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MRCP PACES > Neurology > Flashcards

Neurology Flashcards

1
Q

What are the clinical signs of dystrophia myotonica?

A

Face: long thin expressionless face; wasting of facial muscles; bilateral ptosis; frontal balding; dysarthria
Hands: myotonia; wasting and weakness; percussion myotonia
Cataracts
Cardiomyopathy, arrhythmia (look for PPM)
Diabetes
Dysphagia

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2
Q

Explain the genetics of dystonia myotonica

A

DM1: expansion of CTG trinucleotide repeat sequence in DMPK gene on chromosome 19

DM2: expansion of CCTG tetranucleotide repeat sequence within ZNF9 gene on chromosome 3

Shows genetic anticipation

Both are autosomal dominant

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3
Q

How is dystonia myotonica diagnosed?

A

Clinical features
EEG: dive-bomber potentials
Genetic testing

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4
Q

How is dystrophia myotonica managed?

A

Patients die prematurely of cardiac and respiratory complications
Weakness is a major problem
Phenytoin may help myotonia
Advise against general anaesthetic (high risk cardiac/respiratory complications)

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5
Q

What are the common causes of unilateral ptosis?

A

Third nerve palsy

Horner’s syndrome

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6
Q

What are the clinical signs of cerebellar syndrome?

A

DANISH

Dysdiadochokinesis
Ataxia
Nystagmus
Intention tremor
Scanning dysarthria
Hypotonia/hyporeflexia
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7
Q

What are the causes of cerebellar syndrome?

A

PASTRIES

Paraneoplastic cerebellar syndrome
Alcoholic cerebellar degeneration 
Sclerosis (MS)
Tumour (posterior fossa)
Rare (Friedrich’s ataxia)
Iatrogenic (phenytoin toxicity)
Endocrine (hypothyroidism)
Stroke (brain stem)
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8
Q

What are the clinical signs of multiple sclerosis?

A

Internuclear ophthalmoplegia, optic atrophy, reduced visual acuity, any cranial nerve palsy
Upper motor neurone spastictiy, weakness, brisk reflexes, altered sensation
Cerebellar syndrome (DANiSH)

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9
Q

What are the diagnostic criteria for multiple sclerosis?

A

Central nervous system demyelination causing neurological impairment that is disseminated in both space and time

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10
Q

What is the cause of multiple sclerosis?

A

Unknown
But, both genetic and environmental factors appear to play a role

Environmental- increasing latitude, epstein-barr infection
Genetic - HLA-DR2, interlukin 2 and 7

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11
Q

How would you investigate a patient with suspected multiple sclerosis?

A

CSF: oligoclonal IgG bands
MRI: peri ventricular white matter plaques
Visual evoked potentials: delayed velocity but normal amplitude, suggests previous optic neuritis

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12
Q

What are the other (non-neurological) clinical features of multiple sclerosis?

A 
Depression
Urinary retention or incontinence
Impotence 
Bowel problems
Uthoff’s phenomenon - symptoms worse after a hot bath or exercise
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13
Q

How is MS treated?

A

Multidisciplinary team involvement
Disease-modifying treatments -

Interferon beta and glatiramer reduce relapse rate but don’t affect progression
Monoclonal antibody therapy use may be limited by toxicity

Symptomatic treatments - methylprednisolone during acute relapse may shorten duration, anti-spasmodics, carbamazepine, laxatives

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14
Q

What is the definition of a stroke and a transient ischaemic attack?

A

Stroke - rapid onset, focal neurological deficit due to a vascular lesion lasting more than 24 hours

TIA - focal neurological deficit lasting less than 24 hours

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15
Q

What is the Bamford classification of stroke?

A

Total anterior circulation stroke (hemiplegia, homonomous hemianopia, dysphasia, dyspraxia, neglect)

Partial anterior circulation (2/3 of TACS symptoms)

Lacunar (pure hemi- motor or sensory loss)

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16
Q

What is lateral medullary syndrome?

