Neurology Flashcards
(298 cards)
1
Q
What are the investigations done in stroke in a young person?
A
- Confirm the stroke with repeat MRI
- Patent Foramen Ovale (PFO) is present in 45% of young stroke – requires Trans-oesophageal echo to assess septal abnormality further
- PFO is diagnosed by contrast-enhanced echocardiography (bubble test)
2
Q
Difference between TIA and Subclavian Steal Syndrome?
A
TIA: due to blockage, expect stenosis in the mid- to distal carotids
SSS: due to reduced blood flow in the vertebral artery due to proximal subclavian artery stenosis
3
Q
Investigations of choice for Subclavian Steal Syndrome?
A
Duplex ultrasound and MR angiography
4
Q
Management of Subclavian Steal Syndrome?
A
Smoking cessation
Lipid and blood pressure control
Anti-platelet agents
Surgical: endarterectomy and stenting
5
Q
What is the diagnosis in a patient with confusion, dizziness, falls, horizontal-gaze-evoked nystagmus, microcytic anemia?
A
Wernicke encephalopathy - triad of ophthalmoplegia (as nystagmus) + ataxia (falls) + encephalopathy (confusion)
6
Q
Expected laboratory findings in malnutrition?
A
Globally low electrolyte levels
Anaemia
Low MCV
Hypoalbuminaemia
Low BMI
7
Q
What is the diagnosis in a patient with ophthalmoplegia, ataxia, and areflexia?
A
Miller-Fisher Syndrome - typically follows a viral illness
8
Q
What is the triad of Normal-pressure hydrocephalus?
A
Ataxia
Urinary incontinence
Cognitive impairment
9
Q
How is Wernicke encephalopathy treated?
A
Admission is essential - MEDICAL emergency
Consider 1:1 staffing
IV>IM Thiamine ASAP + other B vitamins (Pabrinex then oral B vitamins long-term)
IF with alcohol dependence: PO chlordiazepoxide
10
Q
What is the probable diagnosis in a patient with confusion, confabulation, personality changes, and memory impairment (amnesia)?
A
Korsakoff syndrome - permanent
11
Q
What is the components of Glasgow Coma Scale?
A
Eye opening
4 spontaneously
3 to speech
2 to pain
1
Verbal response
5 orientated
4 confused
3 inappropriate words
2 inappropriate sounds
1
Motor response
6 obeys commands
5 localises to pain
4 flexion withdrawal from pain
3 abnormal flexion (decorticate)
2 abnormal extension (decerebrate)
1
12
Q
What is the diagnosis in a patient with diplopia, fatigue, and eyelid drooping towards the end of the day, difficulty climbing the stairs and getting up from low chairs?
A
Myasthenia Gravis
13
Q
Investigation to confirm Myasthenia Gravis? Gold standard?
A
(+) anti-ACh receptor antibodies > (+) anti-MuSK (muscle-specific receptor tyrosine kinase)
Gold standard is single-fibre EMG
14
Q
How is Myasthenia Gravis managed?
A
Pyridostigmine - 1st line
Immunotherapy - in those with advanced disease (sx other than ocular) Prednisolone > IVIG, Rituximab
Thymectomy
Steroid sparing agents - long-term Cyclosporin, Azathioprine, Mycophenolate
15
Q
What are the early warning signs of Myasthenic Crisis?
A
Precipitated by infection or medications (aminoglycosides)
- Increasing muscle weakness
- Increasing double vision
- quiet breath sounds with reduced chest expansion on chest examination
- haemodynamic instability
16
Q
Test to predict impending respiratory failure in Myasthenia Crisis?
A
PFT: FVC decreased
17
Q
How is Myasthenic Crisis managed?
A
Airway precaution
Plasmapharesis and IVIG
18
Q
What is the diagnosis in a patient with refractory asthma, mononeuritis multiplex, marked eosinophilia, urticarial rash, confusion, generalised seizures?
A
Eosinophilic Granulomatosis with Polyangiitis (EGPA) aka Churg-Strauss
- confusion, generalised seizures is due to vasculitis
19
Q
How is EGPA managed?
A
High-dose corticosteroids
Immunosuppression: azathioprine, methotrexate, cyclophosphamide
20
Q
Investigation of choice for EGPA?
A
Skin biopsy: small-vessel arteriopathy with necrotising granuloma formation with an eosinophilic core and surrounding macrophages and epithelioid giant cells
21
Q
What is the diagnosis in a patient with tremors at rest and on movement, icteric sclerae, behavioural changes, (+)FHx of tremors?
A
Wilson’s Disease
- Neurological: extrapyramidal
- Psychiatric: behavioural
- Hepatic: liver failure and cirrhosis
- Ocular: brownish Kayser-Fleischer rings
- Renal: acute renal failure
- Anaemia: Coombs negative
22
Q
How is Wilson’s disease diagnosed?
A
Copper studies
- increase urinary copper excretion >1.6umol/24h
- reduced serum caeruloplasmin (decreased by 50%)
MRI: face of giant panda sign (midbrain)
Screen siblings once diagnosis confirmed
23
Q
What is the management for Wilson’s disease?
A
Copper chelation with D-penicillamine life-long
Zinc
Transplantation in ESLD
24
Q
What is the probable diagnosis in a 70y/o patient with weakness of both hands, wasting, fasciculations, spasticity, with cognitive impairment but no sensory findings?
A
Motor Neuron Disease (MND)
ALS: MC, LMN arms UMN legs then bulbar
Progressive bulbar palsy: poorest prognosis
Progressive muscular atrophy: best prognosis, LMN only
Primary Lateral Sclerosis: UMN mostly
25
How is MND diagnosed?
EMG: denervation and renervation of affected muscles
MRI of spine and brain: rule out cervical myelopathy
26
What is the management of MND?
MDT management
Riluzole
Gastrostomy
Respiratory support: PPV, PT/OT
Nutritional support: NGT, PEG
Symptomatic: hyoscine, baclofen, diazepam, phenytoin
27
What is Kennedy Disease? vs Spinal Muscular Atrophy?
Kennedy AKA X-linked Spinobulbar Muscular Atrophy
- CAG trinucleotide repeat
- LMN disease + gynecomastia
Spinal Muscular Atrophy
- Autosomal recessive
- Progressive LMN disease
28
What is the diagnosis in a patient with muscle wasting and weakness with delayed relaxation of grip, frontal hair loss, cataract, male infertility?
Myotonic dystrophy
- CTG repeat
May also present with cardiomyopathy and conduction defects, mild intellectual impairment, glucose intolerance, and low IgG
29
What is the possible diagnosis in a patient with clonic movements of the fingers and over seconds include larger portion?
Jacksonian march phenomenon -- focal aware seizures
30
First-time seizures generally do not require medications, except?
First-time onset in an elderly patient -- investigate for structural cause
If young, needs >1 attacks to require AED
31
What are the first-line AED for generalised tonic-clonic seizures?
Sodium valproate
Carbamazepine - CI in primary generalized epilepsy can worsen myoclonus
Lamotrigine
32
What are the first-line AED for focal seizures?
Carbamazepine
Lamotrigine
33
What are the first-line AED for absence seizures?
Ethosuximide
34
What medication can usually be substituted in the first-line AEDs in general?
Levetiracetam
35
What is the management of generalised tonic-clonic seizures lasting >5 minutes?
Community: Buccal midazolam or rectal diazepam repeated every 15 minutes
(+) IV: IV lorazepam repeated 10-20 mins
Secure airway
Give usual AED if already on treatment
Once stable: Phenytoin infusion 15-18mg/kg or Posphenytoin infusion or Phenobarbital bolus
36
What is the management for refractory generalised tonic-clonic seizure?
