Neurology Flashcards

1
Q

Drug side effects:

Peripheral neuropathy

A
amiodarone
phenytoin
metronidazole
nitrofurantoin
isoniazid
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2
Q

Drug side effects:

thrombocytopenia

A

sodium valproate

heparin

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3
Q

Mechanism of action:

Phenytoin

A

binds to sodium channels increasing their refractory period

P450 inducer

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4
Q

Phenytoin

Adverse effects

Acute

A

initially: dizziness, diplopia, nystagmus, slurred speech, ataxia
later: confusion, seizures

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5
Q

Phenytoin

Adverse effects

Chronic

A

gingival hyperplasia (secondary to increased expression of platelet derived growth factor, PDGF), hirsutism, coarsening of facial features, drowsiness
megaloblastic anaemia (secondary to altered folate metabolism)
peripheral neuropathy
enhanced vitamin D metabolism causing osteomalacia
lymphadenopathy
dyskinesia

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6
Q

Phenytoin

Adverse effects

Idiosyncratic

A
fever
rashes, including severe reactions such as toxic epidermal necrolysis
hepatitis
Dupuytren's contracture*
aplastic anaemia
drug-induced lupus
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7
Q

Phenytoin monitoring

A

Phenytoin levels do not need to be monitored routinely but trough levels, immediately before dose should be checked if:
adjustment of phenytoin dose
suspected toxicity
detection of non-adherence to the prescribed medication

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8
Q

Brain lesions

Gross anatomy

Parietal lobe lesions

A
sensory inattention
apraxias
astereognosis (tactile agnosia)
inferior homonymous quadrantanopia
Gerstmann's syndrome (lesion of dominant parietal): alexia, acalculia, finger agnosia and right-left disorientation
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9
Q

Brain lesions

Gross anatomy

Occipital lobe lesions

A

homonymous hemianopia (with macula sparing)
cortical blindness
visual agnosia

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10
Q

Brain lesions

Gross anatomy

Temporal lobe lesions

A

Wernicke’s aphasia: this area ‘forms’ the speech before ‘sending it’ to Brocas area. Lesions result in word substituion, neologisms but speech remains fluent
superior homonymous quadrantanopia
auditory agnosia
prosopagnosia (difficulty recognising faces)

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11
Q

Brain lesions

Gross anatomy

Frontal lobe lesions

A
expressive (Broca's) aphasia: located on the posterior aspect of the frontal lobe, in the inferior frontal gyrus. Speech is non-fluent, laboured, and halting
disinhibition
perseveration
anosmia
inability to generate a list
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12
Q

Brain lesions

Gross anatomy

Cerebellar lesions

A

midline lesions: gait and truncal ataxia

hemisphere lesions: intention tremor, past pointing, dysdiadokinesis, nystagmus

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13
Q

Brain lesions

Medial thalamus and mammillary bodies of the hypothalamus

A

Wernicke’s and Korsakoff’s

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14
Q

Brain lesions

Subthalamic nucleus of the basal ganglia

A

Hemiballism (coarse, violent, wide amplitude movements, ipsilateral arm and leg).

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15
Q

Brain lesions

Striatum (caudate nucleus) of the basal ganglia

A

Huntington’s Chorea

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16
Q

Brain Lesions

Substantia nigra of the basal ganglia

A

Parkinson’s disease

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17
Q

Brain lesions

Amygdala

A

Kluver-Bucy syndrome (hypersexuality, hyperorality, hyperphagia, visual agnosia

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18
Q

What is Friedreich’s ataxia?

A

Friedreich’s ataxia is the most common of the early-onset hereditary ataxias. It is an autosomal recessive, trinucleotide repeat disorder characterised by a GAA repeat in the X25 gene on chromosome 9 (frataxin). Friedreich’s ataxia is unusual amongst trinucleotide repeat disorders in not demonstrating the phenomenon of anticipation.

The typical age of onset is 10-15 years old. Gait ataxia and kyphoscoliosis are the most common presenting features.

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19
Q

Neurological features of Friedreich’s ataxia

A

absent ankle jerks/extensor plantars
cerebellar ataxia
optic atrophy
spinocerebellar tract degeneration

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20
Q

Other features of Friedreich’s ataxia

A
hypertrophic obstructive cardiomyopathy (90%, most common cause of death)
diabetes mellitus (10-20%)
high-arched palate
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21
Q

uses of carbamazepine

A

focal seizures - epilepsy
trigeminal neuralgia
bipolar disordre

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22
Q

Mechanism of action - carbamazepine

A

binds to sodium channels to increase their refractory period

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23
Q

Side effects of carbamazepine

A
P450 enzyme inducer
dizziness and ataxia
drowsiness
headache
visual disturbances (especially diplopia)
Steven-Johnson syndrome
leucopenia and agranulocytosis
hyponatraemia secondary to syndrome of inappropriate ADH secretion

Carbamazepine is known to exhibit autoinduction, hence when patients start carbamazepine they may see a return of seizures after 3-4 weeks of treatment.

