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

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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

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3
Q

What is ADEM?

A

Acute disseminated encephalomyelitis

Demylinating disease of CNS

aka post infectious encephalomyelitis

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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

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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

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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

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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)

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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

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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

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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
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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

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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
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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

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14
Q

What is an arnold-chiari malformation?

A

Downward displacement (or herniation) of cerebellar tonsils through foramen magnum

Non-communicating hydrocephalus
Headache
Syringomyelia

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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
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16
Q

What are adverse effects of clozapine?

A
Agranulocytosis
Reduced seizure threshold
Constipation
Myocarditis
Hypersalivation
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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

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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

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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
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20
Q

Features of brachial neuritis?

A

Acute onset unilateral severe pain

Followed by shoulder and scapular weakness several days later

Minimal sensory changes

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21
Q

Name the five types of brain herniation and what they are:

A
  1. Subfalcine (displacement of cingulate gyrus under falx cerebri)
  2. Central (downward displacement of brain)
  3. Transtentorial/Uncal (displacement of uncus of temporal lobe under tentorium cerebelli). –> Ipsilateral fixed, dilated pupil and contralateral paralysis
  4. Tonsillar (displacement of cerebellar tonsils through foramen magnum)
  5. Transcalvarial (brain displaced through defect in skull)
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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)
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23
Q

Neurological disorders by anatomy:

Occipital Lobe (3)

A

Homonymous hemianopia
Cortical blindness
Visual agnosia

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24
Q

Neurological disorders by anatomy:

Temporal Lobe (4)

A

Wernicke’s
Superior homonymous quadrantanopia
Auditory agnosis
Prosopagnosia

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25
Q

Neurological disorders by anatomy:

Frontal Lobe (5)

A
Brocas
Disinhibition
Persveration
Anosmia
INability to generate list
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26
Q

Neurological disorders by anatomy:

Cerebellum Lesion (2)

A

Midline - gait and truncal ataxia

Hemisphere lesions - intention tremor, past pointing, dysdiadokinesis, nystagmus

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27
Q

Neurological disorders by anatomy:

Wernicke and Korsakoff Syndrome

A

Medial thalamus and mammillary bodies of the hypothalamus

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28
Q

Neurological disorders by anatomy:

Hemiballism

A

Subthalamic nucleus of basal ganglia

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29
Q

Neurological disorders by anatomy:

Huntington Chorea

A

Striatu (caudate nucleus) of basal ganglia

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30
Q

Neurological disorders by anatomy:

Parkinson’s disease

A

Substantia nigra of basal ganglia

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31
Q

Neurological disorders by anatomy:

Kluver-Bucy Syndrome (hypersexuality, hyperorality, hyperphagia, visual agnosia)

A

Amygdala

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32
Q

Common tumours that metastases to the brain

A
Lung
Breast
Bowel
Skin (melanoma)
Kidney
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33
Q

Glioblastoma Multiforme:

Imaging and histology

A

Imaging: Solid tumour with central necrosis and rim that enhances with contrast. Associated vasogenic oedema

Histology: Pleomorphic tumour cells border necrotic areas

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34
Q

Meningioma:

Location and histology

A

Typically located at falx cerebri, superior sagittal sinus, convexity or skull base

Histology:
Spindle cells in concentric whorls and calcified psammoma bodies

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35
Q

How do vestibular schwannomas present?

A

Hearing loss
Facial nerve palsy
Tinnitus

Histology:
Antoni A or B
Verocay bodies (acellular areas surrounded by nuclear palisades)

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36
Q

Histology of pilocytic astrocytoma?

A

Rosenthal fibres (corkscrew eosinophilic bundle)

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37
Q

Histology of Medulloblastoma?

A

Small blue cells. Rosette pattern of cells with many mitotic figures

Aggressive paediatric brain tumour

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38
Q

Histology of Ependymoma?

A

Perivascular pseudorosettes

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39
Q

Histology of oligodendroma?

A

Calcifications with fried egg appearance

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40
Q

Histology of haemangioblastoma. What is it associated with? What is associated paraneoplastic syndrome?

A

Foam cells with high vascularity
Vascular tumour of cerebellum

Associated with vHL syndrome

Can release Erythropoietin

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41
Q

What is a craniopharyngioma?

A

Solid/cystic tumour of sellar region

Symptoms:
Hydrocephalus
Bitemporal hemianopia

Histology: Derived from remnants of Rathke pouch

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42
Q

Investigation of suspected brain tumour?