General anaesthesia
- Propofol titrated
- Midazolam bolus then infusion titrated
- Thiopental titrated then reduced after 2-3 days
Continue anaesthesia for 12-24h after last clinical or electrographical seizure then taper
37
What is the management of status epilepticus in a child?
Airway precaution, administer glucose if needed
O2 if needed
Midazolam PO or Lorazepam IV
Once established: Phenytoin infusion or Phenobarbital infusion
Refractory: Rapid sequence induction of anaesthesia w thiopental IV
38
What is the most common cause of epilepsy-related death in young adults with uncontrolled epilepsy?
SUDEP - Sudden Unexpected Death in Epilepsy
39
What is the most important supplementation in epilepsy during pregnancy?
High-dose folic acid 5mg daily preconceptionally up to 1st 3 months of pregnancy
IF on phenytoin: vitamin K from 36w AOG
Fetal anomaly scan at 18-20w AOG
40
What is the imaging of choice in seizure?
MRI
CT if emergency
41
Which drug is considered as first choice AED?
Lamotrigine for both generalised and focal seizures
42
How is GBS managed?
IVIG
Respiratory support
Possible ICU admission
Neuro rehabilitation
43
What is the most commonly identified cause of GBS?
Campylobacter jejuni
44
What is the diagnosis in a patient with bilateral weakness, hyporeflexia, with history of URTI/UTI/AGE?
Guillain-Barre Syndrome
45
What is the diagnosis in a patient with ophthalmoplegia, diplopia, blurring of vision, descending paralysis in a setting of multiple cutaneous abscess?
Botulism
46
How is Botulism managed?
Antitoxin/Trivalent antiserum
- IV in infants
- Horse-derived heptavalent in adults
Supportive care in ICU: monitoring of FVC, O2, IC
47
What is the diagnosis in a patient with retroorbital headache, conjunctival injection, nasal stuffiness, eye tearing? Diagnostics to be done?
Cluster headache
Clinical dx but head imaging done in new cluster headache to rule out structural abnormalities
48
How is Cluster headache managed?
Acute: High flow nasal oxygen (nonrebreather mask) 12lpm > Sumatriptan
Alt: Intranasal Lidocaine, Steroids
Prophylaxis: Verapamil
Refractory: Topiramate, Sodium valproate
49
What is the most common hereditary chorea? Mode of inheritance?
Huntington’s disease - autosomal dominant
Trinucleotide CAG repeat with anticipation and complete penetrance
50
What is the diagnosis in a patient with myoclonus induced by startle, seizures, ataxia, mental decline, deafness, optic atrophy?
Mitochondrial Myopathy Syndrome
- MERRF (Myoclonic Epilepsy with Red Ragged Fiber)
51
What is the diagnosis in a patient with orthostatic hypotension, impotence, anhidrosis, proteinuria, heart failure, ascites?
Amyloidosis - multi-system amyloid deposition
52
How is Amyloidosis investigated and diagnosed?
Biopsy: Congo red positive stain of amyloid deposits with green birefeingence
Serum and Urine Immunofixation: (+) monoclonal protein
Immunoglobulin free light chain assay: raised lambda kappa ratio in AL Amyloidosis
Organ-specific tests
53
How is Amyloidosis managed?
Depending on type
AL: Autologous Stem cell transplant, Chemotherapy
AA: managed inflammation, Colchicine in FMF
54
What is the diagnosis in a patient with impotence, postural hypotension, urinary incontinence, cerebellar ssx, and parkinsonism? What is the expected MRI finding?
Multiple Systems Atrophy
MRI: hyperintensity of the lateral margin of putamen
55
What is the pathology in Meralgia Paraesthetica?
Entrapment of the lateral cutaneous nerve
56
What is the diagnosis in a patient with pain and pins and needles sensation at anterolateral aspect of thigh worse with walking and standing reproduced on palpation at ASIS?
Meralgia Paraesthetica
57
How is Carpal Tunnel Syndrome managed?
Surgical release of transverse carpal ligament
Alternatives: NSAIDs, wrist splints, corticosteroid injections into wrist
58
What is the diagnosis in a patient with sensory loss at little finger and 1/2 4th finger, pain and weakness at elbow, weakness of hypothenar muscles?
Cubital tunnel syndrome- impingement of ulnar nerve at elbow
59
Nerve involved in wrist drop?
Radial nerve usually at spiral groove
60
How is Meralgia Paraesthetica managed?
NSAIDs, Lidocaine patch
Nerve block
61
What nerve is involved in weak hip flexion, weak knee extension, sensory loss at anteromedial leg?
Femoral nerve
62
What nerve is involved in weak ankle and toe muscles, sensory loss over whole foot and distal lateral leg, weak ankle reflex?
Sciatic nerve (most commonly peroneal division of sciatic nerve)
63
What nerve is involved in weak ankle eversion, foot drop, sensory loss at dorsum of foot and lateral shin, and complete weakness around ankles?
Common peroneal nerve
64
What nerve is involved in radial deviation on wrist extension, finger drop, no sensory loss?
Posterior interosseous nerve
65
What nerve is involved in weak thumb flexion, weak first 2 fingers flexion, no sensory loss?
Anterior interosseous nerve
66
What is the diagnosis in a patient with severe headache, vomiting, papilloedema, who’s taking OCP, retinoids and is obese?
Idiopathic Intracranial Hypertension (IIH) - headache is worse on bending, coughing. Expect CN VI palsy
67
How is IIH diagnosed?
MR venography > CT venography done first to rule out CVST Cerebral Venous Sinus Thrombosis
LP: increased opening pressure >25
68
What is the management for IIH?
Acute: Prednisone, Diuretics, Acetazolamide
Long-term: Weight reduction, discontinuation of offending drugs
Serial LP
Surgical, VP shunting
IF vision is compromised: Optic nerve sheath fenestration or VP shunting
69
How is migraine treated?
Paracetamol
NSAIDs: Ibuprofen, Aspirin
Anti-emetic: Metoclopramide or Prochlorperazine
Triptans PO started at onset of headache (not aura)
70
How is migraine prevented?
Beta blockers: Propranolol
TCAs: Amitriptyline, Nortriptyline
Topiramate, Valproate
Trial for 3 months at maximum dose
Refractory to 2 or more Px agents: Botulinum toxin or CRGP agents
71
What is the management in a patient with unilateral facial weakness with forehead involvement and hyperacusis?
Bell’s Palsy
72
What are the investigations done in a case of Bell’s Palsy?
Electroneuronography: >90% decreased muscle action potential
Needle EMG: absence of voluntary motor unit potentials
Borrelia burgdorferi: rule out Lyme
Pure Tone audiometry: normal
73
How is Bell’s Palsy managed?
High dose Steroids ASAP (within 72h)
Eye protection
Severe: acyclovir, surgical decompression
Ramsay Hunt: Acyclovir
74
How is viral meningitis managed?
Supportive
Confirmed HSV, Varicella zoster, CMV: IV Acyclovir or Valacyclovir
75
Why is LP not done in patients with increased ICP?
Risk of cerebellar tonsillar herniation
76
What is the type of muscular dystrophy in a patient with contracture and cardiomyopathy
Emery-Dreifuss MD
77
Types of muscular dystrophy with behavioural and learning difficulties and has shortened life expectancy?
DMD
Myotonic
BMD
*Emery-Dreifuss - decreased life expectancy but no behavioural and learning difficulties
78
What is the diagnosis in a patient with proximal limb weakness, calf hypertrophy, waddling gait, respiratory difficulties?
Muscular Dystrophy - MC of which is DMD
79
Most sensitive test for Muscular Dystrophy? Confirmatory?
Creatine Phosphokinase - most sensitive, 3 tests 1 month apart is diagnostic
Muscle biopsy - definitive
EMG
USS
Genetic testing
80
How is Muscular Dystrophy managed?
