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RACP short cases > Neurology > Flashcards

Neurology Flashcards

1
Q

Causes of ptosis with normal pupils

A 
senile ptosis
myotonic dystrophy 
fascioscapulohumeral dystrophy
ocular myopathy 
thyrotoxic myopathy 
myasthenia gravis 
congenital
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2
Q

Causes of Horners

A 
Pancoast tumour (tumour in apex of lung)
Neck malignancy eg - thyroid 
brachial plexus lesion 
carotid artery lesion eg - aneurysm 
brainstem lesion eg - lateral meduallary syndrome
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3
Q

Features of third nerve palsy

A

Ptosis
Eye is down and out (divergent strabismus)
Mydriasis, unreactive to light or accommodation

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4
Q

Causes of CN3 palsy - by location

A

Problem at the nucleus - infarct, haemorrhage, mass
Problem at the fascicles - infarct, tumour, fasicles, demyelination
Problem in the subarachnoid space - Aneurysm (PCOmm)
Problem at the cavernous sinus - tumour, inflammation, carotid aneurysm

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5
Q

Causes of CN3 palsy - central or peripheral

A

Central causes - brain stem infarct, tumour, demyelination, trauma
Peripheral causes - Compression by eg aneurysm, tumour, raised intracranial pressure. Infarct from microvascular ischaemia (eg from diabetes, GCA), trauma, cavernous sinus lesion

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6
Q

Features of CN6 palsy

A

At rest - if severe affected eye adducted
Looking towards the lesion - affected eye unable to abduct, horizontal nystagmus
Looking away from lesion - normal

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7
Q

Causes of CN6 palsy

A

Vascular cause - infarct, haemorrhage, basilar artery aneurysm
Space occupying lesion in the pons
Demyelination - MS
Inflammatory - sarcoid, giant cell arteritis
Cavernous sinus lesion
Mononeuritis multiplex
Trauma
Central - vascular, tumour, Wernicke’s encephalopathy, MS
Peripheral - microsvascular ischaemia (eg - diabetes), trauma, raised intracranial pressure

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8
Q

Causes of jerky horizontal nystagmus

A

Vestibular (peripheral) - unidirectional with fast component is away from the side of the lesion
Vestibular (central) - bidirectional, left beating on left gaze, right beating on right gaze
Cerebellar - fast component towards the side of the lesion
Toxins - phenytoin, alcohol

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9
Q

Causes of jerky vertical nystagmus

A

Brain stem lesion
- Upbeating: lesion at floor of 4th ventricle
- Downbeating: lesion at foramen magnum
Toxins - phenytoin, alcohol

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10
Q

Causes of mononeuritis multiplex

A

Rheumatologic conditions - Sjogrens, SLE, RA
Vasculitis - PAN, giant cell arteritis
Other chronic conditions - diabetes, neurosarcoid, amyloidosis
Paraneoplastic
Infections such as HIV

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11
Q

Causes of peripheral neuropathy

A

D - drugs and toxins like isoniazid, vincristine, platinum based chemo
A - Alcohol, amyloid
M - Metabolic like diabetes, thyroid, uraemia
I - Immune mediated like GBS
T - tumour, lung carcinoma, paraproteinaemia
B - vitamin B12 deficiency
I - Idiopathic
C - Connective tissue disease or vasculitis
H - Hereditary motor and sensory neuropathy

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12
Q

Signs of median nerve lesion

A
  1. Weak thumb abduction
  2. Wasting of the thenar eminence
  3. Hand of benediction if more proximal lesion
  4. Sensory loss to the palmar aspect of thumb, index and middle (and half of ring)
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13
Q

Signs of a radial nerve lesion

A
  1. Wrist drop/finger drop
  2. Sensory loss over the anatomical snuffbox
  3. Weak elbow extension if high enough
  4. Absent triceps jerk
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14
Q

Signs of ulnar nerve lesion

A
  1. Wasting of the hypothenar eminence
  2. Weak abduction and adduction
  3. Claw hand when trying to straighten
  4. Sensory loss over the dorsal and palmar little and half of ring finger
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15
Q

How do you distinguish a C8 nerve root lesion/lower trunk brachial plexus lesion from ulnar nerve lesion?