A
  1. CT for screening
  2. MRI with contrast - gold standard
  3. CT TAP to exclude extracranial primary
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43
Q

What is CADASIL and what does it present with?

A

Cerebral Autosomal Dominant Arteriopathy with Subcortical Infarcts and Leukoencephalopathy

Rare cause of multi-infarct dementia

Present with migraine and ischaemic stroke in 40s.

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44
Q

What is capgras syndrome?

A

Person holds a delusion

Friend or partner replaced by identical looking imposter

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45
Q

What feature is carbamazepine known to exhibit?

A

Autoinduction

May see return of seizures after 3-4 weeks of treatment

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46
Q

What is cataplexy and what is it caused by?

A

Transient and sudden los of muscular tone

Strong emotion (laughter, fear)

2/3 of Narcolepsy patients have cataplexy

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47
Q

What does central retinal artery occlusion look like?

A

Sudden unilateral vision loss

Afferent pupillary defect

Cherry red spot on pale retina

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48
Q

What are the normal values for CSF?

A

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

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49
Q

What is CIPD? What is the onset? What is protein content in CSF?

A

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

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50
Q

Features and management of cluster headaches (acute and prophylaxis)?

A

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

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51
Q

Features of common peroneal nerve lesion?

A

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.

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52
Q

Cranial nerve reflexes:

Corneal
Jaw Jerk
Gag
Carotid Sinus
Pupillary Light
Lacrimation

Please name afferent limb and efferent limb

A

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)

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53
Q

Features of creutzfeldt-Jakob disease? Investigation findings?
Sporadic vs new variant by age? Treatment?

A

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

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54
Q

Management of depression in older people

A

SSRIs first line

Adverse side effect of TCAs is bigger issue

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55
Q

Classificaiton of diabetic retinopathy? Old and new classification system!

A
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

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56
Q

Name five drugs causing peripheral neuropathy?

A
Amiodarone
Isoniazid
Vincristine
Nitrofurantoin
Metronidazole
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57
Q

DVLA driving restrictions for epilepsy, syncope and TIA

A

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

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58
Q

DVLA driving restrictions for syncope

A

Simple faint - none

Single episode explained and treated - 4 weeks

Single episode unexplained - 6 months

Multiple episodes - 12 months

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59
Q

DVLA driving restrictions for TIA

A

Stroke or TIA - 1 month. If no residual deficit, do not inform

Multiple TIA - 3 months off, inform DVLA

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60
Q

DVLA driving restrictions for hypomania, mania, acute psychotic disorder, schizophrenia?

A

Must not drive

Must inform DVLA

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61
Q

What is Duchenne muscular dystrophy? What age?

A

Progressive muscle weakness from age 5

Calf pseudohypertrophy

Gower’s sign

30% have intellectual impairent

X-Linked recessive

Frameshift mutation

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62
Q

Becker Muscular dystrophy? What age?

A

Develops after age 10

X-Linked recessive

Non-frameshift insertion in dystrophin gene

Milder form of disease

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63
Q

Contraindications to ECT? Side effects?

A

Absolute Contraindication:
Raised intracranial pressure

Side effects:
Headache
Nausea
Short term memory impairment
Cardiac arrhythmia
Memory loss
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64
Q

Features of encephalitis? Cause?

Which lobes? (2) Investigations?

A

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

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65
Q

What should be done for epilepsy in pregnancy?

A

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

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66
Q

Treatment of epilepsy:

Generalised seizure

A

Sodium Valproate

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67
Q

Treatment of epilepsy:

Focal seizure

A

Carbamazepine

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68
Q

Treatment of epilepsy:

Generalised tonic clonic (first and second line)

A
  1. Sodium valproate

2. Lamotrigine, carbamazepine

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69
Q

Treatment of epilepsy:

Absence seizures

A
  1. Sodium valproate or ethosuximide
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70
Q

Treatment of epilepsy:

Myoclonic seizures
first and second line

A
  1. Sodium valproate

2. Clonazepam, lamotrigine

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71
Q

Treatment of epilepsy:

Focal Seizrues
first and second line

A
  1. Carbamazepine or lamotrigine

2. Levetiracetam, oxcarbazepine, sodium valproate

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72
Q

Which seizures may carbamazepine exacerbate?

A

Absence seizures

Myoclonic seizures

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73
Q

What is ergotism and how does it present?

Management?

A

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

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74
Q

Management of essential tremor? Key features?