Steroids to strengthen muscles for 6 months to 2 years
Physical aids, PT
Ataluren - for DMD >5 y/o who can still walk
Creatine supplement
Cardiac meds: ACEIs, BBs
Surgical: scoliosis
81
Preferred AED in Juvenile Myoclonic Epilepsy?
Levetiracetam
Lamotrigine - can exacerbate myoclonic jerks in some
Ethosuximide
Valproate
Topiramate
82
What is the diagnosis in a patient with severe continuous pain on shoulder or neck which may radiate to arm which resolves in 2-3 weeks replaced by weakness of the shoulder and upper arms?
Brachial neuritis, treated symptomatically. Functionally resolved but may take up to 12 months
Typically secondary to recent vaccination to deltoid
83
What is the diagnosis in a patient with skin pigmentation, liver cirrhosis, diabetes, erectile dysfunction?
Haemochromatosis
84
What is the diagnosis in a patient with parkinsonism, orthostatic hypotension, erectile dysfunction, cerebellar ssx?
Multiple Systems Atrophy
Autonomic instability: orthostatic hypotension, erectile dysfunction, urinary incontinence
85
What is the diagnosis in a patient with parkinsonism, orthostatic hypotension, erectile dysfunction, dementia, visual hallucinations, paranoia?
Lewy Body Dementia
86
What is the diagnosis in a patient with parkinsonism, vascular parkinsonism?
Corticobasal degeneration
87
What is the diagnosis in a patient with parkinsonism, vertical supranuclear nerve palsy, early falls?
Progressive Supranuclear Palsy - no response w levodopa
88
How is Parkinson’s Disease managed?
Levodopa + Carbidopa (Co-Careldopa) - great with motor symptoms but can also cause motor-related AEs
Ropinirole, Pramipexole, Rotigotine - dopamine agonists - less motor improvement but also less motor AEs
Alt: MAOIs (Selegeline), COMT inh (Entacapone) to improve compliance with levodopa (helps with the motor fluctuations in levodopa treatment)
Refractory: Oral Amantadine - for dyskinesia during on treatment
Advanced: SQ Apomorphine, Duodopa
Deep Brain Stimulation
89
How is gait abnormalities and falls and other non-motor symptoms managed in Parkinson's Disease?
Falls: Physiotherapy
Constipation: Laxative
Postural hypotension: Hydration, salt, fludrocortisone
Bladder: anticholinergics - may have cognitive SEs
Sleep: Clonazepam
Dementia: Cholinesterase inhibitors
Hallucinations: Reduce dopaminergic meds
Psychosis: Clozapine
90
What is the diagnosis in a patient with shooting arm pain worse on coughing, flaccidity and wasting of upper extremities with arreflexia, increased tone and hyperreflexia of the lower limbs, cuts and burns to fingers, UL ptosis and tongue fasciculations?
Syringomyelia - cape-like loss of pain and temperature
LMN in UE + UMN in LE - cervical level
Extension of syrinx upwards cause Horner Syndrome and bulbar symptoms
91
How is Syringomyelia diagnosed and managed?
MRI of brain and spinal cord: LF Chiari malformation
Analgesics if mild pain is the only symptom
Posterior fossa decompression surgery if with signs of neurodegeneration
VP shunt for hydrocephalus
92
How to prevent Analgesia Overuse headache?
Paracetamol or NSAIDs less than 15 days per month
Opiates or triptans less than 10 days per month
93
What are the investigations done to diagnosed Subarachnoid haemorrhage (SAH)?
CT of the brain the sooner the better
IF negative, do LP (ideally 12h post-bleed) with spectrophotometry
IF negative, MR angiography
94
What are the ECG findings that may be seen in SAH?
QT prolongation
Q waves
ST elevation
Dysrhythmias
May mistake for ACS
95
How is SAH acutely managed?
Should be managed in a specialist neurological centre - TRANSFER within 1st 24 HOURS
Aggressive fluid resuscitaiton: 0.9% normal saline
Reduce secondary complications (cerebral ischaemia, hydrocephalus)
- Nimodipine
- Inotropes or vasopressors
- Antihypertensives to keep SBP <180
Prevent further bleeding - occlusion of aneurysm
- Once stabilized
- Surgical clipping of aneurysm
- Coiling of aneurysm
- Surgical evacuation of clot and obliteration of aneurysm in those with large haematoma with focal neurology
96
How is SAH managed in the long-term?
Rehabilitation
Management of RFs: hypertension, smoking
97
What is the diagnosis in a patient with apraxic gait, cognitive impairment, urinary incontinence, inattention?
Normal pressure hydrocephalus (NPH)
Urinary incontinence but no other autonomic dysfunction such as postural hypotension
Apraxic gait: difficulties initiating or sustaining walking, feet seem to stick to the floor
98
What is the diagnosis in a patient with fluctuating level of arousal and varied levels of cognitive impairment, well-formed visual hallucinations, parkinsonism?
Dementia with Lewy Body
- could have REM sleep disorder (remembering vivid dreams)
99
What is the diagnosis in a patient with short-term memory loss, behavioural changes and wandering, apathy, and psychotic symptoms?
Alzheimer's disease
100
What is the diagnosis in a patient with gait disturbance, personality changes, labile mood, urinary symptoms, preserved insight in a setting of CVD?
Vascular dementia
101
What is the diagnosis in a patient with personality change, loss of insight, lack of inhibition, preserved memory?
Frontotemporal degeneration
102
What is the diagnosis in a patient with rapidly progressive myoclonic jerks, seizures, cerebellar ataxia?
Prion disease
103
How is Dementia with Lewy Body managed?
Structured group cognitive stimulation programmes
Memory enhancement strategies
CBT if with anxiety or depression
Cholinesterase inhibitors (Donepezil, Rivastigmine, Galantamine)
- for mild to moderate cognitive symptoms
Dopaminergic medications for parkinsonism
104
Which lobe of the brain is responsible for executive function, social behaviour, speech production and motor?
Frontal lobe
105
Which lobe of the brain is responsible for memory, speech comprehension, verbal semantics, face and object recognition?
Temporal lobe
Dominant: speech comprehension and verbal semantics
Non-dominant: face and object recognition
106
Which lobe of the brain is responsible for reading, writing, calculation, praxis and somatosensory?
Dominant parietal lobe
107
Which lobe of the brain is responsible for visuospatial awareness and dressing?
Non-dominant parietal lobe
108
Which lobe of the brain is responsible for colour and motion perception and visual?
Occipital lobe
109
What is the diagnosis in a patient with bilateral ptosis, difficulty gripping and releasing grip, distal muscle weakness in both hands, worse with cold weather?
Myotonic Dystrophy Type 1 (mutation in DMPK gene) with CTG trinucleotide repeat
Type II has mutation in CNBP gene with tetranucleotide CCTG repeat
Can also present with type II diabetes, cataracts, and cardiac problems
110
How is Myotonic Dystrophy investigated?
Definitive: genetic testing to detect DMPK gene or CNBP gene during Chorionic villus sampling or Amniocentesis
Creatine phosphokinase
EMG
111
What is the diagnosis in a patient with proximal muscle weakness, difficulty climbing stairs, reduced FVC, (+) gene deletion of SMN1?
Spinal Muscular Atrophy
Type 1 - most severe, (+) severe hypotonia, respiratory difficulties, swallowing difficulties
Type 2 - intermediate, muscle weakness of legs > arms, baby can sit but can’t walk, (+) respiratory difficulties
Type 3 - mild, lower limb weakness, difficulty walking, usually no respiratory difficulties
Type 4 - least severe, rare
112
How to manage a patient with Spinal Muscular Atrophy with reduced FVC but does not report nocturnal hypoventilation or daytime hypercapnia?
Risdiplam PO
No need for NIV yet, but will most likely if they develop significant respiratory difficulties
113
What is the diagnosis in a patient with difficulty walking, urinary retention, flaccid paralysis with hyperaesthesia and reduced sensation, with normal vibration and position sense?
