also many glandular structures
- contains a few afferent fibres which originate in the cells of its genicular ganglion concerned with taste
face: muscles of facial expression
ear: nerve to stapedius
taste: anterior 2/3 tongue
tear: parasympathetic fibres to lacrimal glands, also salivary glands
15
Q
Causes of a BL facial nerve palsy?
A
- Bell’s i.e. idiopathic
- sarcoidosis
- Lyme disease
- Guillain-Barre syndrome
- BL acoustic neuromas (NF type 2)
16
Q
Causes of a unilateral lower motor neurone facial VIIth nerve palsy?
A
- Bell’s palsy
- Ramsay-Hunt (herpes zoster)
- acoustic neuroma
- parotic tumours
- HIV
- multiple sclerosis
- diabetes mellitus
17
Q
What is the pass of the facial VIIth nerve:
Subarachoid origin?
Subarachnoid pathway?
Facial canal path?
What are the 3 branches?
And when it passes through the stylomastoid foramen?
A
Subarachnoid origin: - motor pons - sensory nervus intermedius Subarachnoid pathway: - through the petrous temporal bone into the internal auditory meatus with the vestibulocochlear VIIIth nerve -> here the motor & sensory component combine to form the facial nerve
Facial canal path:
- the canal passes superior to the vestibule of the inner ear
- at the medial aspect of the middle ear -> becomes wider & contains the geniculate ganglion
Branches:
- greater petrosal nerve
- nerve to stapedius
- chora tympani
Stylomastoid foramen:
- it passes through this foramen (tympanic cavity anterior & mastoid antrum posteriorly)
- posterior auricular nerve & branch to posterior belly of digastric & stylohyoid muscle
18
Q
Types of 1ry brain injury?
A
Focal:
- contusion (adjacent to i.e. coup or contralateral i.e. contra-coup to the side of impact)
- or haematoma - extradural/subdural/intracerebral
Diffuse: diffuse axonal injury occurs as a result of mechanical shearing following deceleration, causing disruption & tearing of axons
19
Q
Types of 2ry brain injury?
A
- i.e. when cerebral oedema, ischaemia, infection, tonsillar or tectorial herniation exacerbates the original injury
- the normal cerebral auto-regulatory processes are disrupted following trauma, rendering the brain more susceptible to blood flow changes & hypoxia
- Cuching’s reflex of hypertension & bradycardia, is late & pre-terminal
20
Q
Acc-deceleration trauma or blow to head , with features of raised intracranial pressure and sometimes a lucid interval, involves what type of brain injury?
Can often be in the temporal region where a skull fracture can rupture which artery?
A
Extradural haematoma
- between dura mater & skull
- middle meningeal artery rupture
21
Q
Brain injury with slower onset of Sx, most commonly occur around the frontal & parietal lobes?
What are the RFs?
A
Subdural haematoma
- outermost meningeal layer
- old age, alcoholism, anticoagulation
22
Q
Sudden occipital headache of a brain injury, usually spontaneously in the context of a ruptures cerebral aneurysm or in ass with other injuries?
A
Subarachnoid haemorrhage
23
Q
What is myasthenia gravis?
A
- autoimmune disorder resulting in insufficient functioning acetylecholine receptors
- Ab to Ach Rs seen in 85-90% of cases (less common in disease limited to ocular muscles)
- more common in women 2:1
24
Q
What are the key features of myasthenia gravis?
What are the 3 associations?
A
Muscle fatigability - where they get progressively weaker during periods of activity, & slowly improve after periods of rest
- extraocular weakness: diplopia
- proximal weakness: face, neck, limb girdle
- ptosis
- dysphagia
Ass:
- thymomas 15%
- autoimmune disorders: pernicious anaemia, AI thyroid, rheumatoid, SLE
- thymic hyperplasia 50-79%
25
```
Ix in myasthenia gravis:
what is the most sensitive?
what scan is done to exclude a thymoma?
which autoAb are present in most, & in the others?
what is the Tensilon test?
```
Single fibre electromyography 92-100% sensitive
CT thorax to exclude thymoma
AutoAb: 85-90% have Ab to acetylcholine receptors - in the rest, 40% are positive for anti-muscle-specific tyrosine kinase Ab
Tensilon test: IV edrophonium reduces muscle weakness temporarily BUT has a risk of cardiac arrhythmia
26
Management of myasthenia gravis in the long-term (3)?
1. Long-acting anticholinesterase e.g. Pyridostigmine
2. Immunosuppression - initially pred
3. Thymectomy
27
Rx of a myasthenic crisis?
- plasmapheresis
| - IV immunoglobulins
28
What drugs may exacerbate myasthenia gravis?
- penicillamine
- quinidine, procainamide
- beta-blockers
- lithium
- phenytoin
- Abx: gentamicin, macrolides, quinolones, tetracyclines
29
When can anti-epileptic drugs be stopped & how?
If seizure-free for >2 years, can be stopped over 2--3months
30
When to start anti epileptics after the 1st seizure?
- neuro deficit
- structural abnormality on brain imaging
- EEG shows unequivocal epileptic activity
- pt/family/carers consider risk of having a further seizure unacceptable
31
1st line drug Rx for Focal/partial seizures?
| 2nd line?
Carbamazepine or Lamotrigine
| 2nd line: levetiracetam, oxcarbazepine, valproate
32
1st line drug Rx with generalised seizures?
sodium valproate
33
2nd line drug Rx in generalised tonic-clonic seizures?
Lamotrigine, Carbamazepine
34
1st & 2nd line drug Rx in generalised absence seizures?
1st: sodium valproate/ethosuximide
Valproate is particularly effective if co-existent tonic-clonic seizures in 1ry generalised epilepsy
(carbamazepine may actually exacerbate absence seizure)
35
2nd line drug rx in myoclonic seizures?
clonazepam or lamotrigine
36
1st line Rx of neuropathic pain?
2nd line what to do?
What can be used as rescue therapy for exacerbations?
What can be used for localised neuropathic pain?
1st: amitriptyline/duloxetine/pregabalin/gabapentin
2nd: try 1 of the other 3
Rescue: Tramadol
Localised: topical capsaicin
37
What is otosclerosis?
What are the features?
What is the Rx?
- replacement of normal bone by vascular spongy bone
- progressive conductive deafness due to fixation of the stapes at the oval window
- autosomal dominant, typically affecting young adults 20-40yrs onset
- conductive HL
- tinnitus
- normal tympanic membrane +/- flamingo tinge from hyperaemia 10%
- +ve FHx
Rx:
- hearing aid
- stapedectomy
38
Incongruous visual field defect - where is the lesion?
optic tract
39
Congruous (symmetrical) visual field defect - where is the lesion?
optic radiation or occipital cortex
40
Homonymous hemianopia with macular sparing - where is the lesion?
Occipital cortex
41
Superior homonymous quadrantanopia - where is the lesion?
Temporal lobe
42
Inferior homonymous quadrantanopia - where is the lesion?
Parietal lobe
43
Bitemporal hemianopia - where is the lesion?
Optic chiasm
44
Bitemporal hemianopia: upper quadrant defect > lower quadrant defect - where is the lesion?
Inferior chiasmal compression
| = pituitary tumour
45
Bitemporal hemianopia: lower quadrant defect > upper quadrant defect - where is the lesion?
Superior chiasmal compression
| = craniopharyngioma
46
Headache + fever
CT brain: Petechial haemorrhages in the temporal & inferior frontal lobes. No mass effect. Brain parenchyma otherwise normal
What is the Dx?
Herpes simplex encephalitis
| think temporal lobe changes - HSV
47
Features & pathophysiology of herpes simplex encephalitis?
- fever, headache, psychiatric Sx, seizures, vomiting
- focal e.g. aphasia (temporal lobe features)
- HSV-1 responsible in 95%
- typically affects temporal & inferior frontal lobes
48
```
Ix in HSV encephalitis:
CSF?
confirm HSV?
CT?
what scan is better?
EEG?
Rx?
```
CSF: lymphocytosis, elevated protein
PCR for HSV
CT: medial temporal & inferior frontal changes e.g. petechial haemorrhages (but normal in 1/3)
MRI is better
EEG: lateralised periodic discharges at 2Hz
Rx: IV aciclovir
49
What is myotonic dystrophy?
| What are the genetics?
- inherited myopathy with features developing 20-30yrs old
- affects skeletal, cardiac & smooth muscle
- autosomal dominant
- trinucleotide repeat disorder
2 types DM1 & DM2
50
Differences between DM1 & DM2 myotonic dystrophy?
DM1:
- CTG repeat at the end of the DMPK (dystrophia myotonic-protein kinase) gene on chromosome 19
- DISTAL weakness is more prominent
DM2:
- repeat expansion of the ZNF9 gene on chromosome 3
- PROXIMAL weakness more prominent
- severe congenital form not seen
51
General & other features of myotonic dystrophy?
General:
- myotonic facies (long, haggard)
- frontal balding
- BL ptosis
- cataracts
- dysarthria
Other:
- myotonia: tonic spasm of muscle
- weakness of arms & legs (distal initially)
- mild mental impairment
- diabetes mellitus
- testicular atrophy
- cardiac involvement, heart block, cardiomyopathy - prolonged PR interval in 20-40%
- dysphagia
52
What is ataxic telangiectasia?
What are the features?
- autosomal recessive disorder caused by a defect in the ATM gene encoding DNA repair enzymes
- inherited combined immunodeficiency disorder
- typically presents in early childhood with abnormal movements
Features:
- cerebellar ataxia
- telangiectasia (spider angiomas)
- IgA deficiency -> recurrent chest infections
- 10% risk of developing malignancy, lymphoma or leukaemia, but also non-lymphoid tumours
53
Drug causes of tinnitus?
Aspirin
Aminoglycosides
Loop diuretics
Quinine
54
Cause of tinnitus ass with: Associated with hearing loss, vertigo, tinnitus and sensation of fullness or pressure in one or both ears?
Meniere's disease
55
Cause of tinnitus ass with: Hearing loss, vertigo, tinnitus?
- Absent corneal reflex is important sign
- Ass with neurofibromatosis type 2
Acoustic neuroma
56
Features of essential tremor?
| Rx?
- autosomal dominant
- postural tremor worse if arms outstreched
- improved by etoh & rest
- most common cause of titibation (head tremor)
Rx Propranolol 1st
- sometimes primidone is used
57
Head injury criteria for immediate CT head?