A

Most common brainstem vascular syndrome
Due to occlusion of the posterior inferior cerebellar artery
Variable in it’s presentation

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17
Q

How does lateral medullary syndrome present?

A

Ipsilateral: cerebellar signs, nystagmus, horner’s syndrome, palatal paralysis, loss of trigeminal pain and temperature sensation

Contralateral: loss of pain and temperature sensation

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18
Q

What are the causes of lower limb spasticity?

A

Spinal cord lesions i.e myelopathy

Hyperacute - likely spinal cord infarct/ischaemia, trauma
acute - disc bulge, metastatic cord compression
Sub-acute - MS
Chronic - b12 deficiency, genetic causes (hereditary spastic paraparesis)

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19
Q

What are the clinical signs of syringomyelia?

A

Weakness and wasting of the small muscles of the hands
Loss of reflexes in the upper limbs
Loss of pain and temperature sensation with preservation of joint position and vibration sense
Charcot joints
Pyramidal weakness in legs with upgoing plantars
Kyphoscoliosis
Horner’s syndrome

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20
Q

What is syringomyelia?

A

Caused by a progressively expanding fluid filled cavity within the spinal cord, typically spanning several levels

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21
Q

What are the most important causes of a Charcot joint?

A

Tabes dorsalis (hip and knee)
Diabetes (foot and ankle)
Syringomyelia (shoulder and elbow)

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22
Q

What are the clinical signs of motor neurone disease?

A

Wasting and fasiculation
Usually spastic but may be flaccid
Weakness
Reflexes may be absent or brisk, upgoing plantars
Normal sensory examination
Speech may be bulbar (palatal weakness) or pseudo-bulbar (spastic tongue)

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23
Q

What is the pathophysiology of motor neurone disease?

A

Progressive disease of unknown aetiology

Axonal degeneration of upper and lower motor neurones

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24
Q

What are the types of motor neurone disease?

A

Amyotrophic lateral sclerosis: affects cortico-spinal tracts and predominantly produces spastic parapesis and tetraparesis

Progressive muscular atrophy: affects anterior horn cells, predominantly producing wasting, fasciculation and weakness, has the best prognosis

Progressive bulbar palsy: affecting lower cranial nerves and suprabulbar nuclei producing speech and swallow problems, has the worst prognosis

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25
Q

How would you investigate a patient with suspected motor neurone disease?

A

Clinical diagnosis
EMG: fasiculation
MRI: excludes cord compression and brain stem lesions

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26
Q

What is the management of motor neurone disease?

A

Multidisciplinary approach (including communication aids, PT, OT, SALT, dietetics)

Measure FVC and consider ABG as patients may require NIV

Riluzole slows disease progression but does not improve function or quality of life

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27
Q

What is the prognosis of motor neurone disease?

A

Most die within 3 years of diagnosis from pneumonia and respiratory failure

Worse if elderly at onset, female and with bulbar involvement

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28
Q

What are the causes of wasting of the hand muscles?

A 
Motor neurone disease
Syringomyelia 
Cervical cord compression
Polio
Pancoast’s tumour
Trauma
Peripheral neuropathy
Disuse atrophy
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29
Q

What are the lumbosacral root levels and corresponding movements?

A 
L2/3 - hip flexion
L3/4 - knee extension, knee jerk
L4/5 - ankle dorsiflexion
L5/S1 - knee flexion, hip extension
S1/2 - foot plantarflexion, ankle jerk
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30
Q

What are the cervical roots and corresponding movements?

A

C5/6 - elbow flexion and supination, biceps and supinator jerks
C7/8 - elbow extension, triceps jerk
C8/T1 - finger adduction

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31
Q

What are the clinical signs of Parkinsonism?

A 
Expressionless face
Coarse, pill-rolling tremor (usually asymmetrical)
Bradykinesia
Cog-wheel rigidity
Shuffling gait with absent arm swing
Slow, faint and monotonous speech
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32
Q

How would you distinguish Parkinson’s disease from multi-system atrophy?

A

Parkinsonism with postural hypotension, cerebellar and pyramidal signs

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33
Q

What are the causes of Parkinsonism?