Anterior Spinal Artery Syndrome
114
What is the diagnosis in a patient with spastic paralysis with reduced sensation with a history of loss of vision in the eye few months back?
Multiple Sclerosis
Optic neuritis is usually the initial presentation
115
How does optic neuritis usually present?
Diminished visual acuity
Decreased colour perception
Periorbital pain
Visual blurring and diplopia
Fundoscopy usually normal but may present with optic disc swelling in some cases
116
How is Multiple Sclerosis investigated?
MRI of brain and spinal cord: 2 discrete demyelinating lesions disseminated by time and space
CSF: unpaired oligoclonal bands (increased lymphocytes)
117
What is the scoring system used to monitor status of Multiple Sclerosis?
Kurtzke Expanded Disability Status Score
0 - no ssx
10 - death from MS
118
How is Multiple Sclerosis managed in the long-term?
Relapsing-Remiting:
1 relapse in 2 years: SQ Interferon or Glatiramer
2 relapses in 2 years: Teriflunomide Dimethyl Fumarate
Rapidly evolving (2 relapses in 1 year): Natalizumab, Alemtuzumab, Ocrelizumab, Cladribine
Early highly active: Autologous Stem Cell Transplant
Primary Progressive: Ocrelizumab
Secondary Progressive: Siponimod
119
How to manage MS in pregnancy?
Interferon, Glatiramer, Natalizumab are acceptable in pregnancy
Acute relapse: Methylprednisolone > Plasma exchange
120
How to manage an acute relapse of MS?
Methylprednisolone 500mg PO x 5 days or 1g IV x 3 days
121
How to manage spasticity in MS?
Baclofen
122
How to manage bladder dysfunction in MS?
Anticholinergics
Self-catheterisation
123
What is the basic pathology of MS?
Demyelination
124
What is the most significant risk associated with natalizumab?
Increased risk for Progressive Multifocal Leukoencephalopathy due to JC virus
125
What is the diagnosis in a patient with vision loss, rapid, with sudden floaters and flashes, and curtain coming down the vision?
Retinal detachment
126
What is the diagnosis in a patient with vision loss in one eye, pale disc in fundoscopy?
CRAO
127
What is the diagnosis in a patient with vision loss in one eye, hyperaemic retina, flame and dot-blot haemorrhages in fundoscopy?
CRVO
128
What is the diagnosis in a patient with vision loss mainly affecting central vision, later on affecting peripherally
Age-related Macular Degeneration
129
What is the diagnosis in a patient with bilateral vision loss, pain that settles with resting, history of nausea and hiccuping without abnormal investigations?
Neuromyelitis Optica Spectrum Disorder (NMOSD)
History of nausea and hiccup without AB GI tests is Area Postrema Syndrome
130
How is NMOSD investigated?
LP: pleocytosis, negative for oligoclonal bands
(+) AQP4-IgG by cell-based assay
131
How is NMOSD managed?
Methylprednisolone 1g IV x 3d or 500mg PO x 5 days
Plasma Exchange
Steroid tapering until Maintenance Immunosuppression with Mycophenolate, Azathrioprine
*no steroid tapering in MS Mx
Refractory: Rituximab
132
What is the diagnosis in a patient with ataxia, arreflexia of lower limbs, decreased vibration and position sense, extensor plantar reflex, (+)FHx, hearing abnormalities?
Friedrich Ataxia - trinucleotide GAA repeat
- Cardiomyopathy, scoliosis, DM may occur
133
What is the diagnosis in a 30 y/o patient with abrupt cognitive decline, psychiatric abnormalities?
Variant CJD
134
What is the diagnosis in a patient with cognitive decline, dementia, myoclonus, ataxia, psychiatric/behavoural changes?
Sporadic CJD
135
How is CJD investigated?
EEG: periodic sharp and slow wave in Sporadic
LP: abnormally high levels of protein (14-3-3)
MRI: hyperintense signal in thalamus, basal ganglia and cortex
Gold standard: tissue diagnosis but rarely performed
136
How is CJD managed?
No treatment
Myoclonus: Clonazepam, Valproate
Anxiety, depression: sedatives
137
How to managed Restless Leg Syndrome?
Mild, intermittent: lifestyle
Daily symptoms: GABApentinoids, Dopamine agonist (Pramipexole, Ropinirole)
138
How to investigate CNS tumours?
MRI > CT scan
Stereotactic brain biopsy: if negative on CT/MRI
139
How to manage CNS tumours generally?
Radiotherapy for most
Meningioma: watch and wait if symptoms can be treated through supportive measures
Cerebral mets
- Dexamethasone 4mg PO QID
- Anticonvulsants
- Chemotherapy
- Surgical in fit patients, accessible lesion, good prognosis
Primary brain tumour
- Chemotherapy: Temozolomide IV, Carmustine, Gliadel
- Dexamethasone 4mg PO QID
- Anticonvulsants
- Debulking surgery - for fit and young patient but Curative resection not possible
Pituitary tumours
- Prolactinoma: Dopamine agonist
- Transsphenoidal Surgery in functioning + non-functioning with mass effects
140
What are the expected symptoms in Posterior Circulation Stroke?
Cranial nerve abnormalities
Cerebellar dysfunction: vertigo, nystagmus, ataxia
Homonymous hemianopia
Cortical blindness
UL or B sensorimotor deficits
141
What is the acute management of Ischaemic stroke?
Thrombolysis if <4.5H from symptom onset: Alteplase
Aspirin 24H after thrombolysis
Thrombectomy with IV thromblysis if <6H from symptoms onset
Aspirin 300mg PO IF no thrombolysis done
X 2 weeks
142
How to manage Acute Venous Stroke?
Full-dose anticoagulation: heparin then warfarin INR 2-3
143
How to managed Haemorrhagic Stroke?
BP control target SBP 130-140mmHg x 7days
- if SBP >220 at >6H
- if SBP 150-220 at <6H
Medical treatment in those with large haemorrhage but significant comorbids or small haemorrhages, GCS <8
Decompressive Hemicraniectomy
144
When is BP lowering contraindicated in haemorrhagic stroke?
Underlying structural cause: tumour, AVM, aneurysm
GCS <6
For early neurosurgery to evacuate the haematoma
Massive haematoma with poor prognosis
145
When is medical treatment indicated in Haemorrhagic Stroke?
Large haematoma with significant comorbids
Small deep haemorrhage
Posterior fossa haemorrhage
Lobar haemorrhage without hydrocephalus or rapid neurological deterioration
GCS <8
146
When is Decompressive Hemicraniectomyy indicated in Haemorrhagic Stroke?
WITHIN 48 hours
MCA territory + NIHSS >15
Decreased level of consciousness, NIHSS item 1a score of >/=1
CT showing MCA territory >50% infarct
- +/- ACA or PCA
- infarct volume >145cm3 on DW MRI
147
How is TIA managed?
Urgent Admission in those with >1 TIA in a week, ongoing ssx, severe cardiac stenosis, suspected cardio-embolic
ASA 300mg LD x 2 weeks, continued if already on ASA
Specialist stroke physician
- within 24h if TIA <1 week
- within 7d if TIA >1 week
After 2 weeks, shift ASA to Clopidogrel
+ Simvastatin 40mg
148
How is stroke investigated?
CT of the brain
CT angiogram if considering large vessel occlusion
CT/MR venography if haemorrhagic
LP if haemorrhage
RBS
ECG
149
What is the long-term management of Ischaemic stroke?
ECG to assess AFib
- Shift ASA to anticoagulation
MRI if CT is normal
Carotid ultrasound - in those considering carotid stenosis 50-99% — for endarterectomy
After 2 weeks, Start long-term Clopidogrel 75mg OD as maintenance > Dipyridamole 200mg BID
Abstain driving x 4 weeks, no need to inform DVLA unless with persisting neuro ssx
150
How is Trigeminal neuralgia managed?