- initial GCS < 13
- 2h post injury GCS < 15
- suspected open/depressed skull #
- any sign of a basal skull #
- post-traumatic seizure
- focal neurological deficit
- >1 episode of vomiting
58
Head injury criteria for CT head within 8h of injury - for adults with which risk factors who have experinces some loss of consciousness or amnesia since injury?
- age 65+
- any Hx bleeding/clotting disorders
- dangerous mechanism of injury
- >30mins retrograde amnesia of events immediately prior to injury
59
If a patient is on warfarin who have sustained a head injury with no other indications for a CT head scan, when should one be done?
Within 8hours
60
What is the characteristic pathological feature in Lewy body dementia?
Alpha-synuclein cytoplasmic inclusions (Lewy bodies) in the substantia nigra, paralimbic & neocortical areas
- N.b. upto 40% Alzheimer's pts have lewy bodies
61
What durgs should be avoided in Lewy body dementia and why?
Neuroleptics e.g. haloeridol
| - pts are v sensitive and may develop irreversible parkinsonism
62
What are the features of Lewy body dementia?
1. Progressive cognitive impairment i.e. dementia
2. Parkinsonism
3. Visual hallucinations (delusions & non-visual hallucinations may also be seen)
63
Single best Dx Ix for Lewy body dementia?
SPECT/DaT scan
- radioisotope is dopaminergic
- sensitivity 90%, spec 100%
64
Rx of Lewy body dementia?
- Acetylcholinesterase inhibitors e.g. donepezil, rivastigmine & Memantine can be used as in Alzheimer's
65
Wernicke's aphasia -
what type of aphasia is it?
where is the lesion?
Receptive aphasia
- lesion of superior temporal gyrus
- impaired comprehension, word substitutions, nonsense, neologisms but fluent speech
66
Broca's aphasia -
what type of aphasia is it?
where is the lesion?
Expressive aphasia
- lesion of inferior frontal gyrus
- supplied by middle cerebral artery
- normal comprehension but speech is non-fluent, laboured & halting
67
Conduction aphasia - where is the lesion?
| What happens to speech?
- stroke affecting the arcuate fasiculus (connection between Wernicke's & Broca's area)
- normal comprehension, fluent speech but repetition is poor, aware of the errors they are making
68
What happens in global aphasia?
large lesion affecting all 3 areas of: superior temporal gyrus, inferior frontal gyrus & arcuate fasiculus, resulting in severe expressive & receptive aphasia
69
What is the MoA of triptans?
When should they be taken?
What are the adverse effects?
What are the C/I?
- 5-HT1 agonists
- in acute Rx of migraine: should be taken asap after onset of headache, rather than at onset of aura
- oral, orodispersible, nasal spray & SC injections
- 'triptan sensations' - tingling, heat, tightness, heaviness, pressure
- C/I: pts with a Hx/significant RFs for IHD or cerebrovascular disease
70
Acute Rx of migraine?
if young?
if not effective/not tolerated?
what do you need to be careful with with young people?
1st: combo triptan + nsaid/paracetamol
young 12-17yrs: NASAL triptan in preference
if above not effective/not tolerated offer a non-oral prep of metoclopramide/prochlorperazine & consider adding a non-oral NSAID/triptan
- caution metoclopramide in young people due to acute dystonic reactions
71
Migraine prophylaxis Rx:
when to give Rx?
what Rx?
if these measures fail?
what may be effective in reducing migraine frequency & intensity for some people?
what is better for women with predictable menstrual migraine for Rx?
- if 2+ attacks/month, effective in 60%
- Topiramate/Propranolol - preferred in women of childbearing age as may be teratogenic & can reduce efficacy of hormonal contraceptives
- if fails: course of acupuncture over 5-8wks or Gabapentin
- Riboflavin 400mg OD may be effective in reducing migraine frequency & intensity for some
- predictable menstrual migraine: Frovatriptan or Zolmitriptan as a mini-prophylaxis
72
What is pituitary apoplexy?
| What are the features?
= sudden enlargement of pituitary tumour 2ry to haemorrhage/infarction
- sudden onset headache similar to SAH
- neck stiffness, vomiting
- visual field defects: classically bitemporal superior quadrantanopia defect
- extraocular nerve palsies
- features of pituitary insufficiency e.g. hypotension 2ry to hypoadrenalism
73
What features are seen in a parietal lobe lesion?
- sensory inattention
- apraxias
- astereogenesis (tactile agnosia)
- inferior homonymous quadrantanopia
- Gerstmann's syndrome: alexia, acalculia, finger agnosia & R-L disorientation
74
Where is the lesion with features of:
- sensory inattention
- apraxias
- astereogenesis (tactile agnosia)
- inferior homonymous quadrantanopia
- Gerstmann's syndrome: alexia, acalculia, finger agnosia & R-L disorientation??
Parietal lobe
75
What is Gerstmann's syndrome?
Lesion of the dominant parietal lobe:
- alexia
- acalculia
- finger agnosia
- R-L disorientation
76
What are the features in an occipital lobe lesion?
- homonymous hemianopia (with macula sparing)
- cortical blindness
- visual agnosia
77
Where is the lesion with features of:
- homonymous hemianopia (with macula sparing)
- cortical blindness
- visual agnosia
Occipital lobe
78
What are the features of a temporal lobe lesion?
- Wernicke's aphasia (receptive)
- superior homonymous quadrantanopia
- auditory agnosia
- prosopagnosia (difficulty recognising faces)
79
Where is the lesion with features of:
- Wernicke's aphasia (expressive)
- superior homonymous quadrantanopia
- auditory agnosia
- prosopagnosia (difficulty recognising faces)
Temporal lobe lesion
80
What are the features of a frontal lobe lesion?
- Broca's aphasia (expressive)
- disinhibition
- perseveration
- anosmia
- inability to generate a list
81
Where is the lesion with features of:
- Broca's aphasia (expressive)
- disinhibition
- perseveration
- anosmia
- inability to generate a list
Frontal lobe
82
What are the features of a cerebellum lesion?
Midline lesion: gait & truncal ataxia
| Hemisphere lesion: intention tremor, past pointing, dysdiadokinesis, nystagmus
83
Which area of the brain is associated with Wernicke & Korsakoff syndrome?
Medial thalamus & mammillary bodies of hypothalamus
84
Which area of the brain is associated with hemiballism?
| involuntary, violent, coarse & wide-amplitude movements of the ipsilateral UL & LL
Subthalamic nucleus of the basal ganglia
85
Which part of the brain is associated with Huntington chorea?
Striatum (caudate nucleus) of the basal ganglia
86
Which part of the brain is associated with Parkinson's disease?
Sunstantia nigra of the basal ganglia
87
Which part of the brain is associated with Kluber-Bucy syndrome: hyper sexuality, hypoerorality, hyperphagia & visual agnosia?
Amygdala
88
What is the genetics of Friedreich's ataxia?
- commonest of the early-onset hereditary ataxias
- autosomal recessive, trinucleotide repeat disorder characterised by a GAA repeat in the X25 gene on chr 9 (frataxin)
- does not demonstrate anticipation
89
What are the most common presenting features of Friedreich's ataxia?
- gait ataxia & kyphoscoliosis
| - usually age 10-15 onset
90
Neurological features of Friedreich's ataxia?
| What are the other features?
- cerebellar ataxia
- absent ankle jerks/extensor planters
- optic atrophy
- spinocerebellar tract degeneration
- HOCM 90% (commonest cause of death)
- diabetes 10-20%
- high-arched palate/feet
91
What is neuroleptic malignant syndrome?
- rarely seen in those taking antipsychotics with mortality of 10% with atypicals
- can also occur with dopaminergic drugs e.g. levodopa, usually when suddenly stopped or dose reduced
92
Features of neuroleptic malignant syndrome?
Rx?
- more common in young males
- onset usually in first 10days of Rx or after increasing dose
- fever, rigidity, tachycardia
- raised CK & leukocytosis may be seen
- reduced reflexes
Rx:
- stop antipsychotic, IV fluids to prevent AKI
- DANTROLENE
- bromocriptine may also be used
93
Sodium valproate risk in pregnancy
neural tube defects
neurodevelopmental delay
- do not use in pregnancy OR childbearing age women unless clearly necessary
94
Carbamazepine risk in pregnancy?
least teratogenic of the older anti epileptics
95
Phenytoin risk in pregnancy
- ass with cleft palate
| - give vitamin K in last month of pregnancy to prevent clotting disorders in the newborn
96
Lamotrigine risk in pregnancy
- dose may need to be increased in pregnancy
| - rate of congenital malformations may be low
97
Anti epileptics when breastfeeding
- safe (except for barbiturates)
98
When is donepezil contra-indicated for Alzheimer's?
Cardiac disease inc sick sinus syndrome, supra ventricular conduction abnormalities etc due to increased risk of bradycardia & AV node block
- after initiating, recheck pulse etc
- also C/I if severe hepatic impairment
99
What is the triad of normal pressure hydrocephalus?
1. urinary incontinence
2. dementia & bradyphrenia
3. gait abnormality (may be similar to PD)
100
What is the cause of normal pressure hydrocephalus?
| What is shown on imaging?
- 2ry to reduced CSF absorption at the arachnoid villi
- changes may be 2ry to head injury, SAH or meningitis
- hydrocephalus with an enlarged IVth ventricle
- also typically an absence of substantial sulcal atrophy
101
Rx of normal pressure hydrocephalus?
- ventriculoperitoneal shunting
| - 10% with shunts experience significant complications e.g. seizures, infection & intracerebral haemorrhages
102
What is hemiballism?
Which part of the brain is damaged?
What happens during sleep?
Rx?
- involuntary, sudden, jerking movements contralateral to side of the lesion, 1rily affecting proximal limb musculature whilst detail muscles display more choreiform-like movements
- subthalamic mucleus
- Sx decrease whilst asleep
- antidopaminergic agents e.g. haloperidol
103
What is Erb-Duchenne palsy?
- damage to upper trunk of brachial plexus (C5, C6)
- arm hangs by the side, internally rotated, elbow extended, waiter's tip
- may be 2ry to shoulder dystocia
104
What is Klumpke's injury? (T1, due to traction)
- damage of lower trunk of brachial plexus (C8, T1)
- paralysis of all intrinsic hand muscles
- loss of sensation in medial aspect of hand & forearm
- may be caused by a sudden upward jerk of the hand, or should dystocia
- ass with Horner's syndrome
105
What is the motor component of the long thoracic nerve, C5-C7?