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24
Q

Stroke symptoms

anterior cerebral artery

A

Contralateral hemiparesis and sensory loss, lower extremity > upper

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25
Q

Stroke symptoms

middle cerebral artery

A

Contralateral hemiparesis and sensory loss, upper extremity > lower
Contralateral homonymous hemianopia
Aphasia

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26
Q

Stroke symptoms

posterior cerebral artery

A

Contralateral homonymous hemianopia with macular sparing

Visual agnosia

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27
Q

Stroke symptoms

Weber’s syndrome (branches of the posterior cerebral artery that supply the midbrain)

A

Ipsilateral CN III palsy

Contralateral weakness of upper and lower extremity

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28
Q

Stroke Symptoms

Posterior inferior cerebellar artery (lateral medullary syndrome, Wallenberg syndrome)

A

Ipsilateral: facial pain and temperature loss
Contralateral: limb/torso pain and temperature loss
Ataxia, nystagmus

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29
Q

Stroke Symptoms

Anterior inferior cerebellar artery (lateral pontine syndrome)

A

Symptoms are similar to Wallenberg’s (see above), but:

Ipsilateral: facial paralysis and deafness

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30
Q

Stroke symptoms

retinal/ ophthalmic artery

A

amaurosis fugax

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31
Q

Stroke symptoms

Basilar artery

A

Locked in syndrome

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32
Q

Stroke symptoms

Lacunar infarcts

A

present with either isolated hemiparesis, hemisensory loss or hemiparesis with limb ataxia
strong association with hypertension
common sites include the basal ganglia, thalamus and internal capsule

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33
Q

Tuberous sclerosis

A

Autosomal dominant

Like neurofibromatosis, the majority of features seen in TS are neurocutaneous

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34
Q

Cutaneous features of tuberous sclerosis

A

depigmented ‘ash-leaf’ spots which fluoresce under UV light
roughened patches of skin over lumbar spine (Shagreen patches)
adenoma sebaceum (angiofibromas): butterfly distribution over nose
fibromata beneath nails (subungual fibromata)
café-au-lait spots* may be seen

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35
Q

Neurological features of tuberous sclerosis

A

developmental delay
epilepsy (infantile spasms or partial)
intellectual impairment

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36
Q

Other features of tuberous sclerosis

A

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

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37
Q

Sensory lesions of the spinal cord

Neurosyphilis

A

affects the dorsal columns

loss of vibration and proprioception

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38
Q

Motor lesions of the spinal cord

ALS/MND

A

affects both upper (corticospinal tracts) and lower motor neurons
results in a combination of upper and lower motor neuron signs

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39
Q

Motor lesions of the spinal cord

Polio

A

affects anterior horns resulting in lower motor neuron signs

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40
Q

Combined motor and sensory lesions of the spinal cord
Brown-Sequard syndrome (spinal cord hemisection)

Which tracts

A
  1. Lateral corticospinal tract
  2. Dorsal columns
  3. Lateral spinothalamic tract
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41
Q

Combined motor and sensory lesions of the spinal cord
Brown-Sequard syndrome (spinal cord hemisection)

Clinical notes

A
  1. Ipsilateral spastic paresis below lesion
  2. Ipsilateral loss of proprioception and vibration sensation
  3. Contralateral loss of pain and temperature sensation
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42
Q

Combined motor and sensory lesions of the spinal cord
Subacute combined degeneration of the spinal cord (vitamin B12 & E deficiency)
Which tracts

A
  1. Lateral corticospinal tracts
  2. Dorsal columns
  3. Spinocerebellar tracts
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43
Q

Combined motor and sensory lesions of the spinal cord
Subacute combined degeneration of the spinal cord (vitamin B12 & E deficiency)

Clinical notes

A
  1. Bilateral spastic paresis
  2. Bilateral loss of proprioception and vibration sensation
  3. Bilateral limb ataxia
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44
Q

Combined motor and sensory lesions of the spinal cord

Friedreichs ataxia
Which tracts

A
  1. Lateral corticospinal tracts
  2. Dorsal columns
  3. Spinocerebellar tracts
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45
Q

