Neuro/Psych/Ophth Flashcards
1
Q
What is the management of absence seizures? Name two medicines
What might you see on EEG?
A
Sodium Valproate
Ethosuximide
EEG: Bilateral symmetrical 3Hz spike and wave pattern
2
Q
How might you manage acute angle closure glaucoma?
A
Urgent referral to ophthalmology
- IV analgesia and antiemetic
- Topical B-blocker and steroid
- IV Acetazolamide
Followed by
Topical pilocarpine
Followed by
Iridotomy 24-48 hrs
3
Q
What is ADEM?
A
Acute disseminated encephalomyelitis
Demylinating disease of CNS
aka post infectious encephalomyelitis
4
Q
What is the time lag for ADEM? What symptoms and signs might you see?
A
A few days to 2 months
Non-specific signs - headache, N&V, fever, recent illness
Multi-focal neurological symptoms - motor and sensory deficits, occulomotor
5
Q
What might you see on investigation of ADEM? What is the management?
A
MRI - supra and infra tentorial demyelination
Management: IV Glucocorticoids
IVIG
6
Q
What is management of sinusitis?
A
Analgesia
Intranasal decongestants/nasal saline - limited evidence
Intranasal corticosteroids - if symptoms for more than 10 days
Oral antibiotics if severe presentation - Phenoxymethylpenicillin
If systemically unwell, signs and sx of more serious illness or high risk of complications - co-amoxiclav
7
Q
What might you see in dry and wet age related macular degeneration? What is the management?
A
Dry (90%) - drusen (yellow round spots in Bruch’s membrane - in macular area)
Wet (10%) - choroidal neovascularisation
Management:
Dry - zinc with anti-oixidant vitamin A, C, E
Wet -anti-VEGF (ranibizumab, bevacizumab, pegaptanib)
8
Q
What is alcoholic hallucinosis?
A
Distinct from Wernicke’s/Korsakoff’s
Psychosis less than 6 months
Auditory hallucinations (often persecutory or derogatory)
Clear conscious
9
Q
What are the features of anterior uveitis?
What HLA is this associated with?
Name some associated conditions?
What is the management?
A
Features: Acute Pupil may be irregular and small Photophobia Red Lacrimation Ciliary flush Impaired visual acuity
Associated with HLA-B27
Associated with: Ankylosing spondylitis Reactive arthritis UC, Crohn's Behcets disease Sarcoidosis
Management:
Ophthalmology
Dilate pupil )atropine, cyclopentolate)
Steroid eye drops
10
Q
What is anti-NMDA receptor encephalitis and what is it associated with? What symptoms?
What is seen on investigation? What types of investigation?
A
Paraneoplastic syndrome
Associated with Ovarian teratomas
Symptoms:
Agitation, hallucinations, delusions, disordered thinking, seizures, insomnia, dyskineasias, autonomic instability
MRI Head - may be normal
MRI FLAIR - abnormalities in deep subcortical limbic structures
CSF - pleiocytosis - may be normal
Treatment: Immunosuppresion IV Steroids Immunogloblins Rituximab Cyclophosphamide Plasma Exchange Resection of teratoma
11
Q
What are anti-psychotic methods of action? Can you name the extra-pyramidal side effects? How to manage it?
A
Dopamine D2 receptor antagonists
Side effects: Parkinsonism Acute dystonia Akathisia (severe restlessness) Tardive dyskinesia
Manage with procyclidine
NOTE: Increased risk of stroke and VTE
12
Q
Name four types of aphasia and where the lesion is located:
A
Wernicke’s (receptive)
- superior temporal gyrus
- forms speech (sentences make no sense, speech fluent. Impaired comprehension)
Broca’s (expressive)
- inferior frontal gyrus
- speech is non-fluent, laboured, halting
- normal comprehension
Conduction aphasia
- arcuate fasiculus
- fluent speech, repitiiion poor
- aware of errors
- normal comprehension
Global aphasia
- lesion affecting all above
- severe expressive and receptive aphasia
13
Q
What is an aryll-robertson pupil?
A
Neurosyphilis, diabetes mellitus
Small, irregular pupils
No response to light (but there is a response to accommodate)
ARP-PRA
Accommodation Reflex Present
Pupillary Reflex Absent
14
Q
What is an arnold-chiari malformation?
A
Downward displacement (or herniation) of cerebellar tonsils through foramen magnum
Non-communicating hydrocephalus
Headache
Syringomyelia
15
Q
What is ataxia telangiectasia? Which age group? What signs? What inheritance pattern?
A
Autosomal Recessive
Combined immunodeficiency disorder
Cerebellar Ataxia Telangiectasia IgA deficiency Age 1-5 years Increased risk lymphoma and leukaemia
16
Q
What are adverse effects of clozapine?
A
Agranulocytosis Reduced seizure threshold Constipation Myocarditis Hypersalivation
17
Q
Name types of autoimmune encephalitis?
A
Autoimmune limbic encephalitis
Rasmussen’s encephalitis
anti-NMDAR (NR1) encephalitis
Glycine-receptor mediated encephalitis
Bickerstaff brainstem encephalitis
18
Q
What is autonomic dysreflexia? How is it managed?
A
Spinal cord injury at or above T6
Sympathetic spinal reflex via thoracolumbar outflow. Centrally mediated parasympathetic response prevented by cord lesion
Extreme HTN, flushing, sweating above level of cord lesion. Agitation
Management: Removal of stimulus (i.e. constipation, urine retention). Treat life threatening HTN or bradycardia
19
Q
Features of benzodiazepine withdrawal?
A
Can occur up to 3 weeks after stopping a long acting drug. Features include: Insomnia Irritability Anxiety Tremor Loss of appetite Tinnitus Perspiration Perceptual disturbances Seizuers
20
Q
Features of brachial neuritis?
A
Acute onset unilateral severe pain
Followed by shoulder and scapular weakness several days later
Minimal sensory changes
21
Q
Name the five types of brain herniation and what they are:
A
- Subfalcine (displacement of cingulate gyrus under falx cerebri)
- Central (downward displacement of brain)
- Transtentorial/Uncal (displacement of uncus of temporal lobe under tentorium cerebelli). –> Ipsilateral fixed, dilated pupil and contralateral paralysis
- Tonsillar (displacement of cerebellar tonsils through foramen magnum)
- Transcalvarial (brain displaced through defect in skull)
22
Q
Neurological disorders by anatomy:
Parietal Lobe (5)
A
Sensory inattention Apraxias Astereognosis Inferior homonymous quadrantanopia Gerstmann's Syndromee (alexia, acalculia, finger agnosia, right-left disorientation)
23
Q
Neurological disorders by anatomy:
Occipital Lobe (3)
A
Homonymous hemianopia
Cortical blindness
Visual agnosia
24
Q
Neurological disorders by anatomy:
Temporal Lobe (4)
A
Wernicke’s
Superior homonymous quadrantanopia
Auditory agnosis
Prosopagnosia
25
Neurological disorders by anatomy:
Frontal Lobe (5)
```
Brocas
Disinhibition
Persveration
Anosmia
INability to generate list
```
26
Neurological disorders by anatomy:
Cerebellum Lesion (2)
Midline - gait and truncal ataxia
Hemisphere lesions - intention tremor, past pointing, dysdiadokinesis, nystagmus
27
Neurological disorders by anatomy:
Wernicke and Korsakoff Syndrome
Medial thalamus and mammillary bodies of the hypothalamus
28
Neurological disorders by anatomy:
Hemiballism
Subthalamic nucleus of basal ganglia
29
Neurological disorders by anatomy:
Huntington Chorea
Striatu (caudate nucleus) of basal ganglia
30
Neurological disorders by anatomy:
Parkinson's disease
Substantia nigra of basal ganglia
31
Neurological disorders by anatomy:
Kluver-Bucy Syndrome (hypersexuality, hyperorality, hyperphagia, visual agnosia)
Amygdala
32
Common tumours that metastases to the brain
```
Lung
Breast
Bowel
Skin (melanoma)
Kidney
```
33
Glioblastoma Multiforme:
Imaging and histology
Imaging: Solid tumour with central necrosis and rim that enhances with contrast. Associated vasogenic oedema
Histology: Pleomorphic tumour cells border necrotic areas
34
Meningioma:
Location and histology
Typically located at falx cerebri, superior sagittal sinus, convexity or skull base
Histology:
Spindle cells in concentric whorls and calcified psammoma bodies
35
How do vestibular schwannomas present?