A
  1. Propranolol

Autosomal dominant
Postural tremor
Improves with alcohol and rest
Common cause of titubation

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75
Q

Causes of bilateral facial nerve palsy? (5)

A
Sarcoidosis
GBS
Lyme disease
Bilateral acoustic neuroma
Bells Palsy
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76
Q

Causes of unilateral facial nerve palsy?

A

Sarcoidosis
GBS
Lyme disease

Lower motor neuron:
Bells
Ramsay Hunt 
Acoustic Neuroma
Parotid Tumour
HIV
MS
Diabetes

Upper motor neuron:
Stroke (spares the forehead)

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77
Q

What is facioscapulohumeral muscular dystrophy?

A

Autosomal dominant

Affects face, scapula and upper arms

Symptoms from age 20

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78
Q

How to manage a focal dystonia?

A

Geste antagoniste

–> palpation of another unaffected part of body

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79
Q

What is foster kennedy syndrome? Where is the lesion?

A

Frontal Lobe

Optic atrophy (ipsilateral)
Central scotoma (ipsilateral)
Papilloedema (contralateral)
Anosmia

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80
Q

What is Friedreich’s ataxia? Inheritance, age of onset, features?

A

Autosomal recessive
GAA repeat

Age 10-15 years

Cerebellar ataxia
Kyphoscoliosis
Absent ankle jerks
Optic atrophy
Spinocerebellar tract degeneration
HOCM
DM
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81
Q

Drug management of Generalised anxiety disorder?

A

Sertraline

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82
Q

Drug management of panic disorder? When should be started?

A

Start immediately with CBT or medication

  1. SSRI
    - -> otherwise Imipramine or clomipramine
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83
Q

What is an delayed grief reaction?

A

Grieving occurs more than 2 weeks after death

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84
Q

What is prolonged grief?

A

More than 12 months

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85
Q

Features of Miller fisher syndrome? What antibodies present?

A

Varient of GBS

Ophthalmoplegia
Areflexia
Ataxia
Descending paralysis

anti-GQ1b antibodies (90%)

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86
Q

Features of Guillain-Barre Syndrome? What antibodies present?

A

Ascending paralysis
Mild sensory symptoms
May be autonomic involvement

Anti-ganglioside (anti-GM1) in 25%

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87
Q

Investigations and management of GBS?

A

CSF - rise in protein, normal WBC
Never Conduction - demyelination of PNS

Management:
Plasma Exchange
IVIG
FVC to monitor respiratory function

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88
Q

When should you CT head immediately (7)

A

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

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89
Q

When should you CT within 8 hours? (4)

A

Age 65+

History of bleeding or clotting disorder

Dangerous mechanism

> 30 minutes retrograde amnesia

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90
Q

What is the Cushings reflex?

A

HTN

Bradycardia

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91
Q

Symptoms and signs of migraine? What are the criteria?

A
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

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92
Q

Symptoms and signs of Tension headache?

A

Recurrent, non-disabling

Bilateral headache

Tight band

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93
Q

Symptoms and signs of Cluster headache?

A
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
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94
Q

Symptoms and signs of Temporal arteritis?

A
Age 60+
Rapid onset
Unilateral
Jaw claudication
Tender, palpable temporal artery
Raised ESR
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95
Q

Symptoms and signs of Medication over use headache? How to withdraw?

A

15 days + per month
Worsened with regular symptomatic medication
May be psych co-morbidity

Simple anaglesia and triptans - withdraw abruptly
Opioids - withdraw slowly

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96
Q

What are features and management of herpes zoster ophthalmicus?

A

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

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97
Q

What is a Holmes tremor? Management?

A

Lesion in Red nucleus

Irregular low frequency tremor

Resting, postural and action tremor

Treatment:
Levodopa
Neuroleptic agents
Radiation

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98
Q

What is holmes-adie pupil? What is it associated with?

A

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

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99
Q

Horners Syndrome features?

How are these separated by location and what are the causes?

A

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
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100
Q

What is hereditary sensorimotor neuropathy? How many types? What is type 1 vs type 2 pathology?

A

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
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101
Q

What are features of huntington’s disease?

A

CAG repeat expansion

Chorea
Personality changes
Intellectual impairment
Dystonia
Saccadic eye movements

Develop from age 35

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102
Q

Features of idiopathic cranial hypertension? Management?

A
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
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103
Q

What is inclusion body myositis?

A

Cause of myopathy
Cytoplasmic inclusion on muscle biopsy

Proximal and distal muscles

Quads and finger/wrist flexors > extensors

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104
Q

Features of intracranial thrombosis? Name three places it can occur

A

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

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105
Q

Cancer associations of lambert-eaton myasthenic syndrome? What antibody?