A
  1. sensory loss of a C8 root/lower trunk plexus extends above the wrist
  2. Thenar wasting with C8 root/lower trunk plexus
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16
Q

Signs of femoral nerve lesion

A
  1. Weak knee extension
  2. Loss of sensation over inner thigh and leg
  3. Strong adduction
  4. Slight weak hip flexion
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17
Q

Signs of sciatic nerve lesion

A
  1. Foot drop
  2. Knee Extension weakness
  3. loss of sensation in posterior part of thigh and all of lower leg
  4. Loss of power to all muscles below knee
  5. Unable to stand on toes or heels
  6. Loss of ankle jerk
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18
Q

Common peroneal nerve lesion

A
  1. Foot drop
  2. Loss of sensation to the dorsum of foot
  3. Weak eversion
  4. Normal power inversion (L5 nerve root will be all of above and weak inversion)
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19
Q

Nerve fibre types for motor and sensory

A

Motor - Large myelinated nerve fibres
Proprioception and vibration - large myelinated nerve fibres (A-beta)
Pain and temperature - small myelinated and unmyelinated fibres
Autonomic - small fibres

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20
Q

Nerve fibre types for motor and sensory

A

Motor - Large myelinated nerve fibres
Proprioception and vibration - large myelinated nerve fibres (A-beta)
Pain and temperature - small myelinated and unmyelinated fibres

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21
Q

Which nerve pathways carry pain/temperature vs vibration/proprioception

A

DCML - fine touch, proprioception and vibration

Lateral spinothalamic tract - pain and temperature

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22
Q

Causes of mononeuritis multiplex

A 
Metabolic causes - Diabetes
Vasculitis - PAN, Churg-Strauss, Wegeners
Connective tissue - RA, SLE, Sjogrens
Sarcoid 
Amyloid 
Paraneoplastic
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23
Q

Tests to order for peripheral neuropathy

A 
Bloods - FBC, ESR, CRP, U and E, TFTs, HbA1c, B12, Serum protein electrophoresis, Autoimmune profile 
Urine - Bence jones, glucose 
Nerve conduction 
Genetic testing 
Nerve biopsy
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24
Q

Peripheral neuropathies that are predominantly motor

A 
CIDP 
GBS
CMT 
Porphyria 
Lead poisoning
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25
Q

Axonal vs demyelinating polyneuropathy

A

Axonal - more distal sensory loss, wasting and weakness of hands/feet
Demyelinating - vibration/proprioception (large myelinated fibres) loss > pain/temp, weakness can be more generalised

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26
Q

Demyelinating vs axonal neuropathy features on NCS

A

Demyelinating - slow nerve conduction velocities, prolongation of distal latencies
Axonal - reduced amplitude of evoked compound action potentials, relative preservation of the nerve conduction velocity

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27
Q

If there is wasting of the hand in a neuro exam, when can the pathology originate from?

A

Anterior horn cell, nerve root or the lower motor neurons that arise from C8 to T1

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28
Q

Clinical features of Charcot Marie Tooth disease

A
  1. Pes cavus - high arch, short foot, hammer toe
  2. Inverted champagne bottle legs
  3. Distal muscle atrophy and weakness - mainly hands and feet. Get weak eversion
  4. Absent reflexes
  5. Foot drop
  6. Thickened nerves - esp lateral popliteal nerve
  7. Sensory loss - vibration and proprioception
  8. Claw hand
  9. Optic atrophy
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29
Q

Nerve conduction studies in CMT disease

A

Can be motor and sensory involved
Can be predom axon or demyelinating or mixed
Demyelinating - get decreased conduction velocities
Axonal - get decreased amplitudes

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30
Q

Differentiating neuropathy from myopathy

A

Myopathy - usually more proximal, no fasciculations, no sensory loss, deep tendon reflexes intact unless severe, can have mm tenderness
Neuropathy - usually more distal, fasciculations, can have sensory loss, loss of reflexes

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31
Q

What are the main clinical features of myotonic dystrophy?

A

Face - frontal balding, facial muscle atrophy (temporalis, masseter, sternomastoid), high arched palate (some), ptosis, cataracts
Neck - weak neck flexion
Hands - grip myotonia, percussion myotonia
Wasting UL and LL esp intrinsic hand mm

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32
Q

What are some of the systemic features of myotonic dystrophy?