What happens when damaged?
- serratus anterior
| - winged scapula, e.g. sports injury or mastectomy complication
106
What is the motor & sensory components of the ulnar nerve C8-T1?
What happens if damaged?
M: all intrinsic hand muscles (except LOAF) & wrist flexion
S: medial 1.5 fingers
- e.g. medial epicondyle fracture leading to a claw hand
107
What is the motor component of the medial nerve C6, C8 & T1?
Sensory?
Lateral 2 lumbricals
Opponens pollis
Abductor pollis brevis
Flexor pollis brevis
S: palmar aspect of lateral 3.5 fingers
108
What happens in damage to the median nerve C6, C8, T1?
wrist lesion:
- carpal tunnel syndrome
- paralysis of thinner muscles, opponent pollicis
elbow: loss of pronation of forearm & weak wrist flexion
109
What is the motor & sensory component of the radial nerve C5-C8?
What happens with injury?
M: extension of forearm, wrist, fingers, thumb
S: small area between dorsal aspect of 1st & 2nd metacarpals
e.g. humeral midshaft fracture causing palsy results in wrist drop
110
What is the motor & sensory component of the axillary nerve C5-C6?
What happens with injury?
M: shoulder abduction (deltoid)
S: inferior region of deltoid muscle
e.g. humeral neck fracture/dislocation leads to flattened deltoid
111
What is the motor & sensory component of the musculocutaneous nerve C5-C7?
What happens with injury?
M: elbow flexion (biceps brachii) & supination
S: lateral part of forearm
- isolated injury is rare - usually injured as part of brachial plexus injury
112
Bitemporal hemianopia with headaches in children - what is commonest cause?
Craniopharyngioma: derived from Rathke's pouch, often invades the pituitary & hypothalamus (often ventromedial area)
113
Which part of the hypothalamus is responsible for synthesis of ADH & oxytocin? (which are transported to posterior hypothalamus for storage & release)
supraoptic nucleus & paraventricular nucleus of hypothalamus
114
Which part of the hypothalamus is responsible for heat generation to maintain core body temperature?
posterior hypothalamus
115
Which part of the hypothalamus is responsible for heat dissipation to cool down the body to prevent a rise in temp which would be detrimental to body's internal environment?
Anterior hypothalamus
116
Which part of the hypothalamus is often invaded by craniopharyngiomas? It also controls satiety centre & removed during the surgery -> leading to uninhibited hunger causing significant weight gain
Ventromedial area of hypothalamus
117
Dx of multiple sclerosis?
2+ relapses with objective clinical evidence 2+ lesions or 1 lesions with reasonable historical evidence of a previous relapse
i.e. lesions disseminated in time & space
118
```
Visual Sx/signs of multiple sclerosis?
Motor?
Sensory?
Cerebellar?
Other?
```
- optic neuritis, optic atrophy
- internuclear ophthalmoplegia
- Uhthoff's phenomenon (worsening vision after rise in body temp)
- UMN signs: spastic weakness, usually legs
- pins, needles, numbness, trigeminal neuralgia
- Lhermitte's: paraesthesia in limbs on neck flexion
- cerebellar ataxia (usually seen during acute relapse), tremor
- urinary incontinence
- sexual dysfunction
- intellectual deterioration
119
What is the eponymous name for when scratching of the inner side of leg leads to extension of the toes = a sign of cerebral irritation
Oppenheim's sign
120
What are the RFs for degenerative cervical myelopathy DCM?
- smoking (effects on intervertebral discs)
- genetics
- occupation (exposure to high axial loading)
- Asian populations have a higher rate of ossification of the posterior longitudinal ligament (OPLL), which can result in myelopathy
121
DCM: degenerative cervical myelopathy
- early Sx often subtle & can vary in severity day to day, making the disease difficult to detect initially (Dx delayed)
- progressive, worsening, deteriorating or new Sx should be a warning sign
- what are the Sx?
- Pain (neck/UL/LL)
- loss of motor function (digital dexterity, gait & imbalance)
- loss of sensory function (numbness)
- loss of autonomic function (urinary/faecal incontinence/impotence - don't necessarily suggest caudal equina)
122
Gold standard Ix for (degenerative) cervical myelopathy?
MRI - may reveal disc degeneration & ligament hypertrophy, with accompanying cord signal change
- urgent referral to neurosurgery
- timing of decompressive surgery v important (within 6months has best chance of recovery) - shown to prevent disease progression
123
What is Meniere's disease?
| What is the natural Hx?
- disorder of inner ear characterised by excessive pressure & progressive dilation of the endolymphatic system
- Sx resolve in majority after 5-10yrs but majority left with a degree of hearing loss
- psychological distress common
124
What are the features of Meniere's disease?
| Rx?
- recurrent episodes of vertigo, tinnitus & hearing loss (sensorineural) - vertigo is the prominent Sx
- sensation of aural fullness/pressure also common
- +/- nystagmus & +ive Romberg test
- episodes last mins-hours
- typically UL but BL can develop
125
What is herpes zoster ophthalmicus?
What are the features?
Complications?
- reactivation of the varicella zoster virus in the area supplied by the ophthalmic division of the trigeminal nerve
- 10% of shingles
- vesicular rash around the eye
- Hutchinson's sign: rash on the tip/side of the nose - indicates nasociliary involvement & is a strong RF for ocular involvement
- ocular: conjunctivitis, keratitis, episcleritis, anterior uveitis
- ptosis
- post-herpetic neuralgia
126
What is Ramsay Hunt syndrome?
Features?
Rx?
= herpes zoster oticus, caused by the reactivation of the varicella zoster virus in the geniculate ganglion of the VOOth cranial nerve
- auricular pain often 1st
- facial nerve palsy
- vesicular rash around the ear
- vertigo, tinnitus
Rx: oral aciclovir, steroids
127
What is trigeminal neuralgia?
Pain syndrome of severe UL pain - majority idiopathic but compression of trigeminal roots by tumours/vascular problems may occur
- UL disorder characterised by brief electric shock-like pains, abrupt in onset & termination, limited to 1+ divisions of trigeminal nerve
- commonly evoked by light touch with trigger factors, frequently occurs spontaneously
- small areas in the nasolabial fold/chin q susceptible to triggers
- pain usually remits for variable periods
128
Rx for trigeminal neuralgia?
Carbamazepine
| - refer to neurology if failure to respond to Rx or atypical features e.g. <50y.o.
129
When you suspect trigeminal neuralgia, what are red flag symptoms that may suggest an underlying cause?
- sensory changes
- deafness/other ear problems
- Hx of skin/oral lesions
- pain only in ophthalmic division of trigeminal, or BL
- optic neuritis
- Fhx of MS
- age onset <40y.o.
130
Acoustic neuromas = vestibular schwannomas
- what are the features?
- when may it be BL?
- what is the Ix of choice?
- 5% of intracranial tumours, 90% of cerebellopontine angle tumours
CN V: absent corneal reflex
CN VII: facial palsy
CN VIII: hearing loss, vertigo, tinnitus
131
What is multiple sclerosis?
| What are the genetics?
- chronic cell-mediated autoimmune disorder characterised by demyelination in the CNS
- 3x more common in women, most commonly Dx 20-40s, more common at higher latitudes
- monozygotic twin concordance 30%, dizygotic 2%
132
Commonest form of MS?
relapsing-remitting disease
| - acute attacks lasting 1-2months followed by periods of remission
133
Form of MS with the worst prognosis?
1ry progressive disease
- 10% of pts, progressive deterioration from onset
- more common in older people
134
What do 65% of pts with relapsing-remitting MS disease go on to develop within 15yrs of Dx?
2ry progressive disease
- when r-r pts deteriorate & have developed neuro signs & Sx between relapses
- gait & bladder disorders generally seen
135
MRI in MS?
- high signal T2 lesions
- periventricular plaques
- Dawson fingers: often seen on FLAIR images - hyper intense lesions penpendicular to the corpus callosum
136
CSF in MS?
- oligoclonal bands (and not in serum)
| - increased intrathecal synthesis of IgG
137
Visual evoked potentials in MS?
- delyaed, but well preserved waveform
138
Good prognostic features in MS?
- female, young 20s/30s
- relapsing-remitting
- sensory Sx only
- long interval between first 2 relapses
- complete recovery between relapses
139
MS Rs for an acute relapse?
- 5/7 high dose steroids to shorten length of relapse
| - but they don't alter the degree of recovery i.e. if they return to baseline
140
MS Rx for fatigue?
- trial AMANTADINE after excluding other problems e.g. anaemia, thyroid, depression
- CBT & mindfulness training
141
MS Rx for spasticity?
- Baclofen/Gabapentin 1st line
- Physio
- other options inc diazepam, dantrolene, tizanidine
142
MS Rx for oscillopsia: visual fields appear to oscillate?
Gabapentin
143
MS Rx for bladder dysfunction: urgency, incontinence, overflow etc?
- US/S to assess bladder emptying
- if significant residual volume -> intermittent catheterisation
- if no significant residual volume -> anticholinergics may improve urinary frequency
- anticholinergics may worsen Sx in some pts
144
DMARD that reduces relapse rate by 30% in MS?
Beta-interferon
| - reduces no of relapses & MRI changes but doesn't reduce overall disability
145
What are the criteria for using beta-interferon in MS?
- relapsing-remitting disease + 2 relapses in past 2 yrs + able to walk 100m unaided
- 2ry progressive disease + 2 relapses in past 2 yrs + able to walk 10m un/aided
146
Cause of upbeat nystagmus?
cerebellar vermis lesions
147
Cause of downbeat nystagmus?
foramen magnum lesions
| Arnold-Chiari malformation
148
Migraine Dx criteria?
A. 5+ attacks fulfilling criteria B-D
B. headache lasting 4-72h (untreated or successfully Rx)
C. headache with 2+ of the following:
- UL
- pulsating
- moderate/severe intensity
- aggravation by/causing advance of routine physical activity
D. at least 1 of the following during headache:
- nausea &/or vomiting
- photophobia, phonophobia
E. not attributed to another disorder
149
Typical aura in a migraine?
- progressive
- transient hemianopic disturbance or spreading scintillating scotoma (jagged crescent)
- sensory Sx
- develop over at least 5mins, last 5-60mins, fully reversible
150
Atypical Sx of aura in migraine prompting further Ix/referral?
- motor weakness
- double vision
- visual Sx affecting only 1 eye
- poor balance
- decreased GCS
151
1st line Rx for Parkinson's disease if:
- motor Sx affecting pt's QoL?