A 
Parkinson’s disease
Parkinson’s plus syndromes
Drug-induced (phenothiazines)
Anoxic brain injury
Post-encephalitis
MPTP toxicity
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34
Q

What are the Parkinson’s plus syndromes?

A

Multi-system atrophy
Progressive supranuclear palsy
Corticobasal degeneration (unilateral Parkinsonian signs)

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35
Q

What is the pathology of Parkinsonism?

A

Degeneration of the dopaminergic neurones between the substantia nigra and the basal ganglia

36
Q

What is the treatment of Parkinson’s?

A

L-Dopa (issues are nausea, dyskinesia, effects of treatment wearing off)
Dopamine agonists (bromocriptine - less side effects and delay need to start l-dopa)
MAOB inhibitors
Anticholinergics (useful in drug induced tremor)
Surgery such as deep brain stimulation may be useful in some cases

37
Q

What are the causes of tremor?

A

Resting - Parkinsonism

Postural - benign essential tremor, anxiety, thyrotoxicosis, CO2, hepatic encephalopathy, alcohol

Intention - cerebellar disease

38
Q

What are the clinical signs of hereditary sensory motor neuropathy?

A

Wasting of distal lower limb muscles with preservation of thigh muscles (inverted champagne bottle)

Pes cavus
Weakness of ankle dorsiflexion and toe extension
Stocking sensory loss
High stepping gait (foot drop)
Stamping (absent proprioception)
Wasting of hand muscles
39
Q

What are the types of hereditary sensory motor neuropathy?

A

Also known as Charcot-Marie-Tooth disease

HSMN 1 (demyelinating)
HSMN 2 (axonal)

Autosomal dominant inheritance

40
Q

What is the management of hereditary sensory motor neuropathy?

A 
No disease modifying treatments available 
Multi-disciplinary team approach
Orthotics
Occupational therapy
Analgesia
41
Q

What are the causes of pes cavus?

A

Unilateral: burns, previous compartment syndrome, poliomyelitis, spinal trauma, spinal cord tumours

Bilateral: Friedrich’s ataxia, muscular dystrophy, cerebral palsy, syringomyelia, hereditary spastic paraparesis, hereditary sensory motor neuropathy

42
Q

What are the causes of a predominantly sensory peripheral neuropathy?

A

Metabolic: Diabetes, vitamin deficiencies
Toxic: Alcohol, medications (isoniazid), chemotherapy
Auto-immune: rheumatoid arthritis, Hypothyroidism, vasculitis, GBS, SLE
Infective: herpes zoster, HIV
Haematological: MGUS, myeloma, lymphoma
Genetic: Charcot-Marie-Tooth disease

43
Q

What are the causes of a predominantly motor peripheral neuropathy?

A

Acute: Gullian-Barre, botulism

Lead toxicity
Porphyria
HSMN

44
Q

What are the clinical signs of Friedrich’s ataxia?

A

Young adult with an ataxic gait
Pes cavus
Bilateral cerebellar ataxia
Leg wasting with absent reflexes and bilateral upgoing plantars
Posterior column signs (loss of vibration and proprioception)

Others: high arched palate, kyphoscoliosis, HOCM, diabetes (check urine glucose)

45
Q

What do you know about Friedrich’s ataxia?

A

Inheritance is usually autosomal recessive
Onset is during teenage years
Survival rarely exceeds 20 years from diagnosis
Association with HOCM and a mild dementia

46
Q

What are the causes of extensor plantars with absent knee jerks?

A

Friedrich’s ataxia
Subacute combined degeneration of the cord
Motor neurone disease

47
Q

What are the clinical signs of a facial nerve palsy?

A

Unilateral facial droop
Absent nasolabial fold
Absent forehead crease
Inability to raise eyebrows, screw eyes up or smile

48
Q

How can you localise the lesion causing facial nerve palsy?

A

Pons (MS, stroke) - VI palsy and long tract signs

Cerebellar-pontine angle (Acoustic neuroma) - V, VI and VIII and cerebellar signs

Auditory/facial canal (Cholesteatoma, abscess) - VIII

49
Q

What are the causes of a unilateral facial palsy?