Combined motor and sensory lesions of the spinal cord

Friedreichs ataxia

Clinical notes

A
  1. Bilateral spastic paresis
  2. Bilateral loss of proprioception and vibration sensation
  3. Bilateral limb ataxia
    In addition cerebellar ataxia → other features e.g. intention tremor
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46
Q

Combined motor and sensory lesions of the spinal cord
Anterior spinal artery occlusion

which tracts

A
  1. Lateral corticospinal tracts

2. Lateral spinothalamic tracts

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47
Q

Combined motor and sensory lesions of the spinal cord

Anterior spinal artery occlusion

Clinical notes

A
  1. Bilateral spastic paresis

2. Bilateral loss of pain and temperature sensation

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48
Q

Combined motor and sensory lesions of the spinal cord

Syringomelia

Which tracts

A
  1. Ventral horns

2. Lateral spinothalamic tract

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49
Q

Combined motor and sensory lesions of the spinal cord

Syringomelia

Clinical notes

A
  1. Flacid paresis (typically affecting the intrinsic hand muscles)
  2. Loss of pain and temperature sensation
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50
Q

Combined motor and sensory lesions of the spinal tract

MS

Which tracts

A

Asymmetrical, varying spinal tracts involved

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51
Q

Combined motor and sensory lesions of the spinal tract

MS

Clinical notes

A

Combination of motor, sensory and ataxia symptoms

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52
Q

Motor and descending tracts

pyrimadal tracts

A

Lateral corticospinal tracts

anterior corticospinal tracts

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53
Q

Motor and descending tracts

Extrapyrimidal tracts

A

rubrospinal tracts
reticulospinal tracts
olivospinal tracts
vestibular spinal tracts

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54
Q

Sensory and ascending tracts

Dorsal column medial lemniscus system

A

Grascile fasciculus

Cuneate fasciculus

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55
Q

Sensory and ascending tracts

Spinocerebellar tracts

A

posterior spinocerebellar tracts

anterior spinocerebellar tracts

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56
Q

Sensory and ascending tracts

Anterolateral tracts

A

anterior spinothalamic tracts

lateral spinothalamic tracts

spino-oliviary tract

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57
Q

Bitemporal hemianopia

A

lesion of optic chiasm
upper quadrant defect > lower quadrant defect = inferior chiasmal compression, commonly a pituitary tumour
lower quadrant defect > upper quadrant defect = superior chiasmal compression, commonly a craniopharyngioma

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58
Q

Homonymous quadrantanopias

A

superior: lesion of the inferior optic radiations in the temporal lobe (Meyer’s loop)
inferior: lesion of the superior optic radiations in the parietal lobe
mnemonic = PITS (Parietal-Inferior, Temporal-Superior)

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59
Q

homonymous hemianopia

A

incongruous defects: lesion of optic tract
congruous defects: lesion of optic radiation or occipital cortex
macula sparing: lesion of occipital cortex

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60
Q

what are congruent visual defects

A

A congruous defect simply means complete or symmetrical visual field loss and conversely an incongruous defect is incomplete or asymmetric.

incongruous defects = optic tract lesion;

congruous defects = optic radiation lesion or occipital cortex

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61
Q

Key features of myasthenia gravis

A

The key feature is muscle fatigability - muscles become progressively weaker during periods of activity and slowly improve after periods of rest:
extraocular muscle weakness: diplopia
proximal muscle weakness: face, neck, limb girdle
ptosis
dysphagia

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62
Q

Associations with myasthenia gravis

A

thymomas in 15%
autoimmune disorders: pernicious anaemia, autoimmune thyroid disorders, rheumatoid, SLE
thymic hyperplasia in 50-70%

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63
Q

Investigations of myasthenia gravis

A

single fibre electromyography: high sensitivity (92-100%)
CT thorax to exclude thymoma
CK normal
autoantibodies: around 85-90% of patients have antibodies to acetylcholine receptors. In the remaining patients, about about 40% are positive for anti-muscle-specific tyrosine kinase antibodies
Tensilon test: IV edrophonium reduces muscle weakness temporarily - not commonly used any more due to the risk of cardiac arrhythmia

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64
Q

Management of myasthenia gravis

A

long-acting acetylcholinesterase inhibitors
pyridostigmine is first-line
immunosuppression:
prednisolone initially
azathioprine, cyclosporine, mycophenolate mofetil may also be used
thymectomy

Management of myasthenic crisis
plasmapheresis
intravenous immunoglobulins

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65
Q

Pharmacology of triptans

A

Triptans are specific 5-HT1B and 5-HT1D agonists used in the acute treatment of migraine. They are generally used first-line in combination therapy with an NSAID or paracetamol.