- motor Sx not affecting pt's QoL?
Motor Sx affecting QoL -> Levodopa
| Not affecting QoL -> non-ergot derived Dopamine agonist, levodopa or MAO-B inhibitor
152
Which drug in Parkinson's disease has more improvement in motor Sx & ADLs but also more complications?
but fewer specified adverse events (XS sleep, hallucinations & impulse control disorders)
Levodopa
153
Parkinson's D Rx if pt continues to have Sx despite optimal levodopa Rx or has developed dyskinesia?
- Add a dopamine agonist/MAO-B inhibitor or COMT inhibitor as an adjunct?
- dopamine agonists have a higher risk of hallucinations & intermediate risk of adverse event & more off-time reduction
154
Impulse control disorders can occur with any dopaminergic therapy but are more common when?
- with dopamine agonist therapy
- Hx of previous impulsive behaviours
- Hx of etoh consumption &/or smoking
155
If Sx after a medication review in pts with Parkinson's disease, what drug can be considered?
Midodrine - acts on peripheral alpha-adrenergic receptors to increase arterial resistance
156
What does levodopa need to be co-prescribed with?
| What are the side effects of it?
- a decarboxylase inhibitor e.g. carbidopa/benserazide to prevent peripheral metabolism of levodopa to dopamine
- reduced effectiveness with time usually by 2 yrs
- unwanted effects: dyskinesia, on-off effect, dry mouth, anorexia, palpitations, postural hypotension, psychosis, drowsiness, cardiac arrhythmias, nausea & vomiting, reddish discolouration of urine on standing
- NO USE in neuroleptic induced parkinsonism
157
Example of a MAO-B inhibitor? how does it word?
e. g. Selegiline
| - inhibits breakdown of dopamine secreted by the dopaminergic neurons
158
Examples of COMT inhibitors? how does it work?
e. g. Entacapone, tolcapone
| - COMT is an enzyme involved in the breakdown of dopamine so can be used as an adjunct to levodopa in established PD
159
Use of antimuscarinics in Parkinsons disease?
- blocks cholinergic receptors e.g. procyclidine, benzotropine, trihexyphenidyl/benzhexol
- helps tremor & rigidity
- now used more to treat drug-induced parkinsonism
160
Side-effects of Amantadine?
- probably increases dopamine release & inhibits its uptake at dopaminergic synapses
- ataxia, slurred speech, confusion, dizziness, lived reticular
161
What Ix should be done before starting ergot-derived dopamine receptor agonists & why?
- ESR, Cr, CXR, Echo
- e.g. bromocriptine, cabergoline
- pulmonary, retroperitoneal & cardiac fibrosis
162
Side effects of dopamine receptor agonists?
- impulse control disorders
- XS daytime somnolence
- more likely than levodopa to cause hallucinations in older pts
- nasal congestion & postural hypotension also seen
163
What is brachial neuritis?
- acute onset of unilateral severe pain, followed by shoulder & scapular weakness several days later
- sensory changes minimal
- there may be a subsequent rapid wasting of the arm muscles in accordance to which nerve is involved
- precipitating factors: recent trauma, infection, surgery or even vaccination; rarely hereditary
- prognosis usually good except when phrenic nerve involved - breathlessness
164
What is Parkinson's disease?
| What are the classic & other features?
Progressive neurodegenerative condition: degeneration of dopaminergic neurons in the substantial nigra
- 2x more common in men, men age Dx 65yrs, Sx classically asymmetrical
1. Bradykinesia
- poverty of movement also seen (hypokinesia)
- short, shuffling steps with reduced arm swing
- difficulty initiating movement
2. Tremor
- esp at rest 3-5Hz
- worse when stressed/tired
- typically pill-rolling
3. Rigidity
- lead pipe (not velocity-dependent)
- cogwheel: due to superimposed tremor
- mask-like facies, drooling of saliva
- flexed posture, fatigue
- micrographia
- impaired olfaction
- REM sleep disorder
- postural hypotension
- psychiatric features: depression commonest, dementia, psychosis, sleep disturbance
165
Slightly different features of drug-induced Parkinsonism?
- motor Sx generally rapid onset & bilateral
| - rigidity & rest tremor are Uncommon
166
What Ix can be considered if there is difficulty differentiating between essential tremor and Parkinson's disease ?
SPECT (123I-FP-CIT)
167
What is Guillain-Barre syndrome?
- immune-mediated demyelination o the peripheral nervous system, often triggered by an infection e.g. Campylobacter
168
What is the pathogenesis of Guillain-Barre syndrome?
| - correlation between which Ab & clinical features has been demonstrated?
- X-reaction of Abs with gangliosides in the peripheral nervous system
- anti-ganglioside Ab (anti-GM1)
- anti-GB1 Abs in 25%
- most common variant is an AIDP: acute inflammatory demyelinating polyneuropathy
169
What are the features of Guillain-Barre syndrome?
- progressive limb weakness, classically ascending (but tends to affect proximal muscles earlier than the distal), evolving over days/weeks
- areflexia
- mild sensory Sx
- back pain in initial stages
- areflexia
- cranial nerve involvement e.g. diplopia
- autonomic involvement e.g. urinary retention, postural hypotension
- less commonly papilloedema (2ry to reduced CSF absorption)
170
Rx of Guillain-Barre syndrome?
- plasma exchange
- IV immunoglobulins (es effective as plasma exchange. may be easier to administer, with fewer side-effects)
- ***FVC*** regularly to monitor respiratory function - can develop resp failure over a few hours
171
Poor prognostic features of Guillain-Barre?
- age > 40
- poor upper extremity muscle strength
- previous Hx of a diarrhoea illness specifically campylobacter jejune
- high anti-GM1 Ab titre
- need for ventilatory support
172
What is Miller-Fisher syndrome?
- what Ab are present in 90%?
- what are the associated features?
- variant of Guillain-Barre
- descending paralysis
- anti-GQ1b
- ophthalmoplegia, areflexia, ataxia - eye muscles usually affected first
173
TACI: total anterior circulation infarct features? 15%
- ACA & MCA involved
- UL hemiparesis &/orr hemisensory loss of face, arm, leg
- homonymous hemianopia
- higher cognitive dysfunction e.g. dysphasia
174
PACI: partial anterior circulation infarcts features? 25%
- smaller arteries of anterior circulation e.g. upper/lower division MCA
2 out of 3 of:
- UL hemiparesis &/orr hemisensory loss of face, arm, leg
- homonymous hemianopia
- higher cognitive dysfunction e.g. dysphasia
175
Lacunar infarcts (LACI) features? 25%
what arteries involved?
what is a strong association?
- perforating arteries around internal capsule, thalamus & basal ganglia involved
- strong ass with hypertension
1 of the following:
- UL weakness of UL +/- face +/- LL
- pure sensory stroke
- ataxic hemiparesis
176
POCI: posterior circulation infarct features?
```
- vertebrobasilar arteries
1 of the following:
- cerebellar/brainstem syndromes
- LOC
- isolated homonymous hemianopia
```
177
Features of lateral medullary/Wallenberg syndrome?
| what artery is involved?
- posterior inferior cerebellar artery
- IL cerebellar features: ataxia, nystagmus
- IL brainstem features: dysphagia, facial numbness, CN involvement e.g. Horner's
- CL limb sensory loss
178
Features of Weber's syndrome?
- IL IIIrd nerve palsy
| - CL weakness
179
Stroke: - 'locked-in' syndrome
| what artery is affected?
basilar artery
180
Stroke: amaurosis fugax
| what artery is affected?
retinal/ophthalmic artery
181
Stoke - what artery is affected if:
IL facial pain & temp loss
CL limb/torso pain & temp loss
ataxia, nystagmus?
posterior inferior cerebellar artery
(lateral medullary/ wallenberg syndrome)
= most commonly survived stroke that affects the brainstem
182
Stoke - what artery is affected if:
IL facial paralysis & deafness
CL limb/torso pain & temp loss
ataxia, nystagmus?
anterior inferior cerebellar artery (lateral pontine syndrome)
183
Stoke - what artery is affected if:
IL CN IIIrd nerve palsy
CL weakness of upper & lower extremity?
Weber's syndrome (branched of posterior cerebral artery that supply the midbrain)
184
Stoke - what artery is affected if:
CL homonymous hemianopia with macular sparing
visual agnosia?
posterior cerebral artery
185
Stoke - what artery is affected if:
CL hemiparesis & sensory loss, upper extremity > LL
CL homonymous hemianopia
aphasia?
middle cerebral artery
186
Stoke - what artery is affected if:
| CL hemiparesis & sensory loss, LL>UL?
anterior cerebral artery
187
When should BP be lowered in the acute phase of a stroke?
only if there are complications e.g. hypertensive encephalopathy
188
When to start anticoagulants for AF after a stroke?
Only when brain imaging has excluded haemorrhage, & usually not
189
Why delay starting a statin after a stroke?
risk of haemorrhagic transformation
190
When to give thrombolysis e.g. alteplase for a stroke?
- within 4.5h of onset of stroke Sx
| - if haemorrhage has been excluded on imaging
191
Absolute C/I & relative to thrombolysis?
Absolute:
- previous intracranial haemorrhage
- seizure at onset of stroke
- intracranial neoplasm
- suspected SAH
- stroke/traumatic brain injury preceding 3months
- LP in the past 7days
- GI beed in the past 3wks
- active bleeding
- pregnancy
- oesophageal varices
- uncontrolled hypertension >200/120mmHg
Relative:
- concurrent anticoag INR>1.7
- haemorrhagic diathesis
- active diabetic haemorrhagic retinopathy
- suspected intracardiac thrombus
- major surgery/trauma in past 2wks
192
Ischaemic stroke 2ry prevention?
```
Clopidogrel lifelong
(if C/I or not tolerated aspirin + dipyradimole, or then just dipyradimole)
```
193
When is carotid endarterectomy recommended with a stroke?
- if stroke/TIA in carotid territory & not severely disables
- only if stenosis >50-70%
194
Where is a SOL causing headache with palatal myoclonus - exam of UL, LL & CN normal?
Olivary nucleus
- hypertrophic olivary degeneration caused by a lesion in the triangle of Guillain & Mollaret (triangle linking inferior olivary nucleus, red nucleus & contralateral dentate nucleus)
- Ix of choice = MRI brain
195
Genetics of neurofibromatosis type 1?
autosomal dominant gene mutation on chromosome 17 which encodes neurofibromin, affecting 1/4000
aka von Recklinghausen's syndrome
196
Features of neurofibromatosis type 1?