Carbamazepine 100mg BID titrated every 2 weeks
Surgical decompression if with lesion found on MRI
151
What is the diagnosis in a patient with electric shock-like pain in UL face lasting for seconds to minutes, triggered by speaking, chewing, brushing, combing?
Trigeminal neuralgia - compression of trigeminal nerve
152
What is the diagnosis in a patient with peripheral neuropathy, deteriorating vision with pale discs, B SNHL?
Strachan Syndrome or Combination B vitamin Deficiency
- Mostly B6 and B12 deficiency
- Can present with weakness and hyporeflexia, and mucous lesions
153
What is the diagnosis in a patient with dermatitis, and diarrhea with cognitive decline?
Pellagra - B3 niacin deficiency
154
What is the diagnosis in a patient with pellagra ssx, cerebellar ataxia, and emotional lability?
Hartnup Disease
155
What is the diagnosis in a patient with foot drop, high-arched foot, hammer toes, and distal weakness?
Charcot-Marie-Tooth Disease
156
What is the diagnosis in a patient with fever, headache, vomiting, and confusion and seizures?
Encephalitis - most likely viral
157
What is the diagnosis in a patient with ssx of encephalitis and MRI findings of hyperintense lesions at temporal lobes?
Herpes Encephalitis
158
How is Encephalitis investigated?
FBC CRP ESR LFT U&E blood culture
CT: rule out structural pathology, rule out increased ICP
LP + CSF analysis + CSF PCR
159
How is Encephalitis managed?
Admission for IV antibiotics with antibiotic coverage: IV benzpenicillin
IV acyclovir should not be delayed
160
What is the diagnosis in a patient with IL pain and temperature sensory loss in face, CL loss of pain and temperature sense, IL Horner syndrome, and ataxia? How is this diagnosed?
Lateral Medullary Syndrome AKA Wallenberg
- secondary to PICA or vertebral artery occlusion
- Also presents with IL palatal and vocal cord paralysis
MRI and MRA
161
What is the diagnosis in a patient with headache that resolves with indomethacin?
Paroxysmal hemicrania
162
What is the diagnosis in a patient with LMN weakness of lower and upper extremities persisting for more than 6 months, LP showing increased protein, and MRI showing nerve root enhancement?
CIDP - Chronic Inflammatory Demyelinating Polyradiculopathy
163
How is CIPD managed?
PT, OT
Immunomodulation: Azathioprine, IV Corticosteroids
Plasma exchange
Neuropathic pain: Amitryptiline, Gabapentin, Duloxetine, Pregabalin
164
What is the diagnosis in a patient with tingling sensation of arms and legs, bilateral spastic paralysis, loss of position and vibration sense?
Subacute Degeneration of the Spinal Cord - B12 deficiency
165
How is neuropathic pain managed?
Amitriptyline, Duloxetine, Gabapentin, Pregabalin
Topical capsaicin in those unable to tolerate orally
Alt: Tramadol then review with pain clinic
166
In a patient presenting with generalized body weakness and paralysis in the morning that resolves at the end of the day with normal neurological examination, what should be the next investigation to be done?
U&E - check K - can be Hypokalaemic Periodic Paralysis
167
How is Hypokalaemic Periodic Paralysis prevented?
Acetazolamide
168
How is Aspergillosis investigated?
CXR: consolidation, cavitation
HRCT: halo sign, pulmonary infiltrates
RAST, IgE, beta-glucans
BAL: fungal hyphae and fungal PCR
169
How is Cryptococcal meningitis investigated and managed?
LP with India Ink stain
Amphotericin B, Flucytosine
170
How is candidiasis managed?
Oral: Miconazole oral gel, Nystatin swish and swallow
Oseophageal: azoles
Vaginal: clotrimazole pessary or ointment > fluconazole PO
171
How are Tinea infections managed?
Terbinafine topical
172
How is Aspergillosis managed?
Amphotericin B
173
How is Pulmonary Histoplasmosis managed?
Voriconazole
174
What is the diagnosis in a patient with deteriorating school performance, myoclonic jerks, and visual deterioration, then develops pyramidal signs, rigidity and unresponsiveness?
Subacute Sclerosing Panencephalitis - complication of measles
Visual deterioration secondary to choroidoretinitis
175
What is the diagnosis in a patient with fever, headache, nuchal rigidity, vomiting, history of sinus infection, confusion, twitching of right hand and fingers, MRI showing bony erosion of frontal sinuses?
Subdural empyema - usually secondary to frontal or ethmoidal sinusitis
176
How is Subdural empyema managed?
IV antibiotics: Penicillin + Metronidazole + Cephalosporin
Lorazepam
177
What is a relevant complication of Subdural Empyema?
Cerebral Venous Sinus Thrombosis / Cerebral Thrombophlebitis
178
Investigation of choice when considering Cerebellopontine angle tumour?
MRI
179
What is the diagnosis in a patient with proximal muscle weakness that improves on reinforcement, difficulty walking, easily fatigues after activity, dry mouth, hyporeflexia, difficulty micturition, impotence?
LEMS - paraneoplastic to SCLCa, breast, ovarian, GI cancers
180
What investigation is important in considering LEMS?
CT/PET of chest to LF lung malignancy
Repetitive nerve stimulation: incremental response
181
How is LEMS managed?
3,4-diaminopyridamole 4x a day
Steroids probably
Plasma Exchange, IVIG rarely
182
What is the diagnosis in a patient with hemiparesis, Horner syndrome and hypoglossal palsy, pain in neck and behind ear after head trauma? No vomiting no headache
Carotid artery dissection - investigated with CT/MR angiography
183
What is the diagnosis in a patient with short-term memory loss for 2-3 years, MMSE at 21?
Alzheimer's Disease
184
How is Alzheimer's Disease investigated?
MMSE
MRI: medial temporal lobe atrophy
If diagnosis still uncertain: FDG-PET or SPECT, CSF for total tau
185
How is Alzheimer's Disease managed?
Mild to moderate
- Structured group cognitive stimulation programmes
- Memory enhancement strategies
- CBT in depression
- Cholinesterase inhibitors (Rivastigmine, Donepezil, Galantamine)
Moderate to Severe
- Memantine (NMDA antagonist)
186
What is dot sign in CT scan of the brain?
AKA Sylvian fissure sign or the MCA dot sign is a hyperdense vessel sign indicating acute ischaemic stroke suggesting block in a distal branch of the MCA
187
What is the diagnosis in a patient with recurrent migraine with aura, stroke-like episodes, cognitive difficulties and behavioural abnormalities, (+) FHx?
CADASIL - Cerebral Autosomal Dominant Arteriopathy with Subcortical Infarcts and Leukoencephalopathy
188
What is the most likely diagnosis in a patient with pulsatile tinnitus and X, XI, XII nerve palsies, sweating and palpitations?
Paraganglioma - benign slow growing tumour in the ear
May sometimes produce adrenaline causing sweating, flushing, headache, and tachycardia
189
How to differentiate paraganglioma from acoustic neuroma clinically?
Paraganglioma - pulsatile tinnitus + CN X, XI, XII palsies
Acoustic neuroma - CN V, VI, VII, VIII palsies
190
What is the diagnosis in a patient with unsteady gait, blurred vision, decreased up-gaze and abduction, brisk reflexes and upgoing plantars, confusion and drowsiness weeks after a viral illness?
Bickerstaff's encephalitis
- related to GBS and Miller Fischer
Miller Fisher - areflexia, no confusion
191
How is Bickerstaff's encephalitis investigated and managed?
Anti-GQ1b (+)
Steroids, IVIG, Plasma exchange
192
After a generalised tonic-clonic or complex partial seizure, what metabolic abnormality is expected?