A

Cardiac - arrythmias, heart block, mitral valve prolapse, cardiomyopathy
GIT - dysphagia, hypomotility, reflux, delayed gastric emptying
Endocrine - diabetes, hypogonadism, nodular thyroid involvement

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33
Q

What is the EMG finding in myotonic dystrophy?

A

Characteristic “dive bomber” sound of myotonia

34
Q

What is the blood supply to the different parts of the brainstem?

A

Medulla - vertebral arteries
Pons - Basilar artery
Midbrain - either the PCA or the superior cerebellar

35
Q

Where is the lesion in intranuclear ophthalmoplegia ?

A

Medial longitudinal fasciculus (heavily myelinated tract that in simple terms connects ipsilateral CN III with contralateral CN VI and is involved in the control of conjugate eye movements

36
Q

Describe the clinical features of an INO?

A

Unilateral INO (eg - affecting the right medial longitudinal fasciculus) - when looking to the left, the right eye will not adduct, nystagmus in the left eye, diplopia
Bilateral INO - when looking to the right the the left eye cannot adduct, when looking to the left the right eye cannot adduct, opposite eye may have nystagmus
The adduction becomes normal when contralateral (abducting) eye is covered

37
Q

What are cerebellar eye signs?

A

nystagmus towards the side of the cerebellar lesion, hypo/hypermetric saccades, broken smooth pursuit

38
Q

What are the most common causes of an INO?

A
  1. MS - can be unilateral or bilateral
  2. Brainstem lesion
  3. Wernicke’s encephalopathy
  4. SLE
  5. Miller Fisher Syndrome
  6. Drug overdose
39
Q

which cranial nerves are in which part of the brainstem?

A 
I and II - from the cerebrum  
IV - posterior side of midbrain 
III - midbrain-pontine junction 
V - pons 
VI - VIII - pontine-medullary junction 
IX-XII - medulla 
(V, VII, VIII) - cerebellopontine angle
40
Q

Bulbar vs pseudobulbar palsy

A

Bulbar (LMN affecting X, XI, XII) - absent gag reflex, wasted tongue with fasciculations, absent or normal jaw jerk, nasal sounding voice, limb fasciculations
Pseudobulbar (UMN affecting X, XI, XII) - increased or normal gag reflex, spastic tongue, increased jaw jerk, spastic dysarthria, limb UMN signs, labile emotions

41
Q

CN III, IV, V, Vi affected, where may the lesion be?

A

Cavernous sinus

42
Q

CN V, VII, VIII affected, where may the lesion be?

A

Cerebellopontine angle

43
Q

Unilateral Ix, X, XII affected, where may the lesion be?

A

jugular foramen

44
Q

Visual field deficits - where is the lesion?

A

Bitemporal hemianopia - optic chiasm
HH - optic tract to occipital cortex
Upper HQ - temporal lobe
Lower HQ - parietal lobe

45
Q

What are the major causes of myopathy?

A

Inflammatory causes - polymyositis, dermatomyositis, inclusion body myositis, vasculitis
Endocrine - hypo/hyperthyroidism, Cushing’s
Drugs - steroids, alcohol, statins
Inheritied causes - muscular dystrophy
Electrolyte disturbances

46
Q

Which conditions can cause both proximal myopathy and peripheral neuropathy?

A

Alcohol
Endocrine
Paraneoplastic
Connective tissue diseases

47
Q

What are the clinical features of dermatomyositis?

A 
Heliotrope rash 
Periorbital oedema 
Gottron papules 
Periungual telangectasia 
Mechanics hands 
hyperpigmentation and telangectasias in sun-expose areas (shawl sign)
Symmetrical proximal muscle weakness 
Arthritis 
Signs of interstitial lung disease
48
Q

Parkinson’s disease vs Parkinson’s plus differentiation

A 
Clinically 
- Parkinson plus more symmetrical, early onset postural instability, early onset autonomic dysfunction (shy drager), pyramidal signs, cerebellar signs (MSA-C), gaze palsies (PSP), more axial rigidity in PSP, limb apraxia and cortical sensory loss in corticobasal degeneration 
Other 
- Poor response to levodopa 
- Early onset dementia 
- early onset hallucinations
49
Q

What are the Parkinson Plus syndromes

A

Progressive supranuclear palsy
Multiple Systems atrophy
Corticobasal degeneration

50
Q

What is most commonly seen on nerve conduction studies in CMT?