_ take after the onset of headache, not aura

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66
Q

adverse effects of triptans

A

‘triptan sensations’ - tingling, heat, tightness (e.g. throat and chest), heaviness, pressure

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67
Q

triptan contraindications

A

patients with a history of, or significant risk factors for, ischaemic heart disease or cerebrovascular disease

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68
Q

What is restless leg syndrome

A

Restless legs syndrome (RLS) is a syndrome of spontaneous, continuous lower limb movements that may be associated with paraesthesia. It is extremely common, affecting between 2-10% of the general population. Males and females are equally affected and a family history may be present

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69
Q

Features of restless leg syndrome

A

uncontrollable urge to move legs (akathisia). Symptoms initially occur at night but as condition progresses may occur during the day. Symptoms are worse at rest
paraesthesias e.g. ‘crawling’ or ‘throbbing’ sensations
movements during sleep may be noted by the partner - periodic limb movements of sleeps (PLMS)

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70
Q

Causes and associations of restless leg syndrome

A
there is a positive family history in 50% of patients with idiopathic RLS
iron deficiency anaemia
uraemia
diabetes mellitus
pregnancy
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71
Q

Management of restless leg syndrome

A
there is a positive family history in 50% of patients with idiopathic RLS
iron deficiency anaemia
uraemia
diabetes mellitus
pregnancy
72
Q

Features of von-Hippel Lindau

A

cerebellar haemangiomas: these can cause subarachnoid haemorrhages
retinal haemangiomas: vitreous haemorrhage
renal cysts (premalignant)
phaeochromocytoma
extra-renal cysts: epididymal, pancreatic, hepatic
endolymphatic sac tumours
clear-cell renal cell carcinoma

73
Q

Vestibular neuritis

A

a 30-year-old woman who is recovering from an upper respiratory tract infections presents with recurrent attacks of vertigo associated with nausea and vomiting. There is no hearing loss or tinnitus

74
Q

Viral labyrinthitis

e.g. Hx

A

a 30-year-old woman who has recently developed an upper respiratory tract infection presents with vertigo and vomiting. Her hearing is also affected. The symptoms came on suddenly this morning

75
Q

Viral labyrinthitis

A

Recent viral infection
Sudden onset
Nausea and vomiting
Hearing may be affected

76
Q

Vestibular neuritis

A

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

77
Q

Acoustic neuroma

A

Hearing loss, vertigo, tinnitus
Absent corneal reflex is important sign
Associated with neurofibromatosis type 2

78
Q

Vertebrobasilar ischaemia

A

Elderly patient

Dizziness on extension of neck

79
Q

Meniere’s disease

A

Associated with hearing loss, tinnitus and sensation of fullness or pressure in one or both ears

80
Q

Benign paroxysmal positional vertigo

A

Gradual onset
Triggered by change in head position
Each episode lasts 10-20 seconds

81
Q

becker muscular dystrophy

A

develops after the age of 10 years
intellectual impairment much less common
non-frameshift insertion in the dystrophin gene resulting in both binding sites being preserved leading to a milder form

82
Q

Duchene muscular dystrophy

A

progressive proximal muscle weakness from 5 years
calf pseudohypertrophy
Gower’s sign: child uses arms to stand up from a squatted position
30% of patients have intellectual impairment
there is a frameshift mutation resulting in one or both of the binding sites are lost leading to a severe form

83
Q

Dystrophinopathies

A

X-linked recessive
due to mutation in the gene encoding dystrophin, dystrophin gene on Xp21
dystrophin is part of a large membrane associated protein in muscle which connects the muscle membrane to actin, part of the muscle cytoskeleto

84
Q

Stereotypical history of Becker’s dystrophy

A

onset > 10 years old, pseudohypertrophy of calf muscles, trouble standing from sitting, no learning disability

85
Q

Presenting features of Freidreich’s ataxia

A

typical age of onset is 10-15 years old. Gait ataxia and kyphoscoliosis are the most common presenting features.

86
Q

History of posterior cerebral artery stroke eye symptoms

A

contralateral homonymous hemianopia with macular sparing and visual agnosia

87
Q

History of middle cerebral artery stroke

A

contralateral hemiparesis and sensory loss with the upper extremity being more affected than the lower, contralateral homonymous hemianopia and aphasia

88
Q

Von Hipple lindau chromosome

A

Chromosome 3

89
Q

Typical history of MS

A

conjugate horizonal gaze palsy, nystagmus in abducting eye

lethargy, optic neuritis, paraesthesia, spastic weakness

90
Q

Examples of 5-HT3 antagonists

A

ondansetron

granisetron

91
Q

What are 5-HT3 antagonists

A

5-HT3 antagonists are antiemetics used mainly in the management of chemotherapy-related nausea. They mainly act in the chemoreceptor trigger zone area of the medulla oblongata.