A

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

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106
Q

What is the other name for lateral medullary syndrome? Which artery? What features?

A

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
Q

What is maternally inherited diabetes and deafness (MIDD)?

A

Mitochondrial disease

Snesorineural hearing loss
Diabetes
Stroke like sx
Retinal dystrophy
Proximal myopathy
ESRF
Cardiomyopathy
108
Q

Patterns of damage to median nerve?
At wrist
At elbow
Anterior interosseous nerve

A

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
Q

Features of menieres disease? Management?

A

Recurrent vertigo, tinnitus, hearing loss
Sensation of aural fullness
Minutes to hours

Cease driving until sx resolve

Acute: Prochloperazine
Prevention:
Betahistine

110
Q

Management of migraines?

What specifically for women with predictable menstrual migraine?

What for pregnancy?

A

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
Q

Features of Kearns-Sayre syndrome?

A

Age <20
External ophthalmoplegia
Retinitis Pigmentosa
Ptosis

112
Q

Features of MELAS?

A

Mitochondrial encephalomyopathy lactic acidosis and stroke like episodes

113
Q

Features of MERRF

A

Myoclonus epilepsy with ragged red fibres

114
Q

Features and management of motor neuron disease

A

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
Q

What is multifocal motor neuropathy? What antibodies?

What does MMN-CB respond well to?

A

Acquired autoimmune demyelinating motor neuropathy

Motor conduction block

Slowly progressive, distal motor neuropathy

Anti-GM1 antibodies

MMN-CB responds well to IVIG

116
Q

Diagnostic criteria of multiple sclerosis?

What is Uhthoff’s phenomenon?

What is Lhermitter’s syndrome?

Investigation?

A

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
Q

Management of multiple sclerosis?

Acute relapse

Disease modifying drugs

Symptom control
Fatigue
Spasticity
Bladder
Oscillopsia
A

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
Q

Two types of multiple system atrophy? Symptoms?

A

MSA - P (parkinsonian predominant)

MSA - C (cerebellar predominant)

Parkinsonism
Autonomic disturbance
Cerebellar signs

119
Q

Myasthenia Gravis antibodies, investigation and management?

A

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
Q

Exacerbating factors of myasthenia gravis? (6)

A
penicillamine
quinidine, procainamide
beta-blockers
lithium
phenytoin
antibiotics: gentamicin, macrolides, quinolones, tetracyclines
121
Q

Features of myotonic dystrophy? Types?

A

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
Q

Neck lump features:

Pharyngeal pouch

A

Older men

Posteromedial herniation

If large, midline lump that gurgles on palpation

Dysphagia
Regurgitation
Aspiration
Chronic cough

123
Q

Neck lump features:

Cystic Hygroma

A

Congenital lymphatic lesion

Neck (left)

Evident at birth

124
Q

Neck lump features:

Branchial Cyst

A

Oval, mobile cystic mass

Between SCM and pharynx

Failure of obliteration of second branchial cleft

Early adulthood

125
Q

Nerve conduction studies. Conduction velocity? Amplitude?

Axonal

Demyelinating

A

Axonal
CV - normal
Amp - reduced

Demyelinating
CV - reduced
Amp - normal

126
Q

Neurofibromatosis features. Type 1 vs Type 2

A

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
Q

Features and management of neuroleptic malignant syndrome?

A

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
Q

Features, antibodies of neuromyelitis optica? aka?

Management?

A

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
Q

Management of neuropathic pain?

A
  1. Duoloxetine, amitriptyline, duloxetine, gabapentin, pregabalin

–> if does not work, try another

  1. Tramadol for rescue therapy

NOTE: Topical capsaicin for localised neuropathic pain

NOTE: Amitriptyline is contraindicated in arrhythmias and post-MI

130
Q

Features and management of optic neuritis?

A
Unilateral decrease in visual acuity
Red desaturation 
Pain on eye movement
RAPD
Central scotoma

Management:
High dose steroids
Recovery 4-6 weeks

131
Q

Anti-Hu

Associated cancer and symptoms

A

Small cell lung cancer and neuroblastoma

Painful sensory neuropathy
Cerebellar syndromes
Encephalitis

132
Q

Anti-Yo

Associated cancer and symptoms

A

Ovarian and breast cancer

Cerebellar syndrome

133
Q

Anti-Ri

Associated cancer and symptoms

A

Small cell lung cancer

Retinal degeneration
Ocular opsoclonus-myoclonus

134
Q

Anti-GAD

Associated cancer and symptoms

A

Breast, colorectal and small cell lung cancer

Stiff person’s syndrome
Diffuse hypertonia

135
Q

Purkinje Cell Antibody

A

Breast cancer

Peripheral neuropathy

136
Q

Triad of parkinson’s disease?