A

Reduced nerve conduction velocities indicating a demyelinating pattern

51
Q

What are the causes of pronator drift?

A

Downwards drift - UMN pyramidal weakness
Upward drift - cerebellar lesion
Posterior column - Drift in any direction from proprioception loss

52
Q

Which cranial nerves are affected in a cerebellopontine angle lesion?

A

CN 5, 6, 7 and 8 (CN 6 palsy may or may not be present)

53
Q

What are the clinical features of a cerebellopontine angle lesion?

A

Possible CN 6 palsy
Weakness of muscles of facial expression (CN7)
Loss of sensation over face (CN5)
Loss of corneal reflex (CN5)
Weakness of mm of mastication (CN5)
Sensorineural hearing loss (CN8) (Rinnes normal, Weber’s decreased on affected side)
CN IX-XII spared

54
Q

What are the 2 most common causes of a cerebellopontine angle lesion?

A

Acoustic neuroma

Meningioma

55
Q

Spinal cord lesion motor signs?

A

Weakness, spasticity, increased reflexes, Babinski in muscle groups innervated below the level of the lesion
Can also get LMN signs in a myotomal distribution at the specific level of involvement

56
Q

Motor neuron disease features on clinical examination

A

Bulbar or pseudobulbar palsy
Mix of upper and lower motor neuron signs
No or very minimal sensory loss

57
Q

DDx for motor neuron disease

A

Cervical radiculomyelopathy - would get UMN signs below level of lesion and LMN signs at the level. Get sensory loss as differentiator
Syringomyelia - UMN signs in legs and LMN signs in arms
Other spinal cord injury - may cause UMN signs, would get sensory signs
Multifocal motor neuropathy - LMN, slowly progressive distal weakness and atrophy
CIDP - LMN, get autonomic dysfunction and may get sensory loss
Spinal muscular atrophy - LMN, can get bulbar palsy

58
Q

Causes of cerebellar syndrome

A 
Demyelinating disease 
Post circulation stroke 
Haemorrhage
Alcoholic cerebellar degeneration 
Friedrich's ataxia 
Posterior fossa space occupying lesion 
Paraneoplastic 
Drugs 
Hypothyroidism 
MSA-C
59
Q

If in peripheral neuropathy you get pain and temperature loss, what may you also test?

A

Postural BP - pain and temperature is small fibre neuropathy, autonomic nerves also small fibre

60
Q

What type of neuropathy do you get with diabetes?

A

Can be motor, sensory or sensorimotor

Autonomic neuropathy

61
Q

What type of neuropathy do you get with B12 deficiency?

A

Predominantly sensory neuropathy

62
Q

What type of neuropathy do you get with alcohol?

A

Sensory or sensorimotor

63
Q

What are the causes of the demyelinating polyneuropathies?

A 
GBS 
MGUS/myeloma 
CIDP 
CMT (type 1 and 3)
HIV 
Multifocal motor neuropathy
64
Q

Peripheral neuropathy. How would you like to investigate?

A

Bloods - B12, BSL, LFTs, Autoimmune and vasculitic screen, TFTs, EPG/IEPG
Urine - glucose, bence jones protein
Nerve conduction studies

65
Q

Common peroneal nerve palsy signs

A 
High stepping gait 
Foot drop 
Weak DF, big toe ext and Eversion 
Inversion spared 
Ankle reflex spared 
Sensory loss over lateral calf and dorsum of foot sparing 5th toe
66
Q

L5 nerve root lesion

A

High stepping gait
Foot drop
Weak DF, big toe ext, eversion and inversion
Weak hip abduction
Reflexes not affected
Sensory involvement lateral calf and dorsum of foot, can extend to thigh

67
Q

Causes of foot drop

A

L5 nerve root lesion
Lumbosacral plexus
Sciatic nerve
Common peroneal nerve
LMN - MND, other causes of motor neuropathies
Muscular - distal myopathies such as myotonic dystrophy
NMJ - Myasthenia Gravis

68
Q

Friedreich’s ataxia signs

A

Signs of cerebellar syndrome (bilateral)
Pes cavus, kyphoscoliosis, high arched palate
Pyramidal weakness
Distal wasting
Absent LL reflexes
Upgoing plantars
Impaired vibration and proprioception with positive Rombergs

69
Q

What is pyramidal weakness?