A 
Bell’s palsy is the commonest
Herpes-zoster (ramsay hunt syndrome)
Mononeuropathy (diabetes, sarcoid, Lyme)
Tumour
MS/stroke
50
Q

What are the causes of a bilateral facial palsy?

A 
Guillian Barre
Myasthenia gravis
Sarcoidosis
Bilateral Bell’s palsy
Lyme disease
51
Q

What is Bell’s palsy?

A

Rapid onset facial nerve palsy
HSV-1 implicated
Swelling and compression of the nerve within the facial canal

Treatment: prednisolone, aciclovir, eye protection

Prognosis: 70-80% make a full recovery, more common and may have poorer outcomes in pregnancy

52
Q

What are the clinical signs of myasthenia gravis?

A 
Bilateral ptosis
Complicated bilateral extra-ocular muscle palsies
Myasthenic snarl
Nasal speech
Proximal muscle weakness in upper limbs with fatiguability
Normal reflexes 
Sternotomy scars (thymectomy)
Assess FVC
53
Q

What other conditions may be associated with myasthenia gravis?

A 
Diabetes 
Rheumatoid arthritis 
Thyrotoxicosis
SLE
Thymomas
54
Q

What is the cause of myasthenia gravis?

A

Anti-nicotinic acetylcholine receptor antibodies affect motor end plate neurotransmission

55
Q

How would you investigate a patient with suspected myasthenia gravis?

A

Anti acetlycholine receptor antibodies present in 90%
Anti-MuSK often positive if above negative
EMG - decreased response to titanic train of impulses
Tensilon test
CT (thymoma), thyroid function

56
Q

How would you treat a patient with myasthenia gravis?

A

IV immunoglobulin or plasmapharesis in acute setting

Acetylcholine esterase inhibitor (pyridostigmine)
Immunosuppression (steroids, azathioprine)
Thymectomy usually beneficial even if patient does not have a thymoma

57
Q

What is Lamber-Eaton myasthenic syndrome?

A

Diminished reflexes that improve after exercise
Lower limb girdle weakness
Associated with malignancy
Antibodies block presynaptic calcium channels
“Second wind” on EMG

58
Q

What are the causes of bilateral extra ocular palsies?

A

Myasthenia gravis
Graves disease
Miller-Fischer syndrome
Cavernous sinus pathology

59
Q

What are the causes of a bilateral ptosis?

A 
Congenital
Senile
Myasthenia gravis 
Myotonic dystrophy 
Bilateral Horner’s syndrome
60
Q

What are the clinical signs of tuberous sclerosis?

A 
Butterfly rash, periingual fibromas, Shagreen patch, Ash leaf macules
Cystic lung disease
Polycystic kidneys
Evidence dialysis or renal transplant
White patches on retina
Seizures
Signs of epileptic treatment
61
Q

What is tuberous sclerosis?

A

Autosomal dominant condition with variable penetrance
80% have epilepsy
Cognitive defects in 50%

62
Q

How would you investigate a patient with suspected tuberous sclerosis?

A

Skull xray - railroad calcifications
CT/MRI head - tuberous mass in cerebral cortex
Echo
Abdominal USS - renal cysts

63
Q

What are the clinical signs of neurofibromatosis?

A 
Cutaneous neurofibromas
Cafe au lait patches >15
Axillary freckling
Lisch nodules in the iris
Hypertension (renal artery stenosis, phaeochromocytoma)
Fibrosis
Neuropathy with large palpable nerves
Reduced visual acuity
64
Q

What is the inheritance of neurofibromatosis?

A 
Autosomal dominant
Type 1 (chromosome 17) is classical peripheral form
Type 2 (chromosome 22) is central and presents with bilateral acoustic neuromas and deafness
65
Q

What conditions is neurofibromatosis associated with?

A

Phaeochromocytoma

Renal artery stenosis

66
Q

What are the complications of neurofibromatosis?