Hearing loss
Facial nerve palsy
Tinnitus
Histology:
Antoni A or B
Verocay bodies (acellular areas surrounded by nuclear palisades)
36
Histology of pilocytic astrocytoma?
Rosenthal fibres (corkscrew eosinophilic bundle)
37
Histology of Medulloblastoma?
Small blue cells. Rosette pattern of cells with many mitotic figures
Aggressive paediatric brain tumour
38
Histology of Ependymoma?
Perivascular pseudorosettes
39
Histology of oligodendroma?
Calcifications with fried egg appearance
40
Histology of haemangioblastoma. What is it associated with? What is associated paraneoplastic syndrome?
Foam cells with high vascularity
Vascular tumour of cerebellum
Associated with vHL syndrome
Can release Erythropoietin
41
What is a craniopharyngioma?
Solid/cystic tumour of sellar region
Symptoms:
Hydrocephalus
Bitemporal hemianopia
Histology: Derived from remnants of Rathke pouch
42
Investigation of suspected brain tumour?
1. CT for screening
2. MRI with contrast - gold standard
3. CT TAP to exclude extracranial primary
43
What is CADASIL and what does it present with?
Cerebral Autosomal Dominant Arteriopathy with Subcortical Infarcts and Leukoencephalopathy
Rare cause of multi-infarct dementia
Present with migraine and ischaemic stroke in 40s.
44
What is capgras syndrome?
Person holds a delusion
Friend or partner replaced by identical looking imposter
45
What feature is carbamazepine known to exhibit?
Autoinduction
May see return of seizures after 3-4 weeks of treatment
46
What is cataplexy and what is it caused by?
Transient and sudden los of muscular tone
Strong emotion (laughter, fear)
2/3 of Narcolepsy patients have cataplexy
47
What does central retinal artery occlusion look like?
Sudden unilateral vision loss
Afferent pupillary defect
Cherry red spot on pale retina
48
What are the normal values for CSF?
Normal values of cerebrospinal fluid (CSF) are as follows:
pressure = 60-150 mm (patient recumbent)
protein = 0.2-0.4 g/l
glucose = > 2/3 blood glucose
cells: red cells = 0, white cells < 5/mm³
49
What is CIPD? What is the onset? What is protein content in CSF?
Chronic inflammatory demyelinating polyneuropathy
Antibody mediated inflammation
Segmental demyelination of peripheral nerves
Similar to GBS, predominant motor features, but insidious onset over weeks to months
High protein in CSF
Treatment: Steriods and immunosuppressants
50
Features and management of cluster headaches (acute and prophylaxis)?
Headache - 1-2x per day lasting 15mins to 3 hours
Usually at night
Rarely more than 3x daily
Clusters typically 4-12 weeks
Intense sharp stabbing pain around one eye
Autonomic dysfunction: Redness, lacrimation, lid swelling, miosis
<40 yrs
May see transient horner's syndrome
May be triggered by alcohol
Want to smsh head against wall, pace around
Management:
Acute - 100% oxygen. S/C or intranasal triptan
Prophylaxis - verapamil
51
Features of common peroneal nerve lesion?
Foot drop
Weakness of foot dorsiflexion, foot eversion, extensor hallucis longus
Sensory loss over dorsum of foot and lower lateral part of leg
Wasting of anterior tibial and peroneal muscles
NOTE: They tend to have intact ankle inversion and flexion of the big toe.
52
Cranial nerve reflexes:
```
Corneal
Jaw Jerk
Gag
Carotid Sinus
Pupillary Light
Lacrimation
```
Please name afferent limb and efferent limb
Corneal
A: Ophthalmic nerve (V1)
E: Facial nerve (VII)
Jaw jerk
A: Mandibular nerve (V3)
E: Mandibular nerve (V3)
Gag
A: Glossopharyngeal nerve (IX)
E: Vagal nerve (X)
Carotid sinus
A: Glossopharyngeal nerve (IX)
E: Vagal nerve (X)
Pupillary light
A: Optic nerve (II)
E: Oculomotor nerve (III)
Lacrimation
A: Ophthalmic nerve (V1) E: Facial nerve (VII)
53
Features of creutzfeldt-Jakob disease? Investigation findings?
Sporadic vs new variant by age? Treatment?
Dementia (rapid onset)
Myoclonus
Personality change
CSF - normal
EEG: Biphasic, high amplitude sharpwaves
MRI: Hyperintense signals in basal ganglia and thalamus
Sporadic - age 65+
New Variant - age 25. Psych symptoms (anxiety, withdrawal, dysphonia)
Supportive treatment
54
Management of depression in older people
SSRIs first line
Adverse side effect of TCAs is bigger issue
55
Classificaiton of diabetic retinopathy? Old and new classification system!
```
Traditional:
Background
- Microaneurysms
- Blot haemorrhages (<3)
- Hard exudates
```
Pre-proliferative
- cotton wool spots (soft exudates)
- >3 Blot haemorrhages
- Venous beading/looping
- Deep/dark cluster haemorrhages
Proliferative
- Retinal neovascularisation
- Fibrous tissue formation anterior to retinal disc
New Classification:
Mild NPDR
- 1 or more microaneurysm
Moderate NPDR
- Microaneurysms
- Blot haemorrhages
- Hard exudates
- Cotton wool spots, venous beading, intraretinal microvascular abnormalities (IRMA)
Severe NPDR
- Blot haemorrhages and microaneurysms in 4 quadrants
- Venous beading in at least 2 quadrants
- IRMA in at least 1 quadrant
Proliferative
- as above
56
Name five drugs causing peripheral neuropathy?
```
Amiodarone
Isoniazid
Vincristine
Nitrofurantoin
Metronidazole
```
57
DVLA driving restrictions for epilepsy, syncope and TIA
Epilepsy first seizure - 6 months if no other abnormality
Established epilepsy - 12 months without seizure
Epilepsy - no seizure for 5 years - full licence restored
Withdrawal of epilepsy medication: Do not drive until 6 months after last dose
58
DVLA driving restrictions for syncope
Simple faint - none
Single episode explained and treated - 4 weeks
Single episode unexplained - 6 months
Multiple episodes - 12 months
59
DVLA driving restrictions for TIA
Stroke or TIA - 1 month. If no residual deficit, do not inform
Multiple TIA - 3 months off, inform DVLA
60
DVLA driving restrictions for hypomania, mania, acute psychotic disorder, schizophrenia?
Must not drive
| Must inform DVLA
61
What is Duchenne muscular dystrophy? What age?
Progressive muscle weakness from age 5
Calf pseudohypertrophy
Gower's sign
30% have intellectual impairent
X-Linked recessive
Frameshift mutation
62
Becker Muscular dystrophy? What age?
Develops after age 10
X-Linked recessive
Non-frameshift insertion in dystrophin gene
Milder form of disease
63
Contraindications to ECT? Side effects?