How is it different to drug induced parkinsonism?

Investigation if difficult to differentiate between essential tremor and parkinsons?

A

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
Q

What medicaiton should you give to parkinsons disease if affecting QoL?

A

Levodopa

138
Q

What should you do if symptoms with parkinsons despite levodopa?

A

Add one of:

Dopamine Agonist
MAO-B inhibitor
COMT inhibitor

139
Q

Which drugs can cause impulse disorder? What is it more common with?

A

Any dopaminergic therapy.

More common with:
Dopamine agonist therapy
Previous history of impulsive behaviours
Alcohol

140
Q

If develop excessive sleepiness in day with parkinsons. What can you consider?

A

Modafinil

141
Q

If develops orthostatic hypotension in parkinsons, what can you consider?

A

Midodrine

142
Q

Examples of dopamine receptor agonists? (4)

A

Bromocriptine
Ropinirole
Cabergoline
Apomorphine

143
Q

Examples of monoamine Oxidase B inhibitors?

A

Selegiline

144
Q

Examples of COMT inhibitors?

A

Entacapone

Tolcapone

145
Q

Examples of antimuscarinics?

A

Procyclidine
Benzotropine
Trihexyphenidyl

146
Q

Examples of demyelinating pathology?

A
GBS
CIDP
Amiodarone
HSMN 1
Paraprotein neuropathy
147
Q

Examples of axonal pathology?

A
Alcohol
Diabetes mellitus
Vasculitis
Vitamin B12 deficiency
HSMN 2
148
Q

Schizotypal vs Schizoid

A

Schizotypal

  • idea of reference
  • odd beliefs and magical thinking
  • paranoid ideation
  • inappropriate affect

Schizoid

  • indifference
  • solitary
  • lack of interest
  • emotional coldness
  • few interests
149
Q

Features of pituitary apoplexy?

A
Sudden onset headache
Neck stiffness
Bitemporal superior quadrantic defect
Extraocular nerve palsy
Pituitary insufficiency
150
Q

What is POEMS?

A
Polyneuropathy
Organomegaly
Endocrinopathy
M-protein band from a plasmacytoma
Skin pigmentation
151
Q

Features of post-lumbar puncture headache? Risk factors?

Management?

A

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
Q

Features and Management of primary open angle glaucoma?

A

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
Q

Features of progressive supranuclear palsy?

Alternative name?

A

Steele-Richardson-Olszewski syndrome

Impaired vertical gaze (down gaze worse tha up)
Parkinsonism (bilateral)
Falls
Cognitive impairment

154
Q

What suggests pseudoseizures?

A
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
Q

Associations with restless leg syndrome?

Management

A

Iron deficiency anaemia
Uraemia
DM
Pregnancy

Management:
Walking stretching, massage
Rx Iron deficiency
Dopamine Agonist (ropinirole, pramipexole)
Benzodiazepines
Gabapentin
156
Q

Features of retinitis pigmentosa?

Asssociated diseases?

A
  • 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
Q

Features of Reye’s Syndrome? Management?

A

Preceding viral illness
Encephalopathy (confusion, seizure, cerebral oedema, coma)
Fatty infiltration of liver, kidney and pancreas
Hypoglycaemia

Supportive management

158
Q

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?

A

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
Q

Spinal cord lesions. Combined motor and sensory lesions. Features and tracts affected of:

Brown-Sequard

A
  1. Lateral corticospinal tract
  2. Dorsal columns
  3. Lateral spinothalamic tract
  4. Ipsilateral spastic paresis below lesion
  5. Ipsilateral loss of proprioception and vibration sensation
  6. Contralateral loss of pain and temperature sensation
160
Q

Spinal cord lesions. Combined motor and sensory lesions. Features and tracts affected of:

Subacute combined degeneration

(Vitamin B12 Deficiency)

A
  1. Lateral corticospinal tracts
  2. Dorsal columns
  3. Spinocerebellar tracts
  4. Bilateral spastic paresis
  5. Bilateral loss of proprioception and vibration sensation
  6. Bilateral limb ataxia
161
Q

Spinal cord lesions. Combined motor and sensory lesions. Features and tracts affected of:

Friedrich’s ataxia

A
  1. Lateral corticospinal tracts
  2. Dorsal columns
  3. Spinocerebellar tracts
  4. Bilateral spastic paresis
  5. Bilateral loss of proprioception and vibration sensation
  6. Bilateral limb ataxia
  7. Cerebellar ataxia
162
Q

Spinal cord lesions. Combined motor and sensory lesions. Features and tracts affected of:

Anterior spinal artery occlusion

A
  1. Lateral corticospinal tracts
  2. Lateral spinothalamic tracts
  3. Bilateral spastic paresis
  4. Bilateral loss of pain and temperature sensation
163
Q

Spinal cord lesions. Combined motor and sensory lesions. Features and tracts affected of:

Syringomyelia

A
  1. Ventral horns
  2. Lateral spinothalamic tract
  3. Flacid paresis (typically affecting the intrinsic hand muscles)
  4. Loss of pain and temperature sensation
164
Q

Spinal cord lesions. Sensory lesion. Features and tracts affected of:

Neurosyphilis (tabes dorsalis)

A
  1. Dorsal columns

1. Loss of proprioception and vibration sensation

165
Q

What is a spontaneous intracranial hypotension?

How is it managed?

How is it investigated?

A

Result of CSF leak.

Headache worse when upright

Investigation:
MRI with gadolinium - patchy meningeal enhancement

Management:
Conservative
Epidural blood patch

166
Q

Stroke by anatomy?

Anterior cerebral artery

A

Contralateral hemiparesis and sensory loss

Lower extremity > upper

167
Q

Stroke by anatomy?

Middle cerebral artery

A

Contralateral hemiparesis and sensory loss

Upper extremity > lower

168
Q

Stroke by anatomy?

Posterior cerebral artery

A

Contralateral homonymous hemianopia with macular sparing

Visual agnosia

169
Q

Stroke by anatomy? aka

Posterior cerebral artery (supply midbrain)

A

Weber’s Syndrome

Ipsilateral CN III palsy

Contralateral weakness of upper and lower extremity

170
Q

Stroke by anatomy? ala

Posterior inferior cerebellar artery

A

Lateral Medullary Syndrome
Wallenberg syndrome

Ipsilateral: Facial pain and temperature loss
Ataxia, nystagmus

Contralateral: limb/torso pain and temperature loss.

171
Q

Stroke by anatomy? aka

Anterior inferior cerebellar artery

A

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
Q

Stroke by anatomy?

Retinal/ophthalmic artery

A

Amaurosis Fugax

173
Q

Stroke by anatomy?

Basilar Artery

A

Locked In syndrome

174
Q

What is the ROSIER Score? What is positive score?

A

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
Q

Management of stroke in acute setting?

When should you thrombolyse?

When should you perform thrombectomy?

A

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
Q

Secondary prevention of stroke?

A
  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
Q

What should be assessed in the Oxford Stroke (Bamford) Classification?

A

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
Q

Oxford Stroke Classification.

Partial Anterior Circulation Infarct

A

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
Q

Oxford Stroke Classification.

Total Anterior Circulation Infarct

A

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
Q

Oxford Stroke Classification.

Lacunar

A

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
Q

Oxford Stroke Classification.

Posterior circulation infarcts

A

Involves vertebrobasilar arteries.

1 of:

  1. Cerebellar or brainstem syndromes
  2. LOC
  3. Isolated homonymous hemianopia
182
Q

Investigations for Subarachnoid Haemorrhage?

A
  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
Q

Treatment of subarachnoid haemorrhage?

A

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
Q

Complications of subtotal thyroidectomy?

A

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
Q

Differential for sudden loss of vision?

A

Ischaemic (amaurosis fugax)
Vitreous Haemorrhage
Retinal detachment
Retinal migraine

186
Q

Central retinal vein occlusion

A

Severe retinal haemorrhages seen on fundoscopy

187
Q

Central retinal artery occlusion

A

RAPD, cherry red spot on pale retina

188
Q

Vitreous haemorrhage

A

Sudden visual loss, dark spots (small bleeds - floaters)

189
Q

Retinal detachment

A

Dense shadow that starts peripherally and progresses centrally

Veil or curtain over the field

190
Q

Posterior Vitreous Detachment

A

Flashes of light - peripheral field

Floaters - temporal side of central

191
Q

How does superficial siderosis present? What is it?

A
Sensorineural hearing loss
Ataxia
Dementia
Anosmia
Anisocoria

It is the deposition of iron in neurons of the CNS

192
Q

Classical presentation of Syringomyelia?