92
Q

Side effects of 5-HT3 antagonists

A

Constipation

QT prolongation

93
Q

Non-neurological associations with Friedreich’s ataxia

A
hypertrophic obstructive cardiomyopathy (90%, most common cause of death)
diabetes mellitus (10-20%)
high-arched palate
94
Q

Kluver-Bucy syndrome: stereotypical history

A

a man who has recently been treated for herpes simplex encephalitis presents with a sudden increase in appetite and sexual desire

95
Q

Edrophonium

A

readily reversible acetylcholinesterase inhibitor. It prevents breakdown of the neurotransmitter acetylcholine and acts by competitively inhibiting the enzyme acetylcholinesterase, mainly at the neuromuscular junction

96
Q

What is ataxic telangiectasia

A

Ataxia telangiectasia is an autosomal recessive disorder caused by a defect in the ATM gene which encodes for DNA repair enzymes. It is one of the inherited combined immunodeficiency disorders. It typically presents in early childhood with abnormal movements.

97
Q

Features of ataxic telangiectasia

A

cerebellar ataxia
telangiectasia (spider angiomas)
IgA deficiency resulting in recurrent chest infections
10% risk of developing malignancy, lymphoma or leukaemia, but also non-lymphoid tumours

98
Q

Stereotypical history of Branchial cyst

A

a 20-year-old presents with a painless swelling on the lateral aspect of the neck. On examination it is smooth, non-tender and fluctuant

99
Q

What is a branchial cyst

A

An oval, mobile cystic mass that develops between the sternocleidomastoid muscle and the pharynx
Develop due to failure of obliteration of the second branchial cleft in embryonic development
Usually present in early adulthood

100
Q

What is a cervical rib

A

More common in adult females

Around 10% develop thoracic outlet syndrome

101
Q

Cystic hygroma

A

A congenital lymphatic lesion (lymphangioma) typically found in the neck, classically on the left side
Most are evident at birth, around 90% present before 2 years of age

102
Q

Pharyngeal pouch

A

More common in older men
Represents a posteromedial herniation between thyropharyngeus and cricopharyngeus muscles
Usually not seen but if large then a midline lump in the neck that gurgles on palpation
Typical symptoms are dysphagia, regurgitation, aspiration and chronic cough

103
Q

Thyroglossal cyst

A

More common in patients < 20 years old
Usually midline, between the isthmus of the thyroid and the hyoid bone
Moves upwards with protrusion of the tongue
May be painful if infected

104
Q

Idiosyncratic side effects of phenytoin

A
fever
rashes, including severe reactions such as toxic epidermal necrolysis
hepatitis
Dupuytren's contracture*
aplastic anaemia
drug-induced lupus
105
Q

Acute side effects of phenytoin

A

initially: dizziness, diplopia, nystagmus, slurred speech, ataxia
later: confusion, seizures

106
Q

Chronic side effects of phenytoin

A

common: gingival hyperplasia (secondary to increased expression of platelet derived growth factor, PDGF), hirsutism, coarsening of facial features, drowsiness
megaloblastic anaemia (secondary to altered folate metabolism)
peripheral neuropathy
enhanced vitamin D metabolism causing osteomalacia
lymphadenopathy
dyskinesia

107
Q

Ethosuximide

A

Ethosuximide is an antiepileptic that is particularly indicated in patients with absence seizures
blocks T-type calcium channels in thalamic neurons

108
Q

Riluzole- used in management of Amyotrophic lateral scelosis

A

prevents stimulation of glutamate receptors
used mainly in amyotrophic lateral sclerosis
prolongs life by about 3 months
BIPAP at night - prolongs life by approx 7 months

109
Q

Side effects of lamotrigine

A

Stevens-Johnston syndrome

110
Q

Stereotypical history of mytonic dystrophy

A

a 25-year-old man with muscle weakness is reviewed in clinic. On shaking his hand he has difficultly loosening his grip. On examination he has frontal balding, bilateral ptosis and cataracts

111
Q

What is myotonic dystrophy

A

Myotonic dystrophy (also called dystrophia myotonica) is an inherited myopathy with features developing at around 20-30 years old. It affects skeletal, cardiac and smooth muscle. There are two main types of myotonic dystrophy, DM1 and DM2.