Absolute Contraindication:
Raised intracranial pressure
```
Side effects:
Headache
Nausea
Short term memory impairment
Cardiac arrhythmia
Memory loss
```
64
Features of encephalitis? Cause?
| Which lobes? (2) Investigations?
Fever, headache, psychiatric symptoms, seizures, vomiting, focal features
HSV-1
Typically temporal and inferior frontal lobes
Investigations:
CSF - lymphocytosis, elevated protein
PCR for HSV
CT - medial temporal and inferior frontal changes (petechial haemorrhage)
MRI better
EEG - lateralised periodic discharge at 2Hz
65
What should be done for epilepsy in pregnancy?
Advice take 5mg Folic acid
Aim for monotherapy. Risk of uncontrolled epilepsy outweigh risks to foetus.
Breastfeeding safe
If taking Phenytoin - give vitamin K in last month to prevent clotting disorders
Sodium valproate should not be used in pregnancy or childbearing age unless absolutely necessary
66
Treatment of epilepsy:
Generalised seizure
Sodium Valproate
67
Treatment of epilepsy:
Focal seizure
Carbamazepine
68
Treatment of epilepsy:
Generalised tonic clonic (first and second line)
1. Sodium valproate
| 2. Lamotrigine, carbamazepine
69
Treatment of epilepsy:
Absence seizures
1. Sodium valproate or ethosuximide
70
Treatment of epilepsy:
| Myoclonic seizures
first and second line
1. Sodium valproate
| 2. Clonazepam, lamotrigine
71
Treatment of epilepsy:
| Focal Seizrues
first and second line
1. Carbamazepine or lamotrigine
| 2. Levetiracetam, oxcarbazepine, sodium valproate
72
Which seizures may carbamazepine exacerbate?
Absence seizures
Myoclonic seizures
73
What is ergotism and how does it present?
Management?
Can be caused by build up of ergot alkaloids after inhibition of hepatic enzymes
```
Features:
Confusion
Psychosis
Muscle cramps
Seizures
Peripheral and coronary vasospasm
Severe headache
GI sx
MI
Renal infarction
Stroke
```
Supportive management/symptomatic
74
Management of essential tremor? Key features?
1. Propranolol
Autosomal dominant
Postural tremor
Improves with alcohol and rest
Common cause of titubation
75
Causes of bilateral facial nerve palsy? (5)
```
Sarcoidosis
GBS
Lyme disease
Bilateral acoustic neuroma
Bells Palsy
```
76
Causes of unilateral facial nerve palsy?
Sarcoidosis
GBS
Lyme disease
```
Lower motor neuron:
Bells
Ramsay Hunt
Acoustic Neuroma
Parotid Tumour
HIV
MS
Diabetes
```
Upper motor neuron:
Stroke (spares the forehead)
77
What is facioscapulohumeral muscular dystrophy?
Autosomal dominant
Affects face, scapula and upper arms
Symptoms from age 20
78
How to manage a focal dystonia?
Geste antagoniste
| --> palpation of another unaffected part of body
79
What is foster kennedy syndrome? Where is the lesion?
Frontal Lobe
Optic atrophy (ipsilateral)
Central scotoma (ipsilateral)
Papilloedema (contralateral)
Anosmia
80
What is Friedreich's ataxia? Inheritance, age of onset, features?
Autosomal recessive
GAA repeat
Age 10-15 years
```
Cerebellar ataxia
Kyphoscoliosis
Absent ankle jerks
Optic atrophy
Spinocerebellar tract degeneration
HOCM
DM
```
81
Drug management of Generalised anxiety disorder?
Sertraline
82
Drug management of panic disorder? When should be started?
Start immediately with CBT or medication
1. SSRI
- -> otherwise Imipramine or clomipramine
83
What is an delayed grief reaction?
Grieving occurs more than 2 weeks after death
84
What is prolonged grief?
More than 12 months
85
Features of Miller fisher syndrome? What antibodies present?
Varient of GBS
Ophthalmoplegia
Areflexia
Ataxia
Descending paralysis
anti-GQ1b antibodies (90%)
86
Features of Guillain-Barre Syndrome? What antibodies present?
Ascending paralysis
Mild sensory symptoms
May be autonomic involvement
Anti-ganglioside (anti-GM1) in 25%
87
Investigations and management of GBS?
CSF - rise in protein, normal WBC
Never Conduction - demyelination of PNS
Management:
Plasma Exchange
IVIG
FVC to monitor respiratory function
88
When should you CT head immediately (7)
GCS <13 on initial assessment
GCS < 15 at 2 hours
Suspected fracture
Sign of base of skull fracture
Post-traumatic seizure
Focal neurology
>1 vomit
89
When should you CT within 8 hours? (4)
Age 65+
History of bleeding or clotting disorder
Dangerous mechanism
>30 minutes retrograde amnesia
90
What is the Cushings reflex?
HTN
| Bradycardia
91
Symptoms and signs of migraine? What are the criteria?
```
Recurrent severe headache
Unilateral
Throbbing
+/- Aura
Aggravated by or causes avoidance of routine ADLs
```
Criteria:
A - atleast 5 attacks fulfilling B-D
B - 4-72 hours
C - two or more of: 1. Unilateral. 2. Pulsatile. 3. Moderate or severe pain. 4. Cause avoidance of routine physical activity
D - at least on of: causes nausea/vomiting, photophobia/phonophobia
E - not caused by anything else
92
Symptoms and signs of Tension headache?
Recurrent, non-disabling
Bilateral headache
Tight band
93
Symptoms and signs of Cluster headache?
```
Pain 1-2x daily
Last 15 mins to 2 hours
Clusters 4-12 weeks
Intense pain around one eye
Restless during attack
Accompanied by redness, lacrimation lid swelling
```
94
Symptoms and signs of Temporal arteritis?
```
Age 60+
Rapid onset
Unilateral
Jaw claudication
Tender, palpable temporal artery
Raised ESR
```
95
Symptoms and signs of Medication over use headache? How to withdraw?
15 days + per month
Worsened with regular symptomatic medication
May be psych co-morbidity
Simple anaglesia and triptans - withdraw abruptly
Opioids - withdraw slowly
96
What are features and management of herpes zoster ophthalmicus?
Reactivation of VZV in area supplied by opthalmic division of trigeminal nerve
Vesicular rash around eye
Hutchinson's sign - rash on tip or side of nose. Risk factor for occular involvement
Management:
Oral antriviral 7-10 days
IV antivirals if severe or immunocompromised
Ophthalmology if eye involvement
97
What is a Holmes tremor? Management?
Lesion in Red nucleus
Irregular low frequency tremor
Resting, postural and action tremor
Treatment:
Levodopa
Neuroleptic agents
Radiation
98
What is holmes-adie pupil? What is it associated with?
Unilateral 80%
Dilated pupil
Remains small for abnormally long time on constriction
Slowly reactive to accommodation, poorly to light
Associated with absent ankle/knee reflexes
99
Horners Syndrome features?
How are these separated by location and what are the causes?
Miosis
Ptosis
Enophthalmos
Anhidrosis
Central
- Anhidrosis of face, arm and trunk
- Stroke, syringomyelia, MS, Tumour, encephalitis
Pre-ganglionic
- Anhidrosis of face
- Pancoasts tumour, thryroidectomy, trauma, cervical rib
Post-ganglionic
- No anhidrosis
- Carotid artery dissection, carotid aneurysm, cavernous sinus thrombosis, cluster headache
100
What is hereditary sensorimotor neuropathy? How many types? What is type 1 vs type 2 pathology?
7 types
Type 1 - demyelinating
Type 2 - axonal
HSMN 1
- Autosomal dominant
- Motor predominates
- Distal muscle wasting, pes cavus, clawred toe
- Foot drop, leg weakness
101
What are features of huntington's disease?