A

In upper limbs extensors weaker than flexors, In LLs flexors weaker than extensors

70
Q

What systemic features do you get in Friedreich’s ataxia?

A

Optic atrophy, diabetes, HCOM, sensorineural deafness

71
Q

Signs of hereditary spastic paraparesis

A 
LL > UL 
Spasticity 
Weakness
Increased reflexes 
Upgoing plantars 
Spastic gait with scissoring 
Can get loss of proprioception and vibration 
Can get pes cavus
72
Q

Muscular dystrophies - Duchenne’s (less likely to appear as more severe) and Becker’s

A 
Proximal > distal weakness 
Neck flexion > neck extension 
Pseudohypertrophy of the calf 
Weak tibialis anterior and wrist extensors 
contractures and kyphoscoliosis 
Waddling gait
73
Q

Facioscapulohumeral dystrophy features

A

Myopathic facies with generalised wasting and weakness of the muscles
Inability to close eyes tightly, smile or whistle
Weakness of the shoulder and upper arm muscles
Protrusion of the trapezeii muscles
Deltoid sparing
Riding of the scapulae upwards and winging
Forward jutting of the medial ends of the clavicles when the arms are abducted
Forearm muscles usually spared
Weakness of the lower abdominal muscles

74
Q

CIDP clinical features

A

symmetric sensorimotor polyneuropathy (motor predominant)
both proximal and distal muscle weakness
Foot drop
Sensory impairment - more vibration and temperature
Sensory ataxia
Can have decreased reflexes
Autonomic dysfunction

75
Q

Weakness pattern in facioscapulohumeral dystrophy

A

Facial muscle weakness with characteristic myopathic facies (without ptosis)
Weakness at the shoulder girdle
Protrusion of the trapezius
Scapula winging
Wasting of the pectoral muscles with sparing of deltoid
Forward jutting of the clavicles with arm abduction
Wasting of biceps and triceps with sparing of the forearm
Weakness of the lower abdo muscles with abdo protrusion
Upward deflection of the umbilicus with neck flexion (Beevor’s sign)
Weakness in distal LL with possible foot drop

76
Q

Pattern of weakness in Duchenne’s and Becker’s muscular dystrophy?

A 
Proximal > distal 
Legs first then arms and neck 
Neck flexors > extensors 
Facial musculature sparing 
Calf pseudohypertrophy
77
Q

On examination you find features of muscular dystrophy. if you had more time what else would you want to test for?

A

Cough
Forced exp time/spirometry
Cardiovascular exam - can get myocardial involvement

78
Q

Syringomyelia signs

A

Lower motor neuron signs in the upper limb distal > proximal - fasciculations, weakness, areflexia
Sensory dissociation - loss of pain and temperature bilaterally but not loss of vibration or proprioception
Upper motor neuron signs in the lower limbs
Kyphoscoliosis from weakness to the paraspinal muscles

79
Q

Explanation of signs in syringomyelia

A

Syrinx - fluid filled tubular cavity in the central spinal cord - can expand up and down and outwards
Anterior horn cells of spinal cord get affected - damage to LMN at those levels
The decussating fibres of the spinothalamic tract get affected - loss of pain and temperature
The UMNs of the corticospinal tracts get affected - LL UMN signs
The DCML fibres are spared - sparing of vibration and proprioception

80
Q

What is the function of CN4 in the primary position and in adduction of the eye?

A

In primary gaze, CN4 (superior oblique) intorts the eye

In adduction, CN4 (superior oblique) depresses the eye

81
Q

CN4 palsy

A

contralateral eye is slightly hypertropic on primary gaze
In the adducted position, difficult to depress the eye
Head tilt away from the affected eye

RACP short cases (8 decks)

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