112
Q

Genetics of myotonic dystrophy

A

autosomal dominant
a trinucleotide repeat disorder
DM1 is caused by a CTG repeat at the end of the DMPK (Dystrophia Myotonica-Protein Kinase) gene on chromosome 19
DM2 is caused by a repeat expansion of the ZNF9 gene on chromosome 3

113
Q

Myotonic dystrophy DM 2

A
  • ZNF9 gene on chromosome 3
  • Proximal weakness more prominent
  • Severe congenital form not seen
114
Q

Myotonic dystrophy DM1

A

DM1 DM2

  • DMPK gene on chromosome 19
  • Distal weakness more prominent
115
Q

General features of myotonic dystrophy

A
myotonic facies (long, 'haggard' appearance)
frontal balding
bilateral ptosis
cataracts
dysarthria
116
Q

Other features of myotonic dystrophy

A

myotonia (tonic spasm of muscle)
weakness of arms and legs (distal initially)
mild mental impairment
diabetes mellitus
testicular atrophy
cardiac involvement: heart block, cardiomyopathy
dysphagia

117
Q

Treatment of SAH

A
  • coil aneurysms
  • strict bed rest and BP control
  • Vasospasm is prevented using a 21-day course of nimodipine (a calcium channel inhibitor targeting the brain vasculature) and treated with hypervolaemia, induced-hypertension and haemodilution**
118
Q

Complications of SAH

A

Re-bleeding
Vasospasm (also termed delayed cerebral ischaemia), typically 7-14 days after onset
Hyponatraemia (most typically due to syndrome inappropriate anti-diuretic hormone (SIADH))
Seizures
Hydrocephalus
Death

119
Q

Chromosome for neurofibromatosis type 1

A

Chromosome 17

120
Q

Chromosome for neurofibromatosis type 2

A

Chromosome 22

121
Q

Conductive hearing loss

A

Rinne’s test Bone conduction > air conduction in affected ear
Air conduction > bone conduction in unaffected ear Webber’s- Lateralises to affected ear

122
Q

Sensorineural hearing loss

A

Rinne’s test- Air conduction > bone conduction bilaterally

Webber’s test - Lateralises to unaffected ear

123
Q

Stereotypical history of cavernous sinus thrombus

A

a young man presents with an acute headache associated with unilateral periorbital edema. On examination there is a lateral gaze palsy

124
Q

Features of cavernous sinus thrombus

A

headache (may be sudden onset)
nausea & vomiting,
cavernous sinus syndrome: local infection (e.g. sinusitis), neoplasia, trauma
periorbital oedema
ophthalmoplegia: 6th nerve damage typically occurs before 3rd & 4th
trigeminal nerve involvement may lead to hyperaesthesia of upper face and eye pain
central retinal vein thrombosis

125
Q

Features of sagittal sinus thrombus

A

headache, vomiting
seizures and hemiplegia
parasagittal biparietal or bifrontal haemorrhagic infarctions are sometimes seen

126
Q

lateral sinus thrombus features

A

6th and 7th cranial nerve palsies

127
Q

benzhexol

A

antiparkinsonian agent of the antimuscarinic class

128
Q

Stereotypical history of syringomelia

A

a 30-year-old man presents with progressive weakness of his hands. On examination you notice wasting of the small muscles of the hand. Also pain and temperature sensation is reduced but light touch is preserved.

129
Q

What is syringomelia

A

a collection of cerebrospinal fluid within the spinal cord.

130
Q

Syringobulbia

A

similar phenomenon to syringomyelia which there is a fluid-filled cavity within the medulla of the brainstem. This is often an extension of the syringomyelia but in rare cases can be an isolated finding.

131
Q

Presentation of syringomelia

A

‘cape-like’ (neck and arms) loss of sensation to temperature but preservation of light touch, proprioception and vibration. spastic weakness (predominantly of the upper limbs), paraesthesia, neuropathic pain, upgoing plantars and bowel and bladder dysfunction. Scoliosis in long term

132
Q

selegiline

A

MOAB used to treat parkinsons

133
Q

Stereotypical history of normal pressure hydrocephalus

A

an elderly man presents with gradual worsening dementia and bradyphrenia associated with a slow, shuffling gait. He has also recently suffered with urinary incontinence

134
Q

Stereotypical history of multisystem atrophy

A

an elderly man presents with parkinsonism atonic bladder, postural hypotension and cerebellar signs

135
Q

Stereotypical history of conduction aphasia

A

a patient is noted to speak fluently but have difficulty repeating phrases. He is aware of the errors he is making and comprehension remains normal