CAG repeat expansion
```
Chorea
Personality changes
Intellectual impairment
Dystonia
Saccadic eye movements
```
Develop from age 35
102
Features of idiopathic cranial hypertension? Management?
```
Headache
Blurred vision
Palilloedema
Enlarged blind spot
6th Nerve palsy
```
```
Management:
Weight loss
Acetazolamide
Topiramate
Repat Lumber puncture
Optic nerve sheath decompression and fenestration/lumboperitoneal or ventriculoperitoneal shunt
```
103
What is inclusion body myositis?
Cause of myopathy
Cytoplasmic inclusion on muscle biopsy
Proximal and distal muscles
Quads and finger/wrist flexors > extensors
104
Features of intracranial thrombosis? Name three places it can occur
Headache
Nausea and vomiting
Sagittal sinus thrombosis:
- Seizure, hemiplegia
- Parasagittal biparietal or bifrontal haemorrhagic infarctions
- Empty delta sign on CT
Cavernous Sinus Thrombosis:
- Periorbital oedema
- Ophthalmoplegia
- Trigeminal nerve involvement
- Central retinal vein thrombosis
Lateral Sinus Thrombosis:
- 6th and 7th Cranial nerve palsy
NOTE: May get subarachnoid blood
105
Cancer associations of lambert-eaton myasthenic syndrome? What antibody?
Small cell lung cancer
Breast and ovarian cancer
Antibody vs. presynaptic voltage gated calcium channel in peripheral nervous system
Autonomic symptoms - dry mouth, impotence, difficulty micturating
Hyporeflexia
Repeated contraction leads to increased muscle strength
106
What is the other name for lateral medullary syndrome? Which artery? What features?
Wallenberg's Syndrome
Posterior inferior cerebellar artery
Ipsilateral: Dysphagia, facial numbness, temperature loss, CN palsy (i.e. Horner's)
Contralateral: Limb sensory loss
Cerebellar Features:
Ataxia
Nystagmus
107
What is maternally inherited diabetes and deafness (MIDD)?
Mitochondrial disease
```
Snesorineural hearing loss
Diabetes
Stroke like sx
Retinal dystrophy
Proximal myopathy
ESRF
Cardiomyopathy
```
108
Patterns of damage to median nerve?
At wrist
At elbow
Anterior interosseous nerve
Wrist - paralysis and wasting of thenar eminence and opponens pollicis. Sensory loss lateral 2.5 fingers
Elbow - as above plus unable to pronate forearm. Weak wrist flexion, ulnar deviation
Anterior interosseous nerve - Pronation of forearm and weakness of long flexors of thumb and index finger
109
Features of menieres disease? Management?
Recurrent vertigo, tinnitus, hearing loss
Sensation of aural fullness
Minutes to hours
Cease driving until sx resolve
Acute: Prochloperazine
Prevention:
Betahistine
110
Management of migraines?
What specifically for women with predictable menstrual migraine?
What for pregnancy?
Acute:
1. Oral triptan with NSAID/paracetamol
2. If no effect, IV metoclopramide/proclhorperazine
Prophylaxis: If two or more attacks per month
1. Topiramate or propranolol
2. 10 sessions of acupuncture
If child bearing age/pregnant:
Propranolol
Predictable menstrual migraine:
Frovatriptan, zolmitriptan
111
Features of Kearns-Sayre syndrome?
Age <20
External ophthalmoplegia
Retinitis Pigmentosa
Ptosis
112
Features of MELAS?
Mitochondrial encephalomyopathy lactic acidosis and stroke like episodes
113
Features of MERRF
Myoclonus epilepsy with ragged red fibres
114
Features and management of motor neuron disease
Absence of sensory signs
Fasciculations
Mixture of UMN and LMN signs
Does not affect external ocular muscles. No cerebellar signs
Management:
Riluzole - 3 months survival benefit
BiPAP - 7 months survival benefit
115
What is multifocal motor neuropathy? What antibodies?
| What does MMN-CB respond well to?
Acquired autoimmune demyelinating motor neuropathy
Motor conduction block
Slowly progressive, distal motor neuropathy
Anti-GM1 antibodies
MMN-CB responds well to IVIG
116
Diagnostic criteria of multiple sclerosis?
What is Uhthoff's phenomenon?
What is Lhermitter's syndrome?
Investigation?
Evidence of two or more relapses with evidence of two or more lesions
Uhtoff's - worsening vision following rise in body temperature
Lhermitte's - paraesthesiae in limb on neck flexion
MRI - high signal T2 lesions. Periventricular plaques. Dawson fingers on FLAIR
CSF - oligoclonal bands. Increased synthesis of IgG
117
Management of multiple sclerosis?
Acute relapse
Disease modifying drugs
```
Symptom control
Fatigue
Spasticity
Bladder
Oscillopsia
```
Acute relapse
- High dose steroids (5 days)
Disease modifying drugs
- B-IFN
- Glatiramer acetate
- Natalizumab
- Fingolimod
Symptom control
Fatigue - amantadine
Spasticity - baclofen and gabapentin. Physiotherapy
Bladder - intermittent self-catheterisation, anticholinergics if no residual volume
Oscillopsia - gabapentin
NOTE: Glatiramer acetate is not contraindicated in liver dysfunction
Natalizumab - associated with PML and hepatotoxicity
Fingolimod - associated with PML, VZV, tumour formation
118
Two types of multiple system atrophy? Symptoms?
MSA - P (parkinsonian predominant)
MSA - C (cerebellar predominant)
Parkinsonism
Autonomic disturbance
Cerebellar signs
119
Myasthenia Gravis antibodies, investigation and management?
Anti-ACh (85-90%)
Anti-MuSK (40%)
Investigation:
Antibodies
Single fibre electromyography (sensitivity 92-100%)
Tensilon Test
FVC to assess respiratory function. If < 1.5, involve ITU
Management:
anticholinesterase inhibitors (pyridostigmine)
Prednisolone
Thymectomy
Management of crisis:
IVIG
Plasmapheresis
120
Exacerbating factors of myasthenia gravis? (6)
```
penicillamine
quinidine, procainamide
beta-blockers
lithium
phenytoin
antibiotics: gentamicin, macrolides, quinolones, tetracyclines
```
121
Features of myotonic dystrophy? Types?
Age 20-30
Autosomal dominant
Trinucleotide repeat
```
Myotonic facies
Frontal balding
Bilateral ptosis
Cataracts
Dysarthria
```
```
Myotonia
Weakness
Mild mental impairment
DM
Testicular atrophy
Cardiomyopathy/Heart block
Dysphagia
```
Type 1:
DMPK Ch19
Distal weakness more prominent
Type 2:
ZNF9 Ch 3
Proximal weakness more prominent
122
Neck lump features:
Pharyngeal pouch
Older men
Posteromedial herniation
If large, midline lump that gurgles on palpation
Dysphagia
Regurgitation
Aspiration
Chronic cough
123
Neck lump features:
Cystic Hygroma
Congenital lymphatic lesion
Neck (left)
Evident at birth
124
Neck lump features:
Branchial Cyst
Oval, mobile cystic mass
Between SCM and pharynx
Failure of obliteration of second branchial cleft
Early adulthood
125
Nerve conduction studies. Conduction velocity? Amplitude?
Axonal
Demyelinating
Axonal
CV - normal
Amp - reduced
Demyelinating
CV - reduced
Amp - normal
126
Neurofibromatosis features. Type 1 vs Type 2
NF1 Ch 17
NF2 Ch 22
```
Features NF1:
Cafe au lait spots
Axillary/groin freckles
Peripheral neurofibromas
Lisch nodules
Scoliosis
Phaeochromocytomas
```
Features NF2:
Bilateral vestibular schwannomas
Multiple intracranial schwannomas, mengiomas, ependymomas
127
Features and management of neuroleptic malignant syndrome?