Hyperprolactinaemia - elevated 10-20 min after an episode
May be used to differentiate epilepsy vs PNES (psychogenic non-epileptic seizure) where prolactin will be normal
193
What could be the possible reason behind an anaesthetic nurse developing Subacute Combined Degeneration of Spinal Cord?
Exposure to Nitrous oxide - interference with methionine synthetase causing functional B12 deficiency with B12 levels normal
194
What is the diagnosis in a patient with fever, weight loss, difficulty walking, abnormal speech, HIV (+), CT showing multiple ring-enhancing lesions?
Cerebral Toxoplasmosis
195
How is Cerebral Toxoplasmosis managed?
Sulfadiazine + Pyrimethamine + Folinic acid
196
How to diagnose CMV retinitis?
Ophthalmoscopy: creamy coloured retinal discoloration with overlying haemorrhage (pizza pie appearance)
- same as Retinal Toxoplasmosis
197
How to diagnose PML or Progressive multifocal leukoencephalopathy?
MRI: widespread small white matter lesions
CSF: JC virus DNA
Brain biopsy is definitive but rarely done
198
How to diagnose Cryptococcal meningitis?
CSF staining with India ink: (+) yeast cells
Serum cryptococcal antigen (CRAG) is sensitive and specific in patients with HIV
199
How is Toxoplasmosis diagnosed?
CT: single or multiple ring-enhancing lesions
Toxoplasma serology (+)
200
How to investigate an HIV patient with ophthalmic presentation?
CMV and toxoplasma IgG
CMV viral load
201
How is neurosyphilis investigated?
Treponemal and non-treponemal tests
202
How is cryptococcal meningitis in HIV managed?
Combination antifungal: Amphotericin B + Flucytosine + Fluconazole
203
How is TB meningitis in HIV managed?
Prolonged combination therapy: HRZE x 6-12 months + Pyridoxine supplement
Weaning steroids to mitigate IRIS
204
How is neurosyphilis in HIV managed?
IM penicillin + Probenecid PO x 17 days
205
How is PML in HIV managed?
No specific treatment -- early HAART
206
How is CMV retinitis in HIV managed?
Same day referral to ophthalmology
Severe sight-threatening: Intravitreal ganciclovir + IV valganciclovir
207
How to differentiate vertebral artery vs basilar artery dissection?
Vertebral artery dissection - severe occipital headache and nape pain after head or neck injury, IL facial sensory loss, IL limb or trunk sensory loss, CL loss of pain and temperature, IL loss of taste, vertigo
Basilar artery dissection - rare, commonly SAH symptoms and not lateralising
208
What is the diagnosis in a patient with R ptosis, R eye is down and out, L flaccid paralysis of UE and LE with upgoing plantars?
Weber syndrome - stroke of ventrolateral midbrain secondary to occlusion of paramedian basilar or PCA
209
What is the diagnosis in a patient with finger agnosia, L-R disorientation, dysgraphia, acalculia?
Gerstmann's Syndrome - stroke of dominant parietal lobe involving the angular and supramarginal gyri
210
What is the diagnosis in a patient with IL paresis and loss of vibration and proprioception, CL pain and temperature loss?
Brown-Sequard Syndrome
211
How does a pathology to corpus callosum present?
Alexia without agraphia
212
What is the diagnosis in a patient with impaired adduction of one eye and nystagmus in the other eye during lateral gaze
Internuclear Ophthalmoplegia - damage to the medial longitudinal fasciculus (MLF)
213
What is the diagnosis in a patient with sudden occipital headache with nausea vomiting, brief episodes of R hand weakness, episodes of sensory disturbances, B papilloedema?
CVST or Cerebral Venous Sinus Thrombosis
- Could also present with seizures
214
What is the diagnosis in a patient with personality changes, intellectual impairment which evolves into focal neurological signs in a background of neoplasm, CT showing white matter lesions bilaterally?
Progressive multifocal leukoencephalopathy secondary to JC virus
215
What does an EEG finding of isoelectric activity mean?
Absence of brain activity
216
Cause of Ramsay Hunt
Reactivation of VZV virus in the geniculate ganglion of CN VII
Can present with painful vesicles at external auditory meatus and/or soft palate
217
What is the diagnosis in a 20-year old patient presenting with bilateral cramping calf pain on minimal exercise but settles on perseverance of exercise, present since teenage years, passage of dark urine in the evenings after exercise?
McArdle's disease - deficiency in myophosphorylase (Glycogen Storage Disease V)
Second wind phenomenon - relief of symptoms after a few minutes of exercise
218
How is McArdle's disease diagnosed?
Creatine Kinase raised
Myoglobuinuria
Definitive: Muscle biopsy and Genetic sequencing (PYGM gene)
219
How is McArdle's disease managed?
Carbohydrate-rich diet
Sucrose 5 minutes before all types of aerobic exercise
220
What is the diagnosis in a patient with proximal muscle weakness, calf hypertrophy, waddling gait, Gower's sign?
Duchenne Muscular Dystrophy?
221
How is Duchenne Muscular Dystrophy investigated?
Confirmatory: Genetic testing for DMD gene mutations encoding dystrophin
Creatine Kinase elevated
Muscle biopsy is no longer done due to invasiveness
222
What is the diagnosis in a patient with severe neck pain radiating to shoulder and forearm which resolved after 24h, develops weakness of biceps, shoulder, winging of scapula a week after?
Amyotrophic neuralgia - post-infective
Winging of scapula = serratus anterior = long thoracic nerve = C5 C6 C7
223
What is the diagnosis in a patient with fever, rigidity, stupor, unstable BP, profuse sweating in a patient who suddenly stops taking levodopa?
Neuroleptic Malignant Syndrome
- Can also be seen in those taking antipsychotic neuroleptics
- Can lead to AKI
224
How is Neuroleptic Malignant Syndrome investigated and managed?
Creatine Phosphokinase rise to 60,000
Myoglobinuria
Mx: Reinstitute dopaminergic medication
225
What is the diagnosis in a patient with persistent episodes of severe headache unresponsive to analgesia, hypertension, MR angiography findings of multifocal segmental cerebral artery vasoconstriction?
Reversible Cerebral Vasoconstriction Syndrome (RCVS)
- secondary to sympathomimetic drugs (pseudoephedrine), triptans, amphetamines, cocaine, ecstasy, tacrolimus, bromocriptine
226
How is Intracerebral haemorrhage managed?
ABCDE + O2 + GCS
ABG/VBG
C-spine x-ray or CT
CT head ASAP
MR angiography - especially in younger patients -- consider tumour or vascularmalformations
227
What is the most likelye diagnosis in a patient with learning disability, drowsiness and irritability in the morning after lack of sleep, incontinent through the night, muscle pain? Next investigation indicated?
Nocturnal seizures - common in those with severe learning difficulty
Lack of sleep triggers seizures
For video EEG
228
How is NPH investigated?
CT head or MRI head: normal or mild to moderate enlargement of ventricles, periventricular leukomalacia
Levodopa challenge - to rule out PD
CSF: normal constituents, Normal to slightly elevated opening pressure
229
How is NPH managed?
VP shunting or endoscopic third ventriculostomy
- if with good response to CSF procedures
Control CV RFs
230
What is the most common complication of VP shunting?
Shunt overdrainage - postural headache, subdural effusions or haematoma
231
What is the diagnosis in a patient with complex seizures, cognitive decline, UMN palsy, weakness, and MRI finding of multifocal nodular lesions throughout white matter on T2 that enhances on gadolinium?
What is the confirmatory test? How to managed?
DLBCL
Confirmatory: biopsy
Mx: Methotrexate
232
What is the diagnosis in a patient with fever, headache, myalgia, flaccid paralysis, hyporeflexia, WCC elevated, coming from a flight from New York?