136
Q

Stereotypical history of Wernicke’s aphasia

A

a patient is noted to speak fluently but makes little sense with mulitple word substitutions and neologisms. He also has difficulty understanding what people are saying to him

137
Q

Features of parietal lobe lesions

A
sensory inattention
apraxias
astereognosis (tactile agnosia)
inferior homonymous quadrantanopia
Gerstmann's syndrome (lesion of dominant parietal): alexia, acalculia, finger agnosia and right-left disorientation
138
Q

stereotypical history of posterior inferior cerebellar artery stroke

A

sudden onset vertigo and vomiting, dysphagia, ipsilateral facial pain and temperature loss, contralateral limb pain and temperature loss and ataxia

139
Q

Stereotypical history of anterior inferior cerebellar artery stroke

A

sudden onset vertigo and vomiting, ipsilateral facial paralysis and deafness

140
Q

Features of neuroleptic malignant syndrome

A
pyrexia
muscle rigidity
autonomic lability: typical features include hypertension, tachycardia and tachypnoea
agitated delirium with confusion
AKI secondary to raised CK
141
Q

Management of neuroleptic malignant syndrome

A
stop antipsychotic
patients should be transferred to a medical ward if they are on a psychiatric ward and often they are nursed in intensive care units
IV fluids to prevent renal failure
dantrolene 
bromocriptine
142
Q

Features of Meniere’s disease

A

recurrent episodes of vertigo, tinnitus and hearing loss (sensorineural). Vertigo is usually the prominent symptom
a sensation of aural fullness or pressure is now recognised as being common
other features include nystagmus and a positive Romberg test
episodes last minutes to hours
typically symptoms are unilateral but bilateral symptoms may develop after a number of years

143
Q

Natural history of Meniere’s disease

A

symptoms resolve in the majority of patients after 5-10 years
the majority of patients will be left with a degree of hearing loss
psychological distress is common

144
Q

Investigation of Meniere’s disease

A

ENT assessment is required to confirm the diagnosis
patients should inform the DVLA. The current advice is to cease driving until satisfactory control of symptoms is achieved
acute attacks: buccal or intramuscular prochlorperazine. Admission is sometimes required
prevention: betahistine and vestibular rehabilitation exercises may be of benefit

145
Q

Features of CJD

A

dementia (rapid onset)

myoclonus

146
Q

Investigations of CJD

A

CSF is usually normal
EEG: biphasic, high amplitude sharp waves (only in sporadic CJD)
MRI: hyperintense signals in the basal ganglia and thalamus

147
Q

Sporadic CJD

A

accounts for 85% of cases
10-15% of cases are familial
mean age of onset is 65 years

148
Q

New variant CJD

A

younger patients (average age of onset = 25 years)
psychological symptoms such as anxiety, withdrawal and dysphonia are the most common presenting features
the ‘prion protein’ is encoded on chromosome 20 - it’s role is not yet understood
methionine homozygosity at codon 129 of the prion protein is a risk factor for developing CJD - all patients who have so far died have had this
median survival = 13 months

149
Q

Reye’s syndrome

A

Reye’s syndrome is a severe, progressive encephalopathy affecting children that is accompanied by fatty infiltration of the liver, kidneys and pancreas. The aetiology of Reye’s syndrome is not fully understood although there is a known association with aspirin use and a viral cause has been postulated

150
Q

Features of Reye’s syndrome

A

The peak incidence is 2 years of age, features include:
there may be a history of preceding viral illness
encephalopathy: confusion, seizures, cerebral oedema, coma
fatty infiltration of the liver, kidneys and pancreas
hypoglycaemia

151
Q

Stereotypical history of cluster headaches

A

a male smoker presents with recurrent episodes of severe pain around one eye which typical lasts around 1 hour. These episodes have occured daily for the past 6 weeks and are associated with ocular erythema and lacrimatio

152
Q

Features of cluster headaches

A

pain typical occurs once or twice a day, each episode lasting 15 mins - 2 hours
clusters typically last 4-12 weeks
intense sharp, stabbing pain around one eye (recurrent attacks ‘always’ affect same side)
patient is restless and agitated during an attack
accompanied by redness, lacrimation, lid swelling
nasal stuffiness
miosis and ptosis in a minority

153
Q

Management of cluster headaches

A

acute: 100% oxygen (80% response rate within 15 minutes), subcutaneous triptan (75% response rate within 15 minutes)
prophylaxis: verapamil is the drug of choice. There is also some evidence to support a tapering dose of prednisolone

154
Q

stereotypical history of freidreich’s ataxia

A

a teenage boy presents with clumsy walking. On examination he has gait ataxia, an intention tremor and loss of lower limb reflexes

155
Q

Pathophysiology of Lambert-Eaton syndrome

A

Lambert-Eaton myasthenic syndrome is seen in association with small cell lung cancer and to a lesser extent breast and ovarian cancer. It may also occur independently as an autoimmune disorder. Lambert-Eaton myasthenic syndrome is caused by an antibody directed against presynaptic voltage-gated calcium channel in the peripheral nervous system.