Patients taking antipsychotic medication or dopaminergic drugs (when dose stopped or suddenly reduced)
```
Pyrexia
Lead pipe muscle rigidity
Autonomic lability
Tachypnoea
Agitated
Raised CK --> AKI
Reduced reflexes
Hours to days
Normal pupils
```
```
Management:
Stop drug/start if parkinsons
IV Fluids
Dantrolene for muscle rigidity
Bromocriptine
```
128
Features, antibodies of neuromyelitis optica? aka?
Management?
Monophasic or relapse-remitting demyelinating CNS disorder (optic nerves/spinal cord - transverse myelitis)
Two of:
1. Spinal cord lesion involving 3 or more spinal levels
2. Initially normal MRI brain
3. Aquaporin 4 positive antibody
aka Devic Disease
Management:
5 days IV methylprednisolone
Maintenance - azathioprine, rituximab, mycophenolate
129
Management of neuropathic pain?
1. Duoloxetine, amitriptyline, duloxetine, gabapentin, pregabalin
--> if does not work, try another
2. Tramadol for rescue therapy
NOTE: Topical capsaicin for localised neuropathic pain
NOTE: Amitriptyline is contraindicated in arrhythmias and post-MI
130
Features and management of optic neuritis?
```
Unilateral decrease in visual acuity
Red desaturation
Pain on eye movement
RAPD
Central scotoma
```
Management:
High dose steroids
Recovery 4-6 weeks
131
Anti-Hu
Associated cancer and symptoms
Small cell lung cancer and neuroblastoma
Painful sensory neuropathy
Cerebellar syndromes
Encephalitis
132
Anti-Yo
Associated cancer and symptoms
Ovarian and breast cancer
Cerebellar syndrome
133
Anti-Ri
Associated cancer and symptoms
Small cell lung cancer
Retinal degeneration
Ocular opsoclonus-myoclonus
134
Anti-GAD
Associated cancer and symptoms
Breast, colorectal and small cell lung cancer
Stiff person's syndrome
Diffuse hypertonia
135
Purkinje Cell Antibody
Breast cancer
Peripheral neuropathy
136
Triad of parkinson's disease?
How is it different to drug induced parkinsonism?
Investigation if difficult to differentiate between essential tremor and parkinsons?
Bradykinesia
Tremor
Rigidity
Asymmetrical
Drug induced parkinsonism:
- Motor symptoms more rapid onset
- Bilateral
- Rare to have rigidity and rest tremor
Investigation: 123I-FP-CIT SPECT
137
What medicaiton should you give to parkinsons disease if affecting QoL?
Levodopa
138
What should you do if symptoms with parkinsons despite levodopa?
Add one of:
Dopamine Agonist
MAO-B inhibitor
COMT inhibitor
139
Which drugs can cause impulse disorder? What is it more common with?
Any dopaminergic therapy.
More common with:
Dopamine agonist therapy
Previous history of impulsive behaviours
Alcohol
140
If develop excessive sleepiness in day with parkinsons. What can you consider?
Modafinil
141
If develops orthostatic hypotension in parkinsons, what can you consider?
Midodrine
142
Examples of dopamine receptor agonists? (4)
Bromocriptine
Ropinirole
Cabergoline
Apomorphine
143
Examples of monoamine Oxidase B inhibitors?
Selegiline
144
Examples of COMT inhibitors?
Entacapone
| Tolcapone
145
Examples of antimuscarinics?
Procyclidine
Benzotropine
Trihexyphenidyl
146
Examples of demyelinating pathology?
```
GBS
CIDP
Amiodarone
HSMN 1
Paraprotein neuropathy
```
147
Examples of axonal pathology?
```
Alcohol
Diabetes mellitus
Vasculitis
Vitamin B12 deficiency
HSMN 2
```
148
Schizotypal vs Schizoid
Schizotypal
- idea of reference
- odd beliefs and magical thinking
- paranoid ideation
- inappropriate affect
Schizoid
- indifference
- solitary
- lack of interest
- emotional coldness
- few interests
149
Features of pituitary apoplexy?
```
Sudden onset headache
Neck stiffness
Bitemporal superior quadrantic defect
Extraocular nerve palsy
Pituitary insufficiency
```
150
What is POEMS?
```
Polyneuropathy
Organomegaly
Endocrinopathy
M-protein band from a plasmacytoma
Skin pigmentation
```
151
Features of post-lumbar puncture headache? Risk factors?
Management?
24-48 hours post
Last several dasy
Worsens with upright
Improves with recumbent position
Factors which increase risk:
- increased size
- direction of bevel
- not replacing stylet
- multiple attempts
Management:
- analgesia and rest
- blood patch
- epidural saline
- IV caffeine
152
Features and Management of primary open angle glaucoma?
Slow rise in intraocular pressure (>24)
Visual field defect
Cupping of optic disc
Management:
1. Prostaglandin analogue
2. B-blocker, carbonic anhydrase inhibitor, sympathomimetic
3. Surgery
153
Features of progressive supranuclear palsy?
Alternative name?
Steele-Richardson-Olszewski syndrome
Impaired vertical gaze (down gaze worse tha up)
Parkinsonism (bilateral)
Falls
Cognitive impairment
154
What suggests pseudoseizures?
```
Pelvic thrusting
Family member with epilepsy
Female
Cry after seizure
Don't occur when alone
Gradual onset
Short post ictal phase
Eyes closed to resistance
```
Can still have tongue biting and urinary incontinence, but this makes more likely to be true seizure
155
Associations with restless leg syndrome?
Management
Iron deficiency anaemia
Uraemia
DM
Pregnancy
```
Management:
Walking stretching, massage
Rx Iron deficiency
Dopamine Agonist (ropinirole, pramipexole)
Benzodiazepines
Gabapentin
```
156
Features of retinitis pigmentosa?
Asssociated diseases?
- Night blindness
- Tunnel vision
- Fundoscopy - black bone spicule-shaped pigmentation in peripheral retina
Associated:
- Refsum disease (cerebellar ataxia, peripheral neuropathy, deafness, ichthyosis)
- Usher Syndrome
- Abetalipoproteinemia
- Lawrence-Moon-Biedl Syndrome
- Kearns-Sayre Syndrome
- Alports
157
Features of Reye's Syndrome? Management?
Preceding viral illness
Encephalopathy (confusion, seizure, cerebral oedema, coma)
Fatty infiltration of liver, kidney and pancreas
Hypoglycaemia
Supportive management
158
What is the preferred SSRIs?
When should sertraline be used?
What should be used for children?
Adverse effects of SSRIs?
What does triptans and MAOi do with SSRI?
Preferred:
Citalopram or fluoxetine
Sertraline use post MI
Children use fluoxetine
Adverse effects: UGI bleed. Give PPI if also taking NSAID.