West Nile Virus
- GBS ssx + encephalitis ssx
233
What must be remembered in interpreting phenytoin levels?
Phenytoin is protein-bound, in those with hypoalbuminaemia, total phenytoin measured can be falsely low with unbound active phenytoin is not measured accurately
234
What is the diagnosis in a patient with nystagmus, cerebellar signs, hyperreflexia, seizures, bradyarrhythmia, gum hypertrophy?
Phenytoin toxicity
- Cardiac symptoms are associated with IV phenytoin
Therapeutic range: 10-20 mg/L
235
How is phenytoin toxicity managed?
ABCDE
Activated charcoal - presents <1H, can be multiple doses
Check ECG: if with prolonged QRS + hypotensive unresponsive to fluids -- for Sodium bicarbonate infusion
236
How is Common Peroneal Palsy confirmed?
NCV
Creatine kinase: raised
237
When is serum anti-GM1 antibodies indicated?
Looking for evidence of multifocal motor neuropathy with conduction block
238
What is the diagnosis in a patient with headache, sciatic pain, CN palsies, LMN paresis, history of breast CA, negative malignant cells on CSF Cytospin?
Carcinomatous meningitis - from adenoCA of breast, lung, GIT, melanoma, lymphoma
Initial CSF Cytospin can be normal and would need multiple repeat testing
239
How is Carcinomatous meningitis investigated?
MRI brain: scattered leptomeningeal enhancement on T1
CSF: increased opening pressure, lymphocytosis, increased protein, N/low glucose
Cytospin: malignant cells, may not be seen in initial samples
Rule out fungal and TB meningitis
240
How is Carcinomatous meningitis managed?
Intrathecal chemotherapy
241
What is the diagnosis in a patient with fever, headache, neck stiffness, nonblanching rash, photophobia?
Meningitis likely Bacterial
Glass test for rash: non-blanching
242
What is the triad bradycardia, hypertension, and irregular respiration mean?
Cushing's reflex secondary to cerebral herniation
243
How is Bacterial Meningitis managed?
(+) non-blanching rash
Admission
Benzylpenicillin IM single dose >1200mg
(-) non-blanching rash
Admission
Empirical abx: Benzylpenicillin, Cefotaxime, Chloramphenicol
+ Vancomycin if with travel outside UK
(+) skull fracture - think g(+) Staphylococcus aurea
- IV linezolid > IV vancomycin
244
How to managed contacts of bacterial meningitis?
ALL close contact in the past 7 days prior to sx onset
Ciprofloxacin STAT dose
+ Dexamethasone
245
What are the normal values for LP?
WBC <5
RBC <5
CSF glucose 60% of serum glucose
CSF Pressure <20mmHg
246
Differentiate Bazin's disease vs Bornholm disease
Bazin's Disease - erythema induratum
- inflammatory disorder of the SQ fat tissue
Bornhold disease - epidemic pleurodynia
- secondary to Coxsackie B virus
- paroxysmal chest pain, fever, headache, myalgia
247
How does a partially treated bacterial meningitis present in CSF?
CSF: lymphocytic predominance
248
How is Horner's Syndrome confirmed?
Cocaine drop test: failure to dilate pupil
Apraclonidine: mydriasis on affected eye
Hydroxyamphetamine: dilation in a first- to second-order Horner
249
What should be considered in a patient presenting with painful Horner's Syndrome without anhidrosis? How to manage?
Carotid artery dissection
Initiate DAPT
250
How to manage a patient with possible bacterial meningitis with skull fracture?
Consider g(+) organisms
Start IV linezolid > IV vancomycin
251
What is the most common breathing pattern in coma?
Cheyne-Stokes breathing
252
When is brain death declared?
Absent brainstem reflex
Absent motor response
Absent respiratory drive
253
Management of increased ICP in haemorrhagic stroke?
Hypertonic saline - preferred especially if hypotensive
Mannitol - cause diuresis
254
What is the diagnosis in a patient with headache, meningism, vomiting, CN III, IV, V1, V6 palsies, hypotension, hyponatremia, hyperkalemia, N lumbar puncture findings, and bitemporal superior quadrantanopia, CT normal?
Pituitary apoplexy
CN III, IV, V1, V6 palsies = Cavernous sinus involvement
hypotension, hyponatremia, hyperkalemia = Addisonian crisis
CT scan can be normal, must do MRI
255
What is the diagnosis in a patient with CN III, IV, V1, V6 palsies, headche, periorbital edema, proptosis?
Cavernous Sinus Thrombosis
256
What is the diagnosis in a patient with aneurysm, unilateral red eye, recurrent pain in mouth, episode of DVT?
Behcet Syndrome
Unilateral red eye = anterior uveitis
Pain in mouth = mucosal ulceration
Could also present with recurrent genital ulcers
skin: erythema nodosum, acneoiform, nodular
Positive pathergy test
GIT
257
How is Behcet Syndrome managed?
Steroids
Cyclophosphamide
Methotrexate
Colchicine
Azathioprine
(+) aneurysm or DVT: Cyclophosphamide as second line agent of choice
(+) mucocutaneous ulcers
Topical steroids: triamcinolone
Colchicine, oral steoids, immunosuppressive
(+) eye involvement
Prednisolone + Azathioprine
Prednisolone + Infliximab/Adalimumab
(+) GI
Prednisolone + Infliximab
(+) CNS
Prednisolone + Infliximab
(+) Vascular
Prednisolone + Ciclosporin
258
How is Behcet Syndrome investigated?
Rule out alternate dx
Pathergy test: (+)
RF (-)
ANA (-)
ANCA (-)
HLA-B51 (+)
MRI
LP
Colonoscopy
CT angiography
259
How does a Superior Cerebellar Artery Syndrome present?
IL cerebellar ataxia
IL Horner
CL superficial sensory disturbance
hearing loss
nystagmus to IL side
260
What is the diagnosis in a patient with BOV while reading, R pupil larger than left with reduced reaction to light but better with accommodation, arreflexia, N motor, N sensation?
Holmes-Adie Syndrome - degeneration of ciliary ganglia and postganglionic parasympathetic fibres due to a viral infection
Areflexia - damage to dorsal root ganglion
261
What is the diagnosis in a patient with BOV while reading, R pupil larger than left with reduced reaction to light but better with accommodation, arreflexia, and hypohidrosis?
Ross Syndrome - variant of Holmes-Adie Syndrome
262
How to manage Holmes-Adie Syndrome?
Reading glasses
Pilocarpine eyedrops 3x a day
263
What is the diagnosis in a patient with painless ulcer at groin, lymphadenopathy?
Syphilis - primary
264
What is the diagnosis in a patient with maculopapular rash in palms and soles, patchy alopecia, painless, wart-like lesion ingenitals, inner thighs, and around the anus, oral mucosal lesion, fever, headache, generalised lymphadenopathy?
Syphilis - secondary
Condyloma lata - painless, wart-like lesions that appear in moist areas like the genitals, inner thighs, and around the anus
Patchy lesions of the oral mucosa - snail track ulcers
265
What is the diagnosis in a patient with personality changes, dementia, behavioural defects, ataxia, hallucinations, hypotonia of the face and extremities, dysarthria, hyperreflexia in a patient with history of painless genital ulcer?
Neurosyphilis - Syphilitic paresis (General Paralysis of the Insane)
266
What is the diagnosis in a patient with ataxia, painful paraesthesia, hyporeflexia, loss of vibration and proprioception, argyll robertson pupils?
Neurosyphilis - Tabes dorsali (Syphilitic myelitis)
- destruction of dorsal columns and dorsal nerve roots
267
How is Syphilis investigated?
Full PE: genital, skin, eye, neurologicla, cardiovascular
Refer to GUM clinic
- Serology
- Non-specific: VDRL, RPR
- Specific: TPPA, TPHA
- Dark-field microscopy
All positive test must be confirmed with a different serological test
IF negative, repeat at 6 and 12 weeks post-infection
268
How is Syphilis generally managed (non-Rx)?