A

Epilepsy
Sarcomatous change
Scoliosis
Mental retardation

67
Q

What is a Horner’s pupil?

A

Ptosis
Sunken eye
Anhydrosis

68
Q

What are the causes of a Horner’s pupil?

A

MS, Stroke (Brain stem)
Syrinx (Spinal cord)
Aneurysm, trauma, Pancoast tumour (neck)

69
Q

What is a Holmes-Adie pupil?

A

Moderately dilated pupil with a poor response to light and sluggish accommodation

May have absent ankle and knee jerks

A benign condition that is more common in females

70
Q

What is an Argyll Robertson pupil?

A

Small, irregular pupil which accommodates but does not react to light
Atrophied and depigmented iris

Look for sensory ataxia

Usually a manifestation of quaternary syphyllis, but may be related to diabetes

71
Q

What are the clinical signs of an oculomotor nerve palsy?

A

Complete ptosis
Dilated pupil
Eye points down and out

72
Q

What are the causes of an oculomotor nerve palsy?

A

Medical: mononeuritis multiplex, midbrain stroke, MS, migraine

Surgical: posterior communicating artery aneurysm, cavernous sinus pathology, cerebral uncus herniation

73
Q

What are the clinical signs of optic atrophy?

A

Relative afferent pupillary defect

Disc pallor

74
Q

What are the causes of optic atrophy?

A

Glaucoma, tumour, Paget’s and MS most common

Retinitis pigmentosa
Central retinal artery occlusion
Friedrich’s ataxia
Tertiary syphyllis

75
Q

What are the clinical signs of age related macular degeneration?

A

Drusen
Fibrosis
Neovascularisation

76
Q

What are the causes of age related macular degeneration?

A

Risk factors: age, white race, family history, smoking

Associated with coronary artery disease and stroke

77
Q

What are the causes of retinitis pigmentosa?

A

Friedrich’s ataxia
Kearns-Sayre syndrome

Congenital: usually autosomal recessive
Acquired: post-inflammatory retinitis

No treatment although vitamin A may slow progression

78
Q

What are the clinical signs of retinal artery occlusion?

A

Pale fundus with thread like arterioles
Cherry red macula

May have signs AF or a carotid bruit

79
Q

What are the causes of retinal artery occlusion?

A

Embolic (carotid plaque rupture) - treat with aspirin, anticoagulation and endarterectomy

Giant cell arteritis - high dose steroids

80
Q

What are the clinical signs of retinal vein occlusion?

A

Flame haemorrhages
Swollen optic disc
Cotton wool spots
Tortuous veins

May have visual field defect or signs hypertension and diabetes

81
Q

What are the causes of retinal vein occlusion?

A

Hypertension
Hyperglycemia
Hyperviscocity (myeloma)
High intraocular pressure (glaucoma)

82
Q

How would you differentiate cerebellar ataxia from sensory ataxia on examination?

A

Cerebellar ataxia usually has associated nystagmus and dysarthria

Sensory ataxia usually has sensory impairment, particularly joint position sense and vibration sense.
In sensory ataxia, finger-nose test may be normal with eyes open, but impaired with eyes closed

83
Q

How would you investigate a patient with cerebellar ataxia?

A

MRI is far superior to CT for looking at brainstem lesions
However, CT important in the acute setting to rule out haemorrhage

Investigate the cause- LP if ?MS, thyroid function tests

84
Q

What are the causes of sensory ataxia?

A

Central: spinal cord pathology (dorsal column damage) - cervical myelopathy

Peripheral neuropathy - alcohol, diabetes, b12 deficiency, platinum chemotherapies, HIV, Sjorgren’s syndrome, paraneoplastic syndrome

Combined peripheral and central: B12 deficiency

85
Q

How would you investigate a patient with peripheral neuropathy?

A

Look for the cause

Urine dip, fundoscopy, blood glucose
Bloods: FBC, haemantinics, HbA1c, auto-immune screen, vasculitis screen, HIV tests, paraproteins
Nerve conduction studies to distinguish between axonal and demyelinating conditions

Full history including social and family history

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