- 6+ cafe au lait spots, 15mm diameter
- axillary/groin freckles
- peripheral neurofibromas
- iris harmatomas (Lisch nodules) in >90%
- scoliosis
- phaeochromocytomas
197
Genetics of neurofibromatosis type 2?
autosomal dominant gene mutation on chr 22, affecting 1/100,000
198
Features of neurofibromatosis type 2?
- bilateral acoustic neuromas
| - multiple intracranial schwannomas, meningiomas, ependymomas
199
What is CADASIL?
genetics?
how do pts present?
Cerebral Autosomal Dominant Arteriopathy with Subcortical Infarcts & Leukoencephalopathy
- rare cause of multi-infarct dementia
- commonest cause of hereditary cerebral small vessel disease & vascular cognitive impairment in young adults
- NOTCH3 mutation on chr 19
- pts often present with migraine +/- aura, FHx
- imaging: multiple subcortical white matter lesions, particularly in the anterior temporal lobes and basal ganglia, out of keeping with the pts age
200
MoA of procyclidine, used in parkinson's?
Antimuscarinic
201
MoA of Triptans?
Specific agonists 5-HT1 (b/d) receptors
202
What is it important to exclude with a headache & IIIrd nerve palsy?
Posterior communicating artry aneurysm
203
Features of a IIIrd nerve palsy?
- eye deviated down & out
- ptosis
- pupil may be dilated: mydriasis (surgical)
204
Causes of a IIIrd nerve palsy?
- diabetes
- vasculitis
- false localising sign due to uncal herniation through tentorium if raised ICP
- posterior communicating aneurysm (pupil dilated)
- caverous sinus thrombosis
- Weber's syndrome: IL IIIrd n palsy with CL hemiplegia caused by a midbrain stroke
- amyloid, MS
205
Migraine Rx during pregnancy
paracetamol 1st line
| aspirin 300 or ibuprofen 2nd line in 1st & 2nd trimester
206
Why is COCP absolutely C/I if pts have migraine with aura?
increased risk of ischaemic stroke
207
What is syringobulbia & syringomyelia?
```
Syringomyelia = syrinx = collection of CSF within spinal cord
syringobulbia = similar with fluid-filled cavity within medulla of brainstem (often extension of syrinx)
```
208
Causes of syringomyelia?
| Rx?
- Chiari malformation (strong ass)
- trauma
- tumours
- idiopathic
Rx the cause - shunt cane placed if persistent
209
What is the classical presentation of syringomyelia & why?
- cape-like (neck & arms) loss of sensation to pain/temp with preservation of light touch, proprioception & vibration
- due to crossing of spinothalamic tracts in the anterior white commissure of the spinal cord being the first tracts to be affected
- also spastic weakness (UL), paaresthesia, neuropathic pain, upgoing planters & bowel/bladder dysfunction
- scoliosis over years if syrinx not treated
- may cause Horner's due to compression of sympathetic chain but rare
210
What is motor neurone disease?
unknown cause of a neurological condition with UMN & LMN signs
- rarely presents before 40
- various patterns of disease inc ALS, progressive muscular atrophy & bulbar palsy
211
Features of MND?
- fasciculation
- absence of sensory signs/Sx
- mixture of LMN & UMN signs
- wasting of small hand muscles/tibialis anterior is common
- doesn't affect external ocular muscles
- no cerebellar signs
- abdominal reflexes usually preserved & sphincter dysfunction if present is a late feature
212
How is MND Dx?
what is shown on nerve conduction studies?
EMG?
MRI?
- clinical Dx
- motor nerve conduction normal to help exclude a neuropathy
- EMG reduced number of action potentials with increased amplitude
- MRI to exclude DDx of cervical cord compression & myelopathy
213
Neuro exam of a pt with DCM: degenerative cervical myelopathy?
- LMN signs at the level of the lesion
| - UMN signs below the level
214
MoA of Valproate?
| Adverse effects?
- increases GABA activity
```
Vomiting, nausea
Alopecia
Liver toxicity
Pancreatitis, pancytopenia
Retain weight
Oedema
Ataxia, Appetite increase
Thrombocytopenia, Teratogenic, Tremor
Electrolyte (hyponatraemia), Enzyme INHIBITOR
```
215
What is Charcot-Marie-Tooth disease?
= commonest hereditary peripheral neuropathy (/umbrella term for hereditary polyneuropathies)
- predominately motor loss
- physical & occupation therapy (no cure)
216
Features of Charcot-Marie-Tooth disease?
- frequently sprained ankles
- distal muscle weakness
- distal muscle atrophy
- hyporeflexia
- stork leg deformity
- pes cavus
- hammer toes
217
Genetics of Charcot-Marie-Tooth disease?
Commonest is autosomal dominant CMT type 1
| usually caused by duplication of PMP-22 gene on chromosome 17
218
What is Wernicke's encephalopathy?
| What is seen pathologically?
- Neuropsych disorder caused by thiamine deficiency most commonly seen in alcoholics
- rarer causes: persistent stomach cancer, dietary deficiency
- petechial haemorrhages in a variety of structures inc maxillary bodies & ventricle walls
219
Features of Wernicke's encephalopathy?
1. ophthalmoplegia
2. nystagmus (commonest ocular sign)
3. confusion/altered GCS
Also:
- ataxia
- peripheral sensory neuropathy
```
Confusion
Ataxia
Nystagmus
Ophthalmoplegia
PEripheral
Neuropathy
```
220
Ix in Wernicke's encephalopathy
- imaging?
- serum?
Rx?
MRI
decreased red cell transketolase
Rx urgent thiamine replacement
221
What is Korsakoff syndrome?
Wernicke's + antero & retrograde amnesia & confabulation
222
What is restless legs syndrome?
What are the clinical features?
Causes & associations?
- spontaneous continuous LL movements that may be ass with paraesthesia, v common
- akathisia (initially at night, worse at rest, but progress to during the day)
- paraesthesias e.g. crawling, throbbing
- movemets during sleep may be noted by partner - PLMS: periodic limb movements of sleeps
- LOW ferritin
- FHx 50%, IDA, uraemia, DM, pregnancy
223
Rx of restless legs syndrome?
- walk, stretch, massage
- Rx any iron deficiency
- 1st line = dopamine agonists: Pramipexole, Ropinirole
- benzodiazepines, gabapentin
224
What is cataplexy?
sudden & transient loss of muscular tone caused by emotion
- 2/3 of narcolepsy pts have cataplexy
225
paraneoplastic syndrome that can occur independently as an autoimmune disorder or ass with SCLC/breast/ovarian ca
- Ab directed against pre-synaptic voltage gated calcium channel in the peripheral nervous system
Lambert-Eaton myasthenic syndrome
| - anti-VGCC
226
Paraneoplastic syndrome ass with SCLC & neuroblastoma, with features of:
- sensory neuropathy (may be painful)
- cerebellar syndrome
- encephalomyelitis
Anti-Hu
227
Paraneoplastic syndrome ass with ovarian & breast cancer with features of a cerebellar syndrome?
Anti-Yo
228
Ab of a paraneoplastic syndrome ass with breast, colorectal & SCLC with features of a stiff persons syndrome or diffuse hypertonia?
Anti-GAD Ab
229
Ab of a paraneoplastic syndrome ass with peripheral neuropathy in breast cancer?
Purkinje cell Ab
230
Ab of a paraneoplastic syndrome ass with breast & SCLC ass with ocular opsoclonus myoclonus?
Anti-Ri
231
What is NMO: neuromyelitis optica?
| Common presenting complaint?
= Monophasic or relapsing-remitting demyelinating disorder (immune cells & Abs attack optic nerves * spinal cord)
- Asians
- typically involved optic nerves & C spine
- brain imaging often normal
- Vomiting
232
How to diagnose NMO: neuromyelitis optica?
BL optic neuritis + myelitis
+ 2/3 of:
- spinal cord MRI T2 lesion involving 3+ spinal levels
- initially normal MRI brain (i.e. not meeting MS criteria at disease onset)
- NMO-IgG seropositive status: Ab against aquaporin 4 Ag
233
What are the 3 features of classifying epilepsy?
1. where the seizures begin in the brain
2. level of awareness during a seizure
3. other features of seizure
234
Type of seizure that starts on 1 side of the brain in a specific area before spreading to both lobes? (prev termed 2ry generalised)
Focal to bilateral seizure
235
Type of seizure that starts in a specific area on 1 side of the brain where the level of awareness can vary (pre termed partial seizure) ?
How to further describe?
Focal seizures
focal aware = simple partial
focal impaired awareness = complex partial
focal awareness unknown
focal motor (e.g. Jacksonian march)
focal non-motor e.g. deja vu, jamais vu
etc
236
Type of seizures that engage/involve networks on both sides of the brain at the onset with immediate LOC?
How to further describe?
Generalised seizures
motor e.g. tonic-clonic (grand mal), tonic, clonic
non-motor e.g. typical absence (petit mal)
myoclonic: brief, rapid muscle jerks
atonic
237
Drug causes of peripheral neuropathy?
```
amiodarone
isoniazid
vincristine
nitrofurantoin
metronidazole
```
238
MoA of phenytoin?
binds to sodium channels increasing their refractory period
239
Acute adverse effects of phenytoin?
initially dizziness, diplopia, nystagmus, slurred speech, ataxia
later confusion, seziures
240
Chronic adverse effects of phenytoin?
| Teratogenic?
common: gingival hyperplasia (2ry to increased expression of PDGF), hirsutism, coarsening facial features, drowsiness
- megaloblastic anaemia (altered folate metabolism)
- peripheral neuropathy
- enhanced vitamin D metabolism causing osteomalacia
- lymphadenopathy
- dyskinesia
- ass with cleft palate & congenital heart disease
241
Idiosyncratic adverse effects of phenytoin?
```
fever, rashes inc TEN
hepatitis
Dupuytren's contracture
aplastic anaemia
drug-induced lupus
```
242
When to monitor phenytoin?
| how?
If there is:
- adjustment of phenytoin dose
- suspected toxicity
- detection of non-adherence to prescribed meds
Trough level, immediately pre-dose
243
Features of subacute combined degeneration of spinal cord?