How should this be investigated?

What is it?

A

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
Q

Differential for thunderclap headache?

A
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
Q

Treatment of Tics?

A

Clonidine

Atypical antipsychotics

195
Q

What is transient global amnesia

A

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
Q

What is a Transient Ischaemic Attack?

A

a transient episode of neurologic dysfunction caused by focal brain, spinal cord, or retinal ischaemia, without acute infarction.

197
Q

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?

A

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
Q

Differentials of transverse myelitis?

A

Viral (VZV, HSV< CMV, EBV, Flu, HIV)
Bacterial (Syphilis, Lyme)
Post Infectious
First Sx of MS or NMO

199
Q

List differentials for trigeminal autonomic cephalalgias?

A

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
Q

Features of tuberous sclerosis?

A

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
Q

Somatisation disorder

A

Multiple symtoms for at least 2 years

Refuses to accept reassurance or negative results

202
Q

Hypochonrial disorder

A

Persistent belief in underlying serious disease i.e. cancer

Patient refuses to accept reassurance or negative results

203
Q

Conversion disorder

A

Loss of motor or sensory function. Patients indifferent to apparent disorder

Doesn’t consciously fein (factitious disorder) or seek material gain (malingering)

204
Q

Dissociative disorder

A

Separate off certain memories from normal consviousness

205
Q

Factitious disorder

A

Munchausen’s syndrome

Intential production of physical or psychological sx

206
Q

Malingering

A

Fraudulent simulation or exaggeration of sx with intetion for financial or other gain

207
Q

Viral labyrinthitis?

A

Recent viral infection
Sudden onset
N&V
Hearing may be affected

208
Q

Viral neuronitis?

A

Recent viral infection
Recurrent vertigo lasting hours or days
No hearing loss
Horizontal nystagmus

Rx: Prochlorperazine

209
Q

Vestibular Schwannoma presentation?

A

cranial nerve VIII: vertigo, unilateral sensorineural hearing loss, unilateral tinnitus

cranial nerve V: absent corneal reflex

cranial nerve VII: facial palsy

210
Q

What is important about Vigabatrin?

A

40% of patients develop visual field defects, which may be irreversible
visual fields should be checked every 6 months

211
Q

Features of von Hippel-Lindau Syndrome?

A

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
Q

What are the two major side effects of Nataluzumab?

A

Hepatotoxicity

Progressive multifocal leukoencephalopathy (caused by the JC Virus)

213
Q

How is multiple sclerosis diagnosed? Who’s criteria?

A

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
Q

What does lymphangioleiomyomatosis look like on CT? What is it associated with?

A

CT - small irregular lung cysts (innumerable) diffusely distributed

Associated with tuberous sclerosis

215
Q

What is Progressive Multifocal Leukoencephalopathy? (PML)

What is it caused by?

What does it look like on MRI?

A

Demyelinating disease of CNS

Widespread lesions

JC Virus

Seen in immunosuppression

216
Q

Which drug can cause extrapyramidal effects in children and young adults?

How can you tell it is drug induced?

A

Metoclopramide

Often present with bilateral symptoms

217
Q

How does corticobasal degeneration present?

A

Higher order dysfunction
(apraxia, aphasia)

Alien hand syndrome

218
Q

What should upgoing plantars and absent ankle jerks make you think of? (5)

A
Subacute combined degeneration of the cord
Motor neurone disease
Friedrich's ataxia
Tabes Dorsalis
Dual peripheral with central pathology
219
Q

What is SUNCT and can you name some features?

A

Age >40
Any time of day
Last seconds to minutes
Up to 75x per day

220
Q

Features of Paroxysmal Hemicrania?

Management?

A

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
Q

Features of Hemicrania Continua?

Management

A

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
Q

What is an acephalgic migraine?

A

Diagnosis of exclusion

Auras without a headache

223
Q

Features and management of idiopathic intracranial hypertension?

A

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
Q

What is posterior reversible leucoencephalopathy syndrome? (PRES) How does it present?

A

Thunderclap headache. Followed by:
Confusion
Seizure
Visual Sx

Normal CT and LP

Common cause:
HTN encephalopathy
Eclampsia

Diagnosis:
Vasogenic brain oedema on MRI

225
Q

Features of low pressure headache on MRI?

A

Meningeal enhancement

Thickening and shallow subdural haematoma

226
Q

What is reversible cerebrovascular vasoconstriction syndrome?