Refer to GUM Clinic
Avoidance of sexual contact until successful treatment
Contact tracing
Primary: 3 months
Secondary: 2 years
Tertiary: >2 years
269
How is Syphilis managed pharmacologically?
Early
- Procaine penicillin 750mg IM OD x 10 days
- Benzathine penicillin 2.4mg IM x 1-2 doses 1 week apart
- Doxycycline 100mg PO BD x 14 days
Late
- Benzathine penicillin 2.4mg IM x 3 doses 1 week apart
- Amoxicillin 2g PO TDS + Probenecid 500mg QDS x 28 days
- Doxycycline 200mg PO BD x 28 days
270
What is the diagnosis in a patient with ataxia, cognitive decline, abdominal pain, diarrhoea, bloating, weight loss, diffuse hyperpigmentation, supranuclear ophthalmoplegia, Bell's phenomenon, myoclonus of jaw, mouth and tongue?
Whipple's disease - caused by Tropheryma whipplei
Supranuclear ophthalmoplegia - restricted upward gaze
Myoclonus of jaw, mouth and tongue - oculo-masticatory movement
Also presents with arthralgia
271
How is Whipple's disease investigated?
Jejunal biopsy with PAS stain
CSF: PAS positive
272
How to manage an epileptic patient stable on carbamazepine and wants to start OCP?
Start COCP with at least 50ug ethinyestradiol
- effectiveness of COCPs/POPs decreased by carbamazepine
Do not change carbamazepine as she is already stable on this drug
273
What is the localisation in a patient with CN VI palsy, V1 V2 and V3 palsies?
Petrous temporal region
- All 3 CN V means gasserian ganglion or root involvement
274
How to manage a patient with recurrent TIA and presenting with complete occlusion of internal carotid artery?
ABCDE
Commence DAPT - Aspirin + Clopidogrel x 3 weeks then clopidogrel monotherapy in long-term
NOT indicated for endarterectomy as complete occlusion is contraindicated due to higher risk of stroke and damage
Higher dose of statin: Simvastatin 40mg
BP control, smoking cessation
Refer to stroke physician within 24h if <1 week, within 7 days if >1 week
- DO NOT drive until seen by specialist team or at least 4 weeks
275
When is DAPT given in TIAs and stroke?
Commenced in all TIAs and MINOR stroke (NIHSS <3)
- Usually by TIA clinic, at least ASA 300mg OD at A&E
Major stroke indicated single antiplatelet therapy due to higher risk of bleeding
276
How is TIA investigated?
Blood glucose levels
ECG: LF Afib
CT of head to exclude stroke
At TIA clinic
- MRI brain
- Ultrasound of carotids
277
What is the diagnosis in a patient with disinhibition, cognitive issues, aphasia?
Frontotemporal Dementia - tau protein pathology (Pick's disease)
FTD can develop MND and vice versa
278
How are benign fasciculations managed?
Usually none
Phenytoin, carbamazepine if absolutely necessary
279
What is the diagnosis in an infant with convulsions, irritability, poor feeding, jaundice, vomiting, liver dysfunction, cataracts?
Galactosaemia
280
What is the diagnosis in a patient with severe headache after an orgasm, no other neurological deficit, no meningism, CT and LP normal?
Benign coital headache
281
What is the diagnosis in a patient with ataxia, nystagmus, dysarthria, hearing problems, arreflexia, positive extensors, loss of joint and vibration sense in a 22 year old?
Friedrich's ataxia
- GAA trinucleotide repeat
- Defect in FXN gene coding for fraxatin protein
Dorsal columns: loss of joint and vibration sense
Cerebellum: ataxia, nystagmus
Medulla: dysarthria
Can present with MSK deformities: pes cavus, hammer toes, scoliosis
Can present with hyeprtorphic cardiomyopathy, fibrosis, HF, arrhythmias
Can present with DM, optic atrophy
282
How is Friedrich's ataxia investigated?
NCV: motor velocities >40ms arms, absent sensory potentials
Genetic analysis
ECG: hypertrophy, T wave inversion
MRI of brain and SC: atrophic cervical spinal cord
283
How is Friedrich's ataxia managed?
Antioxidant therapy: coenzyme Q10, Vitamin E
MDT and annual reviews
Diabetes: insulin
284
What is the diagnosis in a patient with pain and paraesthesia of the buttocks radiating to posterolateral thigh to dorsum of foot, sensory loss over dorsum of foot and anterolateral thigh, weakness of hip extension, ankle inversion, great toe extension, N DTRs?
L5 radiculopathy
285
What is the diagnosis in a patient with balanitis, urinary frequency, painful sensations in the legs, reduced sensation in feet, diminished proprioception in toes, absent ankle reflexes, and painless ulceration of the big toe?
Diabetic neuropathy
- commonly presents as distal symmetric sensorimotor polyneuropathy with axonal and demyelinating features
286
How is Diabetic neuropathy investigated?
FBS HbA1c
EMG: active denervation in lower limbs with positive sharp waves and fibrillations
NCV: reduced motor conduction velocities + poorly formed small sensory action potentials
287
How does diabetic amyotrohy present?
Lumbosacral radiculoplexus neuropathy
Asymmetric pain then weakness in the proximal leg along with weight loss
288
How is diabetic neuropathy managed?
Glycaemic control
Good foot care and appropriate foot ware
Gabapentinoid: pregabalin, gabapentin
Amitryptiline, Duloxetine
Tramadol as rescue therapy
Autonomic neuropathy with postural hypotension: fludrocortisone, midodrine
GI: antidiarrhoeals, antiemetics
289
What is the diagnosis in a patient with papilloedema, B CN IV V VI VII palsies, flu-like illness, arthralgia, CSF findings of lymphocytic pleocytosis and raised protein after a camping trip to New England, US?
Neuroborreliosis - Lyme disease
May have erythema chronicummigrains - expanding rash by a tick bite
290
How is Lyme disease investigated?
Borrelia-Burgdorferi serology with ELISA or IFA
291
What is the diagnosis in a patient with Horner syndrome, wasting and weakness of small muscles of the left hand, clawing mostly on left ring and little finger, hypo-aesthesia to pinprick on ulnar border of L hand and forearm?
C8-T1 nerve root involvement -- Brachial plexus injury
292
What is the diagnosis in a patient with Marfanoid habitus, livedo reticularis, malar flush, cognitive impairment, hemiparesis?
Homocystinuria - AB methionine metabolism
Hair is blonde, sparse and brittle
Downward dislocation of lenses
Mild learning disability differentiates it from Marfan syndrome
Abnormal thrombotic hence may present with stroke symptoms
293
What is the diagnosis in a patient with subacute confusion, seizure, afebrile, all PE and labs normal except hyponatraemia?
Limbic encaphalitis - affects temporal lobe
CSF: raised protein, unmatched oligoclonal bands
Paraneoplastic or autoimmune
- Ab to VKCC causing hyponatraemia
294
How is Limbic encaphalitis managed?
Immunosuppression
- IVIG
- Plasma exchange
- Steroids
Treatment of underlying malignancy
295
What is the diagnosis in a patient with dizziness, vomiting, ataxia, R CN V VI palsies, R LMN weakness in UE LE, impaired pain and temperature in L?
AICA Syndrome - lateral pontine infarct
Can also present with Horner syndrome, deafness, tinnitus
296
What is the diagnosis in a patient with short-term memory amensia, intentional myoclonus, recently discharged from ITU?
Lance-Adams Syndrome - after a period of cerebral hypoxia (secondary to stroke or cardiac arrest)
297
How is Lance-Adams Syndrome managed?
Levetiracetam, clonazepam, valproate as first line
No trials on effective treatment
298