- vitamin b12 deficiency
- dorsal & lateral columns affected
- joint position & vibration lost first then distal paraesthesia (dorsal column)
- (lateral column) UMN signs typically develop in legs: extensor planters, brisk knee reflexes, absent ankle jerks
- damage to peripheral nerves (LMN signs of absent reflexes)
- if untreated, stiffness & weakness persist
244
What is tuberous sclerosis?
autosomal dominant neurocutaneous condition
245
What are the features of tuberous sclerosis?
neuro
cutaneous
other
Neuro:
- developmental delay
- epilepsy (infantile spasms/partial)
- intellectual impairment
Cutaneous:
- depigmented 'ash-leaf' spots which fluoresce under UV light
- Shagreen patches: roughened patches of skin over lumbar spine
- adenoma sebaceum angiofibromas: butterfly distribution over nose
- subungual fibromata
- cafe-au-lait spots may be seen
Other:
- retinal hamartomas: dense white areas phakomata
- rhabdomyomas of heart
- gliomatous changes can occur in brain lesions
- polycystic kidneys, renal agiolipomata
- lymphangioleiomyomatosis: multiple lung cysts
246
What side is the lesion with UL cerebellar signs?
ipsilateral - same side
247
Sx/signs of cerebellar disease?
```
Dysdiadochokinesis, Dysmetria
Ataxia
Nystagmus (horizontal = IL hemisphere)
Intention treor
Slurred staccato speech, Scanning dysarthria
Hypotonia
```
248
Causes of a cerebellar syndrome?
Paraneoplastic eg lung ca, Neoplastic eg cerebellar haemangioma
Abscess/atrophy
Stroke, multiple sclerosis
Trauma
Raised ICP
Infection
ETOH & drugs: phenytoin, lead poisoning
Spinocerebellar ataxia, Friedrichs ataxia, ataxic telangiectasia
249
What is multiple system atrophy?
| What are the 2 predominant types?
Parkinson-plus syndrome of:
1. Parkinsonism
2. Autonomic disturbance (atonic bladder, postural hypotension, erectile dysfunction)
3. cerebellar signs
```
MSA-P = predominant parkinsonian features
MSA-C = predominant cerebellar features
```
250
In multiple system atrophy, What are the clues to this Dx?
| What is the pathological hallmark?
- early falls
- postural instability
- lack of response to levodopa
- alpha-synuclein containing glial cytoplasmic inclusions in the basal ganglia, cerebellum & motor cortex
251
What is corticobasal syndrome?
= movement disorder, with a unilateral absence of movements and muscle rigidity with a tremor
- progressive neurological disorder that can also affect cognition
- tauopathy
252
What is progressive supra nuclear palsy?
Rx?
pathological feature?
= parkinson plus syndrome with features of:
- impairment of vertical gaze (down worse than up, difficulty reading or going downstairs)
- symmetrical parkinsonism
- cognitive impairment
- early falls
- bulbar Sx e.g. slurring speech
Rx - poor response to L-dopa
- abnormal accumulation of tau (τ) proteins and degeneration of the substantia nigra, subthalamic
nucleus & mid-brain
- therefore a tauopathy
rather than synucleinopathy
253
65M is referred to the neuro OP clinic due to a UL resting tremor. A Dx of Parkinson's disease is made. He is otherwise well and is not currently disabled by his Sx. What is the most appropriate treatment?
New generation dopamine receptor agonist e.g. ropinirole
254
Causes of a brain abscess?
| Features?
- extension of sepsis from middle ear or sinuses, trauma/surgery to scalp, penetrating head injuries & embolic event from endocarditis
- mass effect, raised ICP
- headache, fever
- focal neurology
255
Features of Lambert-Eaton syndrome?
- repeated muscle contractions lead to increased muscle strength
- limb girdle weakness, affecting LL first
- hyporeflexia
- autonomic Sx: dry mouth, impotence, difficulty micturating
256
What is shown on EMG in Lambert-Eaton syndrome?
incremental response to repetitive electrical stimulation
257
Rx of Lambert-Eaton syndrome?
Rx underlying cancer
immunosuppression e.g. prednisolone/azathioprine
IV Ig/plasma exchange may help
258
Features of idiopathic intracranial hypertension?
RFs?
Rx?
headache, blurred vision, papilloedema, enlarged blind spot, VIth nerve palsy
- obesity, female, pregnancy
- OCP, steroids, tetracycline, vitamin A, lithium
- weight loss
- diuretics e.g. acetazolamide
- topiramate
- repeated LP
- surgery:optic nerve sheath decompression & fenestration may be needed to prevent damage to optic nerve; a shunt can also help reduce ICP
259
Trinucleotide repeat in Friedreich's ataxia?
GAA
260
Trinucelotide repeat in myotonic dystrophy?
CTG
261
Trinucleotide repeat in fragile X syndrome?
CGG
262
Trinucleotide repeat in Huntington's disease?
CAG
263
What are the genetics of Huntington's disease?
| What are the features?
- autosomal dominant
- trinucleotide repeat disorder: repeat expansion of CAG
- defect in huntingtin gene on chr 4
- results in degeneration of cholinergic & GABAergic neutrons in the striatum of the basal ganglia
- chorea
- personality changes & intellectual impairment
- dystonia
- saccadic eye movements
264
Causes of macroglossia?
```
hypothyroid
acromegaly
amyloid
Duchenne muscular dystrophy
mucopolysaccaridosis
(Down's - apparent macroglossia)
```
265
Typical features of a post-LP headache?
Factors which may contribute to headache?
Rx?
- usually within 24-48h, may last several days
- worsens with upright, improves with recumbent position
- increased needle size
- direction of bevel
- inc no of LP attempts
Rx supportively, but indicated if persists >72h to prevent subdural haematoma
- blood patch, epidural saline, IV caffeine
266
Neurological sequalae of meningitis?
- sensorineural deafness most common
- epilepsy, paralysis
- sepsis, intracranial abscess
- brain herniation, hydrocephalus
267
What is a spastic paraparesis?
| What are the causes?
- UMN pattern of weakness in the LL
- demyelination e.g. MS
- cord compression: trauma, tumour
- parasagittal meningioma
- tropical spastic paraparesis
- transverse myelitis e.g. HIV
- syringomyelia
- hereditary spastic paraplegia
- OA of the cervical spine
268
What are the RFs for cluster headaches?
| What are the features?
- more common in men 3:1 & smokers
- ETOH can trigger an attack, may be a relation to nocturnal sleep
- pain OD/BD, each ep 15mins-2h
- clusters typically last 4-12wks
- intense sharp, stabbing pain around 1 eye (recurrent attacks always affect same side)
- pestless & agitated during attack
- redness, lacrimation, lid swelling
- nasal stuffiness
- miosis & ptosis in minority
269
acute Rx of cluster headaches?
| prophylaxis?
- 100% O2 (80% response rate within 15mins)
- SC triptan (75%)
- Verapamil prophylaxis
- tapering dose prednisolone may help
- neuro referral (to consider neuroimaging)
270
Commonest MND type
- typically LMN signs in UL
- UMN signs in LL ?
in familial cases what is the genetics?
ALS 50%: amyotrophic lateral sclerosis
| - gene codes for superoxide dismutase on chr 21
271
MND type with UMN signs only?
1ry lateral sclerosis
272
MND with best prognosis, that consists of LMN signs only and affects distal muscles before proximal?
Progressive muscular atrophy
273
MND with worst prognosis?
Progressive bulbar palsy
| - palsy of tongues, muscles of chewing/swallow & facial muscles, due to loss of function of brainstem motor nuclei
274
HSV encephalitis:
what lobe does it characteristically affect?
what are the features?
pathophysiology?
- Temporal (can have focal features eg aphasia) & inferior frontal lobes
- fever, headache, psychiatric Sx, seizures, vomiting
- HSV-1 in 95%
275
```
HSV encephalitis, what is shown on:
CSF?
how to Dx HSV?
CT?
what scan is better?
EEG?
```
How to Rx?
CSF: lymphocytosis, elevated protein
HSV Dx by PCR
CT: medial temporal & inferior frontal lobe changes e/g/ petechial haemorrhages - but normal in 1/3
MRI is better
EEG: lateralised periodic discharges at 2Hz
Rx with IV aciclovir - early Rx determines prognosis
276
What is CJD?
= rapidly progressive neuro condition caused by prion proteins, which induce formation of amyloid folds -> tightly packed beta-pleated sheets resistant to proteases
- 85% sporadic mean age onset 65yrs
277
CJD: What are the features?
| What is shown on CSF, EEG & MRI?
- rapid-onset dementia
- myoclonus
CSF normal
EEG: biphasic, high amplitude sharp waves in sporadic CJD
MRI: hyperintense signals in the basal ganglia & thalamus
278
What is new variant CJD?
- younger pts with average onset 25yrs
- psych Sx eg anxiety, withdrawal, dysphonia
- 'prion protein' on chr 20
- methionine homozygosity at codon 129 of prion protein is a RF for developing it - pts who have died have had this
- medial survival 13months
279
Immediate antithrombotic therapy in suspected TIA?
Aspirin 300mg immediately unless:
- bleeding disorder/on anticoagulant (immediate admission for excluding haemorrhage)
- pt already taking low-dose aspirin regularly (cont until specialist review)
- aspirin C/I (discuss urgently with specialist)
280
Referral it pt has had a suspected TIA in last 7days?
Urgent specialist stroke assessment within 24h
281
Referral if pt has had a suspected TIA >1wk ago?
Specialist stroke assessment referral asap within 7days
282
Referral if pt has had crescendo TIA or suspected cardioembolic source or severe carotid stenosis?
discuss need for admission urgently withs stroke specialist
283
What is & what are the features of Brown-Sequard syndrome?
Hemisection of spinal cord
- IL weakness below lesion
- IL loss of proprioception & vibration sensation
- CL loss of pain & temperature sensation
284
What are the features of central cord syndrome?
BL motor weakness
285
Causes of raised lymphocytes in CSF?
- viral meningitis/encephalitis
- TB meningitis
- partially Rx bacterial meningitis
- Lyme disease
- Behcet's, SLE
- lymphoma, leukaemia
286
Features & Rx of medication overuse headache?
- present for 15+days/month
- developed/worsened whilst taking regular Sx medication
- using opioids & triptans most at risk
- may be psychiatric co-morbidity
Rx:
- withdraw simple analgesics & triptans abruptly
- gradually withdraw opioids
- withdrawal Sx can occur when medication stopped e.g. vomiting, hypotension, tachycardia, restlessness, sleep disturbances, anxiety
287
Infantile spasms/West's syndrome:
- what are the features
- what is on eeg?