A

Hypertension
Seizures

Arterial beeding on angiography

227
Q

What is management of vertebral artery dissection?

A

Anticoagulation (once bleed excluded)

Prevents further VTE complications

228
Q

Exclusion criteria for stroke thrombolysis?

A

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
Q

What is malignant middle cerebral artery syndrome? How is it managed?

A

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
Q

Imaging studies and their use:

DWI-MRI

FLAIR MRI

CTA and DSA

A

DWI-MRI - acute stroke

FLAIR MRI - chronic ischaemic change

CTA and DSA - significant intravascular stenoses or thrombosis

231
Q

What scan can be used to aid diagnosis of parkinson’s disease?

A

DaT Scan

232
Q

What VTE prophylaxis should be given for stroke patients?

A

Intermittent pneumatic compression for 30 days or until mobile

233
Q

Should you reverse a benzodiazepine overdose?

A

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
Q

What should happen in MS during pregnancy and afterwards?

A

‘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
Q

What is valproate associated hyperammonaemic encephalopathy? Treatment?

A

Non-specific symptoms
Abnormal ammonia
Occur in all levels of dosing

Treat with L-carnitine

236
Q

Are oligoclonal bands in CSF specific for MS?

A

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
Q

Who should be treated after one episode of epilepsy?

A

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
Q

What is SUNA?

A

Short unilateral neuralgiform pain with autonomic symptoms

239
Q

Vague descriptions of CSF in viral, bacterial, GBS and TB

A

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
Q

How should atypical trigeminal neuralgia be investigated? i.e. with sensory loss

A

MRI with gadalonium

241
Q

What is hereditary neuropathy with liability to pressure palsy?

A

Trivial trauma to a peripheral nerve results in mononeuropathy which may take weeks to resolve

Demyelinating neuropathy

Conservative management

242
Q

What is multifocal motor neuropathy?

A

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
Q

What is the causative agent of meningitis from an ear infection?

A

Streptococcus pneumoniae

244
Q

What are the features and causative agent of tropical spastic paraparesis?

A

HTLV1

Paraparesis
Urinary retention

Treatment:
Steroids

245
Q

What is writers cramp?

A

Focal dystonia

Flexion, extension or rotation of the muscles of the hund

246
Q

What is first line investigation for neck mass?

A

USS Neck

247
Q

What is a watershed infarct?

A

Ischaemia occurs in most vulnerable region of brain between two artery territories

248
Q

What are the key featrues of Kearns-Sayer Syndrome? What type of disease is it?

What is seen on muscle biopsy?

A

Progressive external ophthalmoplegia
Pigmentary retinopathy
Cardiac conduction defects

Mitochondrial disease

Muscle biopsy - ragged red fibres

249
Q

What is seen in a left sided ventral pontine syndrome? aka

A

Millard Gubler

Ipsilateral facial weakness

Contralateral body hemiparesis

250
Q

What would you expect to see in CSF for lyme disease?

A

CSF shows a lymphocytic pleocytosis

251
Q

Can you name some drugs which can cause lymphadenopathy?

A

Phenytoin
Carbamazepine
Allopurinol
Sulphonamides

252
Q

What symptoms should you warn patients starting on levetiracetam about?

A

Irritabiliy
Frank aggression

May be best to avoid if history of psychotic symptoms

253
Q

What is the only licensed treatment for parkinsons disease dementia?

A

Rivastigmine

254
Q

How should hyperglycaemia be managed in NG fed patients?

A

Never give PRN actrapid
Never stop NG feed

Aim 6-12 mmol/l

When over 12, use biphasic insulin

255
Q

What is the rule of fours for CN localisation?

A

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
Q

What does MELAS present with? What does it mean? What type of disease is it?

A

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
Q

What is capgras syndrome?

A

Believes someone close is replaced by an imposter

258
Q

What is cotard syndrome?

A

Believe they or part of them is dead

259
Q

What is fregoli syndrome?

A

Believe an individual has taken on may different guises

260
Q

What is MERRF and how does it present?

A

Myoclonic epilepsy with ragged red fibres

Mitochondrial DNA disorder

Ragged red fibres on ibopsy
Optic atrophy
WPW
Myoclonic jerks
Seizures
261
Q

Name three drug induced causes of tics

A

Methylphenidate
Dextroamphetamine
Lamotrigine

262
Q

What is characteristic of McArdle disease?

A

Second Wind

Occurs as change in fatty acid metabolism

263
Q

What is Usher’s syndrome? What are the sympoms?

A

Autosomal recessive

Deaf-Blind