Triptans and MAOi increase risk of serotonin syndrome
159
Spinal cord lesions. Combined motor and sensory lesions. Features and tracts affected of:
Brown-Sequard
1. Lateral corticospinal tract
2. Dorsal columns
3. Lateral spinothalamic tract
1. Ipsilateral spastic paresis below lesion
2. Ipsilateral loss of proprioception and vibration sensation
3. Contralateral loss of pain and temperature sensation
160
Spinal cord lesions. Combined motor and sensory lesions. Features and tracts affected of:
Subacute combined degeneration
(Vitamin B12 Deficiency)
1. Lateral corticospinal tracts
2. Dorsal columns
3. Spinocerebellar tracts
1. Bilateral spastic paresis
2. Bilateral loss of proprioception and vibration sensation
3. Bilateral limb ataxia
161
Spinal cord lesions. Combined motor and sensory lesions. Features and tracts affected of:
Friedrich's ataxia
1. Lateral corticospinal tracts
2. Dorsal columns
3. Spinocerebellar tracts
1. Bilateral spastic paresis
2. Bilateral loss of proprioception and vibration sensation
3. Bilateral limb ataxia
4. Cerebellar ataxia
162
Spinal cord lesions. Combined motor and sensory lesions. Features and tracts affected of:
Anterior spinal artery occlusion
1. Lateral corticospinal tracts
2. Lateral spinothalamic tracts
1. Bilateral spastic paresis
2. Bilateral loss of pain and temperature sensation
163
Spinal cord lesions. Combined motor and sensory lesions. Features and tracts affected of:
Syringomyelia
1. Ventral horns
2. Lateral spinothalamic tract
1. Flacid paresis (typically affecting the intrinsic hand muscles)
2. Loss of pain and temperature sensation
164
Spinal cord lesions. Sensory lesion. Features and tracts affected of:
Neurosyphilis (tabes dorsalis)
1. Dorsal columns
| 1. Loss of proprioception and vibration sensation
165
What is a spontaneous intracranial hypotension?
How is it managed?
How is it investigated?
Result of CSF leak.
Headache worse when upright
Investigation:
MRI with gadolinium - patchy meningeal enhancement
Management:
Conservative
Epidural blood patch
166
Stroke by anatomy?
Anterior cerebral artery
Contralateral hemiparesis and sensory loss
Lower extremity > upper
167
Stroke by anatomy?
Middle cerebral artery
Contralateral hemiparesis and sensory loss
Upper extremity > lower
168
Stroke by anatomy?
Posterior cerebral artery
Contralateral homonymous hemianopia with macular sparing
Visual agnosia
169
Stroke by anatomy? aka
Posterior cerebral artery (supply midbrain)
Weber's Syndrome
Ipsilateral CN III palsy
Contralateral weakness of upper and lower extremity
170
Stroke by anatomy? ala
Posterior inferior cerebellar artery
Lateral Medullary Syndrome
Wallenberg syndrome
Ipsilateral: Facial pain and temperature loss
Ataxia, nystagmus
Contralateral: limb/torso pain and temperature loss.
171
Stroke by anatomy? aka
Anterior inferior cerebellar artery
Lateral pontine syndrome
Ipsilateral: Facial paralysis and deafness
+ Symptoms similar in Lateral Medullary Syndrome:
Ipsilateral: Facial pain and temperature loss
Contralateral: limb/torso pain and temperature loss. Ataxia, nystagmus
172
Stroke by anatomy?
Retinal/ophthalmic artery
Amaurosis Fugax
173
Stroke by anatomy?
Basilar Artery
Locked In syndrome
174
What is the ROSIER Score? What is positive score?
Score > 0 positive
LOC -1
Seizure -1
```
New, acute onset:
Asymmetric facial weakness +1
Asymmetric arm weakness +1
Asymmetric leg weakness +1
Speech disturbance _1
Visual field defect +1
```
175
Management of stroke in acute setting?
When should you thrombolyse?
When should you perform thrombectomy?
Maintain BG, O2, Temp and fluid in normal limits
BP should not be lowered unless complications
300mg Aspirin (as soon as haemorrhagic stroke excluded)
AF - anticoagulants not started until 14 days
Statin at 48 hours if cholesterol >3.5
Thrombolysis:
Within 4.5hrs of onset
Haemorrhage excluded
Thrombectomy:
Pre-stroke functional status <3 (Rankin Scale) and >5 (NIHSS)
Within 6 hours if:
- Acute ischaemic stroke with confirmed occlusion of proximal anterior circulation
Within 6-24hrs (incl. wake up strokes) if:
- Acute ischaemic stroke with confirmed occlusion of proximal anterior circulation and potential to salvage tissue as on MRI Diffusion weighted sequence
Consider within 4.5 hours if last well 24 hours before with acute ischaemic stroke confirmed to proximal posterior circulation (basilar, posterior cerebral artery) and potential to salvage brain tissue
176
Secondary prevention of stroke?
1. Clopidogrel
2. Aspirin + MR Dipyridamole (if clopidogrel not tolerated)
3. MR Dipyridamole (if aspirin and clopidogrel not tolerated)
Offer carotid artery endarterectomy if stroke/TIA in carotid territory and not severely disabled:
If stenosis >70% (ECST) or >50% (NASCET) within 14 days
177
What should be assessed in the Oxford Stroke (Bamford) Classification?
If the following is present:
1. Unilateral hemiparesis and/or hemisensory loss of the face, arm and leg
2. Homonymous hemianopia
3. Higher cognitive dysfunction
178
Oxford Stroke Classification.
Partial Anterior Circulation Infarct
Involves smaller arteries
2 of:
1. Unilateral hemiparesis and/or hemisensory loss of the face, arm and leg
2. Homonymous hemianopia
3. Higher cognitive dysfunction
179
Oxford Stroke Classification.
Total Anterior Circulation Infarct
Middle and Anterior Cerebral Arteries
1. Unilateral hemiparesis and/or hemisensory loss of the face, arm and leg
2. Homonymous hemianopia
3. Higher cognitive dysfunction
180
Oxford Stroke Classification.
Lacunar
Perforating arteries around internal capsule, thalamus, basal ganglia
One of:
1. Unilateral hemiparesis and/or hemisensory loss of the face, arm and leg
2. Pure sensory stroke
3. Ataxia hemiparesis
181
Oxford Stroke Classification.
Posterior circulation infarcts
Involves vertebrobasilar arteries.
1 of:
1. Cerebellar or brainstem syndromes
2. LOC
3. Isolated homonymous hemianopia
182
Investigations for Subarachnoid Haemorrhage?
1. CT - hyperdense bright blood
2. Lubmar Puncture - at 12 hours for Xanthochromia
After confirmed:
CT Intracranial Angiogram (for ID of vascular lesion)
+/- Catheter angiogram
183
Treatment of subarachnoid haemorrhage?
Treat causative pathology
- Aneurysm - coil or clip
Vasospasm prevention - 21 days of nimodipine
Vasospasm treatment - hypervolaemia, induced HTN, haemodilution
Hydrocephalus - external ventricular drain / long term VP shunt
184
Complications of subtotal thyroidectomy?
Hypothyroidism
Haematoma (airway compression)
Hypoparathyroidism (parasthesia, spasm)
Recurrent laryngeal nerve palsy (hoarseness, bovine cough)
Superior laryngeal nerve injury (loss of pitch)
Thyroid crisis (fast AF, pulmonary oedema)
185
Differential for sudden loss of vision?
Ischaemic (amaurosis fugax)
Vitreous Haemorrhage
Retinal detachment
Retinal migraine
186
Central retinal vein occlusion
Severe retinal haemorrhages seen on fundoscopy
187
Central retinal artery occlusion
RAPD, cherry red spot on pale retina
188
Vitreous haemorrhage
Sudden visual loss, dark spots (small bleeds - floaters)
189
Retinal detachment
Dense shadow that starts peripherally and progresses centrally
Veil or curtain over the field
190
Posterior Vitreous Detachment
Flashes of light - peripheral field
Floaters - temporal side of central
191
How does superficial siderosis present? What is it?
```
Sensorineural hearing loss
Ataxia
Dementia
Anosmia
Anisocoria
```
It is the deposition of iron in neurons of the CNS
192
Classical presentation of Syringomyelia?
How should this be investigated?
What is it?