- brief spasms starting in first few weeks of life
- flexion of head, trunk, limbs -> arm extension (salaam attack), last 1-2sec, repeat unto 50x
- progressive mental handicap
- usually 2ry to serious neuro abnormality/cryptogenic e.g. TS, encephalitis, birth asphyxia
- poor prognosis
- EEG: hypsarrhythmia
- Rx: vigabatrin/steroids
288
Features of typical (petit mal) absence seizures in children?
what is on eeg?
- onset 4-8yrs
- duration few-30secs, no warning, quick recovery, often many/day
- EEG: 3Hz generalised, symmetrical
- Rx: sodium valproate, ethosuximide
- good prognosis: 90-95% become seizure-free in adolescence
289
What is Lennox-Gastaut syndrome in children?
| what is on eeg?
- may be extension of infantile spasms, onset 1-5yrs
- atypical absences, falls, jerks
- 90% moderate-severe mental handicap
- EEG: slow spike
- ketogenic diet may help
290
What is characteristic of benign rolandic epilepsy?
- most common in childhood, M>F
| - paraesthesia e.g. UL face, usually on waking up
291
What are the features of juvenile myoclonic epilepsy (Janz syndrome)?
Rx?
- onset teens, F:M 2:1
- infrequent generalised seizures, often in morning
- daytime absences
- sudden shock-like myoclonic seizure
Rx: usually good response to sodium valproate
292
Causes of seizures/epilepsy in neonatal period?
- 2ry: hypoglycaemia, meningitis, head trauma
- pyridoxine dependency (recessive, give IV B6)
- benign familial neonatal seizures (dominant)
- benign neonatal convulsions (day 5)
293
Causes of otitis externa?
- bacterial staph aureus, pseudomonas aeruginosa
- fungal
- seb derm
- contact dermatitis
294
Initial Rx of otitis extern?
| 2nd line?
- topical Abx +/- steroid combined
- if tympanic membrane perforated, aminoglycosided traditionally not used
- consider removal if canal debris
- ear wick can be inserted if canal is extensively swollen
2nd line:
- consider contact dermatitis 2ry to neomycin
- oral Abx (flucloxacillin) if infection spreading
- swab canal
- empirical use of anti fungal agent
295
What is malignant otitis externa?
| who is it commoner in?
- extension of infection into bony ear canal & deeper soft tissues
- elderly diabetics
- IV Abx
296
What intervention has the most benefit to survival in MND?
NIV (usually BiPAP) at night
| - survival benefit 7months
297
How does Riluzole work in MND?
- prevents stimulation of glutamate receptors
| - used mainly in ALS
298
What is Arnold-Chiara malformation?
| what are the features?
- downward displacement/herniation of the cerebellar tonsils through the foramen magnum
- congenital/2ry to trauma
- non-communicating hydrocephalus may develop as a result of obstruction of CSF flow
- headache
- syringomyelia
299
Features of autonomic neuropathy?
- impotence, inability to sweat, postural hypotension
- loss of decrease in HR after deep breathing
- pupils: dilate after adrenaline instillation
300
Causes of autonomic neuropathy?
- diabetes
- HIV, Chagas', neurosyphilis
- antihypertensives, tricyclics
- Parkinson's
- multisystem atrophy
- guillain-barre
- craniopharyngioma
301
Causes of peripheral neuropathy - predominately motor loss?
- guillain-barre syndrome
- porphyria
- lead poisoning
- HSMN - Charcot-Marie tooth
- CIDP: chronic inflammatory demyelinating polyneuropathy
- diphtheria
302
Causes of peripheral neuropathy - predominately sensory loss?
- diabetes
- uraemia
- leprosy
- alcoholism (2ry to direct toxic effects & reduced absorption of B vitamins) - sensory Sx present before motor Sx
- vitamin b12 deficiency - SCDSC, dorsal column affected first before distal paraesthesia
- amyloid
303
What is chorea?
- involuntary, rapid, jery movements which often move from 1 part of the body to another
- caused by damage to the basal ganglia, esp the caudate nucleus
- athetosis = slower, sinuous movements of the limbs
304
Causes of chorea?
- Huntington's, Wilson's, ataxic telangiectasia
- SLE, anti-phospholipid syndrome
- rheumatic fever: Sydenham's chorea
- OCP, L-dopa, antipsychotics
- neuroacanthocytosis
- pregnancy: chorea gravidarum
- thyrotoxicosis
- polycythaemia rubra vera
- carbon monoxide poisoning
- cerebrovascular disease
305
What is an acute subdural haematoma?
- fresh blood collected within subdural space
- e.g. high-impact trauma
- spectrum of severity of Sx & presentation depending on size
- CT: crescent not limited by suture lines, hyper dense
- if large, may show mass effect with midline shift
- surgical options inc monitoring intracranial pressure & decompressive craniectomy
306
What is a chronic subdural haematoma?
- collection of blood within subdural space that has been present for weeks-months
- rupture of the small bridging veins within the subdural space rupture & cause slow bleeding
- elderly & etoh at risk as they have brain atrophy therefore fragile bridging veins
- weeks-month progressive Hx of confusion, reduced GCS or neuro deficit
- infants also have fragile bridging veins and can rupture in shaken baby syndrome
- CT: crescent, not restricted by suture lines, with mass effect
- HYPOdense (vs acute subdural)
- if surgical option indicated then e.g. surgical decompression with burr holes
307
Which conditions are associated with raised CSF protein?
- TB, fungal & bacterial meningitis
- viral encephalitis
- Guillain-Barre syndrome
- Froin's syndrome: increase in CSF protein below a spinal canal blockage
308
What is von Hippel-Linday syndrome?
| Features?
- autosomal dominant condition predisposing to neoplasia - due to abnormality in VHL gene on short arm of chr 3
- cerebellar haemangiomas
- retinal haemangiomas: vitreous haemorrhage (BL in 25%)
- renal cysts (premalignant)
- phaeochromocytoma
- extra-renal cysts: epididymal, pancreatic, hepatic
- endolymphatic sac tumours
- clear cell renal cell carcinoma
309
What is the MoA of selegiline?
MAO-B inhibitor - inhibitor breakdown of dopamine secreted by the dopaminergic neurons
310
Most of entacapone & tolcapone?
COMT inhibitor
| - enzyme involved in dopamine breakdown
311
Features of vestibular neuronitis?
- recurrent vertigo attacks after a viral infection
- nausea+/- vomiting
- horizontal nystagmus
- NO hearing loss/tinnitus
312
Rx of vestibular neuronitis?
- vestibular rehab preferred if chronic Sx
| - Betahistine
313
What are features of an intracranial venous thrombosis?
- headache, may be sudden onset
- nausea & vomiting
- 50% have isolated sagittal sinus thromboses - the rest have coexistent lateral sinus thromboses & cavernous sinus thromboses
- can cause cerebral infarction, much less common than arterial causes
314
Features of a sagittal sinus thrombosis?
- may present with seizures & hemiplegia
- parasagittal biparietal/bifrontal haemorrhagic infarctions sometimes seen
- empty delta sign on contrast CT
315
Features of a cavernous sinus thrombosis?
- (local infection, neoplasia & trauma can also cause a cavernous sinus syndrome)
- periorbital oedema
- ophthalmoplegia: 6th nerve damage typically occurs before 3rd & 4th
- trigeminal nerve involvement may lead to hyperaesthesia of upper face & eye pain
- central retinal vein thrombosis
316
Features of a lateral sinus thrombosis?
- 6th & 7th cranial nerve palsies
317
What is narcolepsy associated with?
What are the features?
Ix?
- HLA-DR2
- low levels of orexin (hypocretin) = protein responsible for controlling appetite & sleep patterns
- early onset of REM sleep
- typical onset in teenage years
- hypersomnolence
- cataplexy
- sleep paralysis
- vivid hallucinations on going to sleep/waking up
Ix = multiple sleep latency EEG
318
A 34y.o. man with a Hx of migraine finds that paracetamol taken at the recommend dose often fails to relieve his acute attacks. He drinks 12 units of etch/week & smokes 15 cigarettes/day
What factor is likely to contribute to this problem?
Delayed gastric emptying - often occurs during attacks therefore analgesics often combined with pro kinetics e.g. metoclopramide
319
What is a subarachnoid haemorrhage? How can it be classified?
- intracranial haemorrhage: blood within the subarachnoid space (deep to the subarachnoid layer of the meninges)
- traumatic SAH
- spontaneous SAH
320
Causes of a spontaneous SAH?
- intracranial saccular berry aneurysm 85% (APKD, Ehlers-Danlos & coarctation of the part are associations)
- AV malformation
- pituitary apoplexy
- arterial dissection
- mycotic aneurysm
- perimesencephaic (idiopathic venous bleed)
321
Classical presenting features of a SAH?
- sudden-onset thunderclap occipital headache
- nausea & vomiting
- meningism
- seizures, coma, sudden death
322
How to confirm SAH?
| What to do next?
- CT head (negative in 7%)
- LP if CT head negative: at least 12h post Sx onset to allow xanthochromia to develop = result of red cell breakdown
- > immediate neurosurgery referral
323
After confirming spontaneous SAH, how to Ix underlying cause that may require urgent Rx?
- CT intracranial angiogram (to identify vascular lesion e.g. aneurysm or AV malformation)
- +/- digital subtraction angiogram (catheter angiogram)
324
What are important predictive factors in SAH?
- conscious level on admission
- age
- amount of blood visible on CT head
325
Complications of aneurysmal SAH?
- re-bleed 30%
- vasospasm (=delayed cerebral ischaemia), typical 7-14days after onset
- hyponatraemia (siadh)
- seizures
- hydrocephalus
- death
326
Rx of SAH?
- depends on underlying cause
- most intracranial aneurysms: coil by interventional neuroradiologists
- minority require craniotomy & clipping by neurosurgery
- to help prevent rebelled: bed rest, well controlled BP, avoid straining, esp until aneurysm is treated
- to help prevent vasospasm: 21 days nimodipine, hypervolaemia, induced-hypertension & haemodilution
- Rx hydrocephalus: temporarily with an external ventricular drain (CSF diverted into a bag at bedside), or, if required, a long-term VP shunt
327
What is BPPV?
What are the features?
Sx relief?