156
Q

Features of Lambert-Eaton Syndrome

A

repeated muscle contractions lead to increased muscle strength (in contrast to myasthenia gravis)
in reality, this is seen in only 50% of patients and following prolonged muscle use muscle strength will eventually decrease
limb-girdle weakness (affects lower limbs first)
hyporeflexia
autonomic symptoms: dry mouth, impotence, difficulty micturating
ophthalmoplegia and ptosis not commonly a feature (unlike in myasthenia gravis)

157
Q

Kluver-Bucy syndrome

A

hypersexuality, hyperorality, hyperphagia, visual agnosia

disease of the amygdala

158
Q

Features of Ramsay Hunt syndrome

A

auricular pain is often the first feature
facial nerve palsy
vesicular rash around the ear
other features include vertigo and tinnitus

159
Q

What is Ramsay Hunt syndrome

A

Ramsay Hunt syndrome (herpes zoster oticus) is caused by the reactivation of the varicella zoster virus in the geniculate ganglion of the seventh cranial nerve.

160
Q

Management of Ramsay Hunt Syndrome

A

oral aciclovir and corticosteroids are usually given

161
Q

Stereotypical history of BPPV

A

a 55-year-old woman presents with recurrent episodes of vertigo that are often triggered by a change in head position. Each episode lasts around 10-20 seconds and is associated with nausea

162
Q

sub acute combined degeneration of the spinal cord

A

due to vitamin B12 deficiency
dorsal columns + lateral corticospinal tracts are affected
joint position and vibration sense lost first then distal paraesthesia
upper motor neuron signs typically develop in the legs, classically extensor plantars, brisk knee reflexes, absent ankle jerks
if untreated stiffness and weakness persist

163
Q

Complications of SAH (aneurysmal)

A
Re-bleeding
Vasospasm (also termed delayed cerebral ischaemia), typically 7-14 days after onset
Hyponatraemia (SIADH)
Seizures
Hydrocephalus
Death
164
Q

Klumpke’s palsy (claw hand)

A

traction or tear of the lower trunk of the brachial plexus (C8 to T1)
wasting of intrinsic hand muscles and flexion deformities of the proximal interphalangeal (PIP) & distal interphalangeal (DIP) joints.

165
Q

Cerebral Autosomal Dominant Arteriopathy with Subcortical Infarcts and Leukoencephalopathy (CADASIL)

A

rare cause of multi-infarct dementia

patients often present with migraine

166
Q

Causes of gingival hyperplasia

A

phenytoin
ciclosporin
calcium channel blockers (especially nifedipine)

167
Q

Normal pressure hydrocephalus

A

urinary incontinence
dementia and bradyphrenia
gait abnormality (may be similar to Parkinson’s disease)

168
Q

Lambert-Eaton myasthenic syndrome

A

associated with small cell lung cancer (also breast and ovarian)
antibody directed against pre-synaptic voltage gated calcium channel in the peripheral nervous system
can also occur independently as autoimmune disorde

169
Q

Lambert-Eaton myasthenic syndrome

A

associated with small cell lung cancer (also breast and ovarian)
antibody directed against pre-synaptic voltage gated calcium channel in the peripheral nervous system
can also occur independently as autoimmune disorder

170
Q

Anti-Hu

A

associated with small cell lung carcinoma and neuroblastomas
sensory neuropathy - may be painful
cerebellar syndrome
encephalomyelitis

171
Q

Anti-Yo

A

associated with ovarian and breast cancer

cerebellar syndrome

172
Q

Anti-GAD antibody

A

associated with breast, colorectal and small cell lung carcinoma
stiff person’s syndrome or diffuse hypertonia

173
Q

Anti- Ri

A

associated with breast and small cell lung carcinoma

ocular opsoclonus-myoclonus

174
Q

Purkinje cell antibody

A

peripheral neuropathy in breast cancer

175
Q

hemiballismus

A

type of chorea which is caused by a decreased activity in the subthalamic nucleus of the basal ganglia in most case

176
Q

Wernicke’s area

A

Brodmann area 22 in the superior temporal gyrus