Cape like loss of sensation to temperature, spastic weakness, paraesthesia, neuropathic pain, upgoing plantars, bowel and bladder dysfunction
Perservation of light touch, proprioception and vibration
Investigation: MRI Spine and Brain
Formation of fluid filled cavitation within central canal of spinal cord. Commonly caused by arnold chiari malformation obstructing CSF flow
193
Differential for thunderclap headache?
```
subarachnoid hemorrhage
cerebral venous sinus thrombosis
internal carotid artery dissection
pituitary apoplexy
reversible cerebral vasoconstriction syndrome
primary sexual headache
posterior reversible leucoencephalopathy syndrome
acute hypertensive crisis
```
194
Treatment of Tics?
Clonidine
| Atypical antipsychotics
195
What is transient global amnesia
Transient loss of memory function. No cognitive defect
Anxious. Repeatedly ask same question. Aware of defect. Dense anterograde amnesia
No recall after attack
Reassurance and control of vascular risk factors
196
What is a Transient Ischaemic Attack?
a transient episode of neurologic dysfunction caused by focal brain, spinal cord, or retinal ischaemia, without acute infarction.
197
Management of transient ischaemic attack acutely?
What is the follow up?
TIA in last 7 days
TIA >1 week ago
More than 1 TIA
Further management?
Aspirin 300mg
unless (bleeding disorder, on anticoagulant, takes aspirin, aspirin contraindicated)
TIA in last 7 days
- Stroke doc within 24 hours
TIA >1 week ago
- Stroke doc within 7 days
More than 1 TIA
- admit for observation
Further management:
Clopidogrel
(aspirin + dipyridamole if not tolerated)
Carotid Artery Endarterectomy if >70% stenosis ECST or >50% NASCET
198
Differentials of transverse myelitis?
Viral (VZV, HSV< CMV, EBV, Flu, HIV)
Bacterial (Syphilis, Lyme)
Post Infectious
First Sx of MS or NMO
199
List differentials for trigeminal autonomic cephalalgias?
cluster headache
paroxysmal hemicrania
hemicrania continua
short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT syndrome)
short-lasting unilateral neuralgiform headache attacks with cranial autonomic symptoms (SUNA
200
Features of tuberous sclerosis?
Ash leaf spots
Adenoma Sebaceum
Shagreen Patches
Subungal Fibromata
Epilepsy
Developmental problems
Retinal hamartomas
Also:
retinal hamartomas: dense white areas on retina (phakomata)
rhabdomyomas of the heart
gliomatous changes can occur in the brain lesions
polycystic kidneys, renal angiomyolipomata
lymphangioleiomyomatosis: multiple lung cysts
201
Somatisation disorder
Multiple symtoms for at least 2 years
Refuses to accept reassurance or negative results
202
Hypochonrial disorder
Persistent belief in underlying serious disease i.e. cancer
Patient refuses to accept reassurance or negative results
203
Conversion disorder
Loss of motor or sensory function. Patients indifferent to apparent disorder
Doesn't consciously fein (factitious disorder) or seek material gain (malingering)
204
Dissociative disorder
Separate off certain memories from normal consviousness
205
Factitious disorder
Munchausen's syndrome
Intential production of physical or psychological sx
206
Malingering
Fraudulent simulation or exaggeration of sx with intetion for financial or other gain
207
Viral labyrinthitis?
Recent viral infection
Sudden onset
N&V
Hearing may be affected
208
Viral neuronitis?
Recent viral infection
Recurrent vertigo lasting hours or days
No hearing loss
Horizontal nystagmus
Rx: Prochlorperazine
209
Vestibular Schwannoma presentation?
cranial nerve VIII: vertigo, unilateral sensorineural hearing loss, unilateral tinnitus
cranial nerve V: absent corneal reflex
cranial nerve VII: facial palsy
210
What is important about Vigabatrin?
40% of patients develop visual field defects, which may be irreversible
visual fields should be checked every 6 months
211
Features of von Hippel-Lindau Syndrome?
cerebellar haemangiomas: these can cause subarachnoid haemorrhages (consider in the young with family history of cancers below)
retinal haemangiomas: vitreous haemorrhage
renal cysts (premalignant)
phaeochromocytoma
extra-renal cysts: epididymal, pancreatic, hepatic
endolymphatic sac tumours
clear-cell renal cell carcinoma
212
What are the two major side effects of Nataluzumab?
Hepatotoxicity
| Progressive multifocal leukoencephalopathy (caused by the JC Virus)
213
How is multiple sclerosis diagnosed? Who's criteria?
McDonald Criteria
Lesions or clinical episodes must be disseminated in both time and place.
If MRI scan demonstrates demyelinating plaques that are:
- Distinguished in age
- In at least 2 of 4 areas typical of MS
214
What does lymphangioleiomyomatosis look like on CT? What is it associated with?
CT - small irregular lung cysts (innumerable) diffusely distributed
Associated with tuberous sclerosis
215
What is Progressive Multifocal Leukoencephalopathy? (PML)
What is it caused by?
What does it look like on MRI?
Demyelinating disease of CNS
Widespread lesions
JC Virus
Seen in immunosuppression
216
Which drug can cause extrapyramidal effects in children and young adults?
How can you tell it is drug induced?
Metoclopramide
Often present with bilateral symptoms
217
How does corticobasal degeneration present?
Higher order dysfunction
(apraxia, aphasia)
Alien hand syndrome
218
What should upgoing plantars and absent ankle jerks make you think of? (5)
```
Subacute combined degeneration of the cord
Motor neurone disease
Friedrich's ataxia
Tabes Dorsalis
Dual peripheral with central pathology
```
219
What is SUNCT and can you name some features?
Age >40
Any time of day
Last seconds to minutes
Up to 75x per day
220
Features of Paroxysmal Hemicrania?
Management?
5x per day
Last 2-30 minutes
Side locked
Retro-orbital/temporal
Autonomic parasympathetic features (watering eye, red eye, runny nose, miosis) ipsilateral to side of pain
Responds to indomethacin
221
Features of Hemicrania Continua?
Management
No headache free periods
Side locked
Retro-orbital/temporal
Autonomic parasympathetic features (watering eye, red eye, runny nose, miosis) ipsilateral to side of pain
Responds to indomethacin
222
What is an acephalgic migraine?
Diagnosis of exclusion
Auras without a headache
223
Features and management of idiopathic intracranial hypertension?
Headaches worse on coughing. Worse lying down
Pulsating tinnitus
Papilloedema
Diagnosis with Modified Dandy Criteria (CSF Pressure >25 mmHg and normal MRI)
Overweight
Management:
weight loss
Stop causative agents (tetracyclines, retinoids, contraceptives, steroids, levothyroxine, lithium, cimetidine)
Acetazolamide
Lumbar-peritoneal shunt/repeat LP
224
What is posterior reversible leucoencephalopathy syndrome? (PRES) How does it present?
Thunderclap headache. Followed by:
Confusion
Seizure
Visual Sx
Normal CT and LP
Common cause:
HTN encephalopathy
Eclampsia
Diagnosis:
Vasogenic brain oedema on MRI
225
Features of low pressure headache on MRI?
Meningeal enhancement
| Thickening and shallow subdural haematoma
226
What is reversible cerebrovascular vasoconstriction syndrome?
Hypertension
Seizures
Arterial beeding on angiography
227
What is management of vertebral artery dissection?
Anticoagulation (once bleed excluded)
Prevents further VTE complications
228
Exclusion criteria for stroke thrombolysis?