- sudden onset dizziness & vertigo triggered by changes in head position, average age onset 55yrs
- +/- nausea
- episode lasts 10-20seconds
- positive Dix-Hallpike manoeuvre
- good prognosis, usually resolves spontaneously after a few weeks-months
Sx relief
- Epley manoeuvre 80% success
- home exercises (vestibular rehab)
- meds eg betahistine but limited value
328
Alexia without agraphia i.e. can write but not read - where is the lesion?
Corpus callosum
- due to infarction of the left posterior cerebral artery which perfuses the splenium of the corpus callosum
- therefore only the right visual cortex can process info, but it can't be transmitted to the left hemisphere, therefore can't reach the Wernicke/Broca's area in the left (dominant) hemisphere
329
What is ADEM: acute disseminated encephalomyelitis?
- autoimmune demyelinating disease of the CNS = post-infectious encephalomyelitis e.g. MMR, varicella, smallpox
- acute onset of multifocal neuro Sx with rapid deterioration a few days-2months after
- non-specific signs e.g. headache, fever, nausea, vomiting
- motor/sensory deficits
- brainstem involvement
- MRI can show supra & infra-tensorial demyelination
- IV steroids & consider IV Ig
330
Drugs used in MS (except for baclofen):
1. immunomodulating drug that acts as an 'immune decoy'?
2. a recombinant mAb that antagonists alpha-4 beta 1-integrin found on surface of leukocytes, thus inhibiting migration of leucocytes across the endothelium across the blood-brain barrier
3. sphingosine 1-phosphate receptor modulator, prevents lymphocytes from leaving lymph nodes
1. glatiramer acetate
2. natalizumab
3. fingolimod
331
Causes of foot drop?
- common perennial nerve lesion (commonest)commonest
- L5 radiculopathy
- sciatic nerve lesion
- superficial/deep perennial nerve lesion
- central nerve lesion e.g. stroke
332
On/exam of a foot drop?
| when would you consider a specialist referral?
- weakness of foot dorsiflexion & eversion + normal reflexes if isolated perennial neuropathy
- weakness of hip abduction suggests an L5 radiculopathy
Refer if:
- BL Sx
- fasciculations
- or other abnormal neuro findings
333
MoA of apomorphine?
dopamine receptor agonist
334
What is Foster-Kennedy syndrome? (brain lesion)
- frontal lobe tumour
- IL optic atrophy (direct damage from SOL)
- CL papilloedema
335
Causes of mitosis: small pupil?
- Horner's syndrome
- Argyll-Robertson pupil
- senile miosis
- pontine haemorrhage
- congenital
- opiates, pilocarpine, organophosphate toxicity
336
```
DVLA restriction if:
simple faint?
explained & Rx syncope?
unexplained syncope?
2+ episodes syncope?
stroke/tia?
multiple TIAs?
craniotomy?
pituitary tumour?
narcolepsy/cataplexy?
chronic neuro disorder?
single seizure?
established epilepsy or multiple unprovoked seizures?
withdrawal of anti-epileptics?
```
simple faint - no restriction
explained & Rx syncope - 4 weeks off
unexplained syncope - 6 months off
2+ episodes syncope - 12months off
stroke/tia - 1month off, may not need to inform delve if no residual deficit
multiple TIAs - 3months off & inform dvla
craniotomy - 1yr off
pituitary tumour - 6months off if craniotomy
narcolepsy/cataplexy - stop driving when Dx, can restart when satisfactory control of Sx
chronic neuro disorder - inform dvla, complete PF1 form
single seizure - 6months off. 12 if structural abnormalities or epileptiform activity on eeg
established epilepsy or multiple unprovoked seizures - license if seizure-free for 12months, license till 70 if seizure free 5 yrs
withdrawal of anti-epileptics - cannot drive during this period & 6months after last dose
337
MoA of ondansetron?
5-HT3 antagonist
338
Causes of gingival hyperplasia?
- phenytoin
- ciclosporin
- calcium channel blockers esp nifedipine
- AML
339
What is the use of nerve conduction studies?
Help determine between axonal & demyelinating pathology
340
Axonal pathology on nerve conduction studies?
- reduced amplitude
| - normal conduction velocity
341
Demyelinating pathology on nerve conduction studies?
- reduced conduction velocity
| - normal amplitude
342
Biopsy of lymphadenopathy showing regressed germinal centres surrounded by prominent mantle zones - what is the Dx?
Unicentric Castleman's disease = lymphoproliferative disorder associated in a subset of cases with HIV & HHV-8
- pts tend to be aSx & lymphadenopathy is constrained to one LN group
- chest 24%, neck 20%, abdo 18%, retroperitoneum, 14%
343
Convex hyPOdense colection on head CT - Dx?
chronic subdural haematoma
344
Anti-epileptic drug Vigabatrin - what is a common adverse effect that must be monitored?
Visual field defects in 40% - may be irreversible
| - check visual fields every 6months
345
Localising features of focal seizures: auditory/gustatory/olfactory hallucinations, epigastric rising/emtional, automatisms e.g. lip smacking, deja vu/dysphasia post-ictal
- what lobe is likely affected?
Temporal lobe
| HEAD
346
Localising features of focal seizures: head/leg movements, posturing, post-ictal weakness
- what lobe is likely affected?
Frontal lobe (motor)
347
Localising features of focal seizures: paraesthesia
| - what lobe is likely affected?
Parietal lobe (sensory)
348
Localising features of focal seizures: floaters/flashes
| - what lobe is likely affected?
Occipital lobe (visual)
349
What are the main subtypes of vascular dementia?
stroke-related VD - multi-infarct or single-infarct dementia
subcortical VD - small vessel disease
mixed - VD + Alzheimer's
350
RFs for vascular dementia?
How do pts usually present?
Sx?
- Hx of stroke/TIA, AF, HTN, DM, hyperlipidaemia, smoking, obesity, IHD, FHx stroke/IHD
- several months/years of a Hx of a sudden/stepwise deterioration of cognitive function
- focal neuro abn, difficulty with attention & concentration, seizures, memory, gait, speech, emotion disturbance
351
Rare inherited cause of vascular dementia?
CADASIL: cerebral autosomal dominant arteriopathywith subcortical infarcts & leukoencephalopathy
352
How to Dx vascular dementia?
Cognitive decline that interferes with ADLs, not due to 2ry effects of the cerebrovascular event
- establish using clinical exam & neuropsych testing
Cerebrovascular disease
- neuro signs &/or brain imaging
A relationship between the above 2disorders inferred by:
- dementia onset within 3months post-stroke
- abrupt deterioration in cognitive functions
- fluctuating, stepwise progression of cognitive deficits
353
Management of vascular dementia?
- support, Sx & address CV RFs
- cognitive stimulation programmes, multisensory stimulation, music & art therapy, animal-assisted therapy etc
- manage challenging behaviours e.g. address pain, communication
- only consider acetylcholineesterase inhibitors/memantine if suspected comorbid AD, PD dementia or DLB
- no evidence for aspirin
- tstains not evaluated
354
What is a CN IVth nerve palsy?
- supplies superior oblique (depresses eye, moves inward)
| - therefore vertical diplopia in a palsy, classically noticed when reading book/going down stairs
355
What anti epileptic drug may exacerbate absence seizures and is therefore contra-indicated?
carbamazepine
356
What is transient global amnesia?
- transient loss of memory function <24h: anterograde & retrograde amnesia
- pt may seem anxious & repeatedly ask the same question
- no recall of events after the attack
- unknown aetiology, thought to be due to transient ischaemia to the thalamus (amygdala & hippocampus)
- absence of other cognitive/neuro impairments
357
What is transient psycogenic amnesia (vs global)?
Person with psychogenic amnesia is profoundly unable to remember personal information about themselves; there is a lack of conscious self-knowledge which affects even simple self-knowledge, such as who they are
358
What is complex regional pain syndrome?
what are the types?
what are the features?
management?
- umbrella term for a number of neurological & related Sx which typically occur after surgery/minor injury, 3x more common in women
- type I (commonest): no demonstrable lesion to a major nerve
- type II: lesion to a major nerve
- progressive, disproportionate Sx to the original injruy/surgery
- allodynia
- temp & skin colour changes
- oedema & sweating
- motor dysfunction
- early physio important
- neuropathic analgesia
- specialist management e.g. pain team
359
What are the causes of peripheral neuropathy with demyelinating pathology?
- guillain-barre
- CIDP: chronic inflamm demyelin polyneuropathy
- amiodarone
- HSMN type I
- paraprotein neuropathy
360
What are the causes of peripheral neuropathy with axonal pathology?
- etoh
- diabetes mellitus (but may also cause a demyelinating picture)
- vitamin b12 deficiency (*)
- vasculitis
- HSMN type II
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ParinauD syndrome occurs as a result of a lesion at the Dorsal midbrain
- what is the aetiology?
- Sx?
- brain tumours in midbrain or pineal gland
- MS
- midbrain stroke
Rostral interstitial nucleus of medial longitudinal fasciculus lies at the dorsal midbrain & controls vertical gaze; they project to the vestibular nuclei. Therefore Sx inc:
- upward gaze palsy, often manifesting as diplopia
- pupillary light-near dissociation (pseudo argyll robertson pupils)
- convergence-retraction nystagmus
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What is facioscapulohumeral muscular dystrophy?
- autosomal dominant form of muscular dystrophy
- affects face, scapula & UL first
- Sx present ~20yrs
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1ry pathology in a subdural haematoma?
tearing of bridging veins
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1ry pathology in extradural haematoma?
damage to middle meningeal artery
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Which medicine for Parkinsons is ass with valvular dysfunction?
Pergolide
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Thrombectomy for acute ischaemic stroke:
- check pre-stroke functional status
- when to offer
- to whom
- when to consider
- ASAP within 6h of Sx with IV thrombolysis if within 4.5h if ischaemic stroke + proximal anterior circulation occlusion on CTA/MRA
- or ASAP to people last known to be well between 6-24h ago with confirmed proximal anterior circulation on CTA/MRA and if there's a potential to salvage brain tissue as shown by CT perfusion imaging or diffuse-weighted MRI sequences showing limited infarct core volume
Consider thrombectomy with IV thrombolysis if within 4.5h last known to be well upto 24h previously:
- acute ischaemic stroke & confirmed occlusion of proximal posterior circulation (that is basilar/posterior cerebral artery) on CTA/MRA etc
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Pontine haemorrhage
Reduced GCS, paralysis, BL pinpoint pupils
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Watershed infarct
In most vulnerable regions of brain in-between 2 cerebral artery territories