History
Stroke or head trauma within the last 3 months
Prior history of intracranial haemorrhage
Major surgery within 14 days
Gastrointestinal or genitourinary bleeding within 21 days
MI in the last 3 months
Lumbar puncture within the last 7 days
Arterial puncture at non-compressible site in the last 7 days
Clinical
```
Rapidly improving stroke syndrome
Minor/isolated neurological deficit
Seizure at the onset of stroke
Symptoms suggestive of sub arachnoid haemorrhage even if imaging is normal
Acute MI or post-MI pericarditis
BP > 185 mmHg systolic or >110mmHg diastolic or aggressive therapy required to control BP
Pregnancy or lactation
Active bleeding or acute trauma
```
Laboratory
Platelets <100,000/mm3
Serum glucose <2.8 mmol/l or >21.2 mmol/l
INR >1.7 if on warfarin
Elevated APTT if on heparin
229
What is malignant middle cerebral artery syndrome? How is it managed?
Extensive ischaemic stroke (usually MCA)
Intracranial HTN develops due to swelling
Present with reduced conscious level
Urgent decompressive craniotomy
Criteria:
<60
NIHSS>15
Reduced conscious to score 1 or more on item 1a of the NIHSS.
CT infarct atleast 50% of MCA or infarct volume > 145 cm3 on DWI MRI
230
Imaging studies and their use:
DWI-MRI
FLAIR MRI
CTA and DSA
DWI-MRI - acute stroke
FLAIR MRI - chronic ischaemic change
CTA and DSA - significant intravascular stenoses or thrombosis
231
What scan can be used to aid diagnosis of parkinson's disease?
DaT Scan
232
What VTE prophylaxis should be given for stroke patients?
Intermittent pneumatic compression for 30 days or until mobile
233
Should you reverse a benzodiazepine overdose?
No.
Only if you gave the drug
This is because they might withdraw - you do not know if they are dependent
Safer to intubate
234
What should happen in MS during pregnancy and afterwards?
'relapse rates may reduce during pregnancy and may increase 3 to 6 months after childbirth before returning to pre-pregnancy rates'.
does not increase the risk of progression of disease; the net effect of protection during pregnancy and subsequent increased relapse rate carries no increased risk of exacerbation.
235
What is valproate associated hyperammonaemic encephalopathy? Treatment?
Non-specific symptoms
Abnormal ammonia
Occur in all levels of dosing
Treat with L-carnitine
236
Are oligoclonal bands in CSF specific for MS?
No.
Also found in:
```
Lyme disease
Systemic lupus erythematosus
Neurosarcoidosis
Subacute sclerosing panencephalitis
Subarachnoid hemorrhage
Syphilis
Primary central nervous system lymphoma
Sjögren's syndrome
Guillain-Barre syndrome
Meningeal carcinomatosis
Neuromyelitis optica
```
237
Who should be treated after one episode of epilepsy?
after 2 seizures
OR
risks of a further seizure is unacceptable to the patient
an underlying structural defect is proven to be the likely cause of the seizure
EEG demonstrated epileptiform activity.
238
What is SUNA?
Short unilateral neuralgiform pain with autonomic symptoms
239
Vague descriptions of CSF in viral, bacterial, GBS and TB
Features of cerebrospinal fluid analysis in HSV encephalitis include an elevated white blood cell count with lymphocytic predominance and an elevated protein.
Bacterial meningitis causes a clear, cloudy or purulent CSF sample, an elevated opening pressure, raised white cell count (unless partially treated), low glucose (<40% of serum glucose) and an elevated protein count. (answer 2)
Guillain Barré syndrome causes a clear CSF sample, a normal or elevated opening pressure, a normal or elevated white cell count, a normal glucose with an elevated protein level. (answer 3)
Tuberculosis causes a clear or opaque CSF sample, an elevated opening pressure, a raised white cell count, a very low glucose and an elevated protein. (answer 5)
240
How should atypical trigeminal neuralgia be investigated? i.e. with sensory loss
MRI with gadalonium
241
What is hereditary neuropathy with liability to pressure palsy?
Trivial trauma to a peripheral nerve results in mononeuropathy which may take weeks to resolve
Demyelinating neuropathy
Conservative management
242
What is multifocal motor neuropathy?
Asymmetric muscle weakness in distribution of named nerve
Slowly progressive
No sensory signs
No wasting
Area of nerve conduction block in MMNCB
Management:
IVIG
243
What is the causative agent of meningitis from an ear infection?
Streptococcus pneumoniae
244
What are the features and causative agent of tropical spastic paraparesis?
HTLV1
Paraparesis
Urinary retention
Treatment:
Steroids
245
What is writers cramp?
Focal dystonia
Flexion, extension or rotation of the muscles of the hund
246
What is first line investigation for neck mass?
USS Neck
247
What is a watershed infarct?
Ischaemia occurs in most vulnerable region of brain between two artery territories
248
What are the key featrues of Kearns-Sayer Syndrome? What type of disease is it?
What is seen on muscle biopsy?
Progressive external ophthalmoplegia
Pigmentary retinopathy
Cardiac conduction defects
Mitochondrial disease
Muscle biopsy - ragged red fibres
249
What is seen in a left sided ventral pontine syndrome? aka
Millard Gubler
Ipsilateral facial weakness
Contralateral body hemiparesis
250
What would you expect to see in CSF for lyme disease?
CSF shows a lymphocytic pleocytosis
251
Can you name some drugs which can cause lymphadenopathy?
Phenytoin
Carbamazepine
Allopurinol
Sulphonamides
252
What symptoms should you warn patients starting on levetiracetam about?
Irritabiliy
Frank aggression
May be best to avoid if history of psychotic symptoms
253
What is the only licensed treatment for parkinsons disease dementia?
Rivastigmine
254
How should hyperglycaemia be managed in NG fed patients?
Never give PRN actrapid
Never stop NG feed
Aim 6-12 mmol/l
When over 12, use biphasic insulin
255
What is the rule of fours for CN localisation?
Rule 1:
1. There are four CNs which originate from the the medulla (CN IX-XII)
2. There are four CNs which originate from the pons (CN V-VIII)
3. There are four CNs which originate from above the pons (CN I and CN II from the cerebrum, and CN III and IV from the midbrain)
Rule 2: There are 4 structures in the ‘midline‘ beginning with M
1. Motor pathway (corticospinal tract)
2. Medial lemniscus
3. Medial longitudinal fasciculus
4. Motor nuclei of CNs III, IV, VI and XII
Rule 3: There are 4 structures to the ‘side‘ (lateral) beginning with S
1. Spinothalamic tract
2. Sympathetic fibres
3. Sensory nuclei of CN V
4. Spinocerebellar tract
Rule 4:
1. The 4 motor nuclei that are in the midline are those that divide equally into 12 except for 1 and 2, that is 3, 4, 6 and 12 (5, 7, 9 and 11 are in the lateral brainstem)
256
What does MELAS present with? What does it mean? What type of disease is it?
Mitochondrial encephalopathy with lactic acidosis and stroke like episodes
Mitochondrial disease
```
Significant cognitive impairment
Lactic acidosis
Multiple ischaemic infarcts inconsistent with vascular territories
Seizures
Dementia
```
257
What is capgras syndrome?
Believes someone close is replaced by an imposter
258
What is cotard syndrome?
Believe they or part of them is dead
259
What is fregoli syndrome?
Believe an individual has taken on may different guises
260
What is MERRF and how does it present?
Myoclonic epilepsy with ragged red fibres
Mitochondrial DNA disorder
```
Ragged red fibres on ibopsy
Optic atrophy
WPW
Myoclonic jerks
Seizures
```
261
Name three drug induced causes of tics
Methylphenidate
Dextroamphetamine
Lamotrigine
262
What is characteristic of McArdle disease?
Second Wind
Occurs as change in fatty acid metabolism
263
What is Usher's syndrome? What are the sympoms?
Autosomal recessive
Deaf-Blind
