Neurology SC Flashcards

1
Q

CMT

A

Disorder:
Autosomal dominant, peripheral nerve demyelination (CMT2 = more axonal) resulting in symmetrical distal sensorimotor neuropathy

Sensation: reduced, glove/stocking distribution (dorsal>spinoT)

Reflexes: reduced/absent reflexes.

Weakness: atrophy and weakness of the small muscles of the hand, LL distal muscle weakness & atrophy (inverted champagne bottle legs).

Pes cavus.

High stepping gait (foot drop, proprioceptive deficits)

Management:
Ankle-foot orthoses.

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2
Q

What are the motor functions of the median nerve?

A

Thumb opposition
Digits 1, 2, 3 flexion
Wrist flexion and abduction
Forearm pronation

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3
Q

What are the motor functions of the ulnar nerve?

A

Thumb + finger abduction
finger adduction
Digits 4-5 flexion
Wrist flexion and adduction

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4
Q

What are the branches of the sciatic nerve? What are there motor and sensory function?

A

Common peroneal:
Superficial peroneal - foot eversion; lateral foot + calf
Deep peronal - foot DF, toe extension; patch between toes 1 and 2

Tibial nerve: foot PF, inversion, toe flexion; lateral aspect of foot

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5
Q

Foot drop with loss of ALL foot movements, absent ankle jerk + weak knee flexion
Where is the lesion?

A

Sciatic neuropathy.

NOTE - peroneal nerve often more affected than tibial; may have sparing of PF despite weak AJ

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6
Q

Lower trunk plexopathy

  • signs
  • spinal levels
A

C8, T1

  • Claw hand
  • Horners
  • loss of movement of intrinsic muscles of the hand (T1)+ wrist and finger flexion (C8).
  • senosry loss of ulnar side of hand AND forearm
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7
Q

Upper trunk plexopathy

  • signs
  • spinal levels
A

“Erbs palsy”
C5,C6

  • loss of shoulder movements and elbow flexion = “waiters tip” - limp handing arm + internal rotation of the forearm + wrist and finger flexion.
  • sensory loss over lateral aspect of arm AND forearm
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8
Q

Causes of upbeat nystagmus

A

Pontine lesions - MS, stroke, tumor

Wernickes
Bilateral INO

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9
Q

Causes of downbeat nystagmus

A

Cervicomedullary junction lesions (arnold chiari malformations)
Cerebellar degeneration
Drug intoxication (PHY, CBZ)
Demyelination

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10
Q

Causes of proximal myopathy

A

Toxins/drugs - ETOH, steroids
Metabolic - Thyrotoxicosis, diabetic amyotrophy
Muscular - PM/DM, Mitochondrial myopathies, Muscular dystrophy (i.e. scapulohumeral dystrophy)

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11
Q

causes of distal myopathy

A

IBM (with quadriceps wasting + hand grip weakness)

Myotonic dystrophy

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12
Q

Features of arnold chiari malformation

A

Lower cranial neuropathies (9/10/11/12), myelopathy (compression of medulla + spinal chord)

Ataxia, dysmetria, downbeat nystagmus (cerebellar compression)

Headache, syringomyelia - central chord syndrome (CSF blockage)

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13
Q

4 differences between critical illness myopathy (CIM) and and polyneuroapathy (CIP)

A

Reflexes - preserved or mildy reduced in CIM, absent in CIP
Sensation - normal in CIM, reduced in CIP
Facial muscles - weak in CIM, cranial nerves perserved in CIP
NCS CIP: generalized axonal sensorimotor polyneuropathy with low motor AND sensory amplitudes in CIP
NCS CIM: low motor amplitudes and prolonged CMAP in CIM

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14
Q

Features of IBM

A

Older age (>50 typically)
Typically proximal + distal weakness
Weakness of wrist and finger flexors > extensors
Weakness of quadriceps and knee extensors > flexors
Hyporeflexia
Facial muscle weakness with oculomotor sparing
Muscle atrophy
Dysphagia

Ix - raised CK, muscle biopsy

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15
Q

Features of Adies syndrome

Where is the lesion?

A

Mydriatic pupil with impaired light reaction and intact (but slowed) accomodation

Often associated with hyporeflexia

Commonly affects females

Usually idiopathic but may be due to inflammation (inc. migraine) or infection

Lesion - efferent parasympathetic pathway

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16
Q

Features of Argyll Robertson pupil

4 causes

A

Miotic irregular pupils unreactive to light with intact accomodation

  1. Diabetes mellitus
  2. Neurosyphillis
  3. Alcoholic midbrain degeneration
  4. Midbrain lesions
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17
Q

9 causes of peripheral neuropathy

A

Drugs/toxins - amiodarone, chemo (vincristine, cisplatinum), anti-infective (isoniazid, nitrofurantoin), heavy metals, phenytoin, alcohol

Infiltrative - amyloidosis, tumor/paraneoplastic

Metabolic - diabetes, hypothyroidism, porphyria, uremia, B12 or B1 deficiency

Immune mediated - GBS (AMSAN, AIDP)

Connective Tissue disorder/vasculitis - SLE, polyarteritis nodosa, sjogrens

Hereditary (i.e. CMT)

Idiopathic

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18
Q

8 causes of predominantly sensory neuropathy

A

Infective - Syphyllis
Autoimmune - Sjogrens
Metabolic - B6 intoxication, B12 deficiency, Diabetes
Neoplastic - paraneoplastic (lung, ovary, breast), paraproteinemia
Idiopathic

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19
Q

6 causes of painful small fibre neuropathy

A
Diabetes
Alcohol
B12 or B1 deficiency
Carcinoma
Porphyria
Arsenic/thallium poisoning
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20
Q

Definition of synkinesis and what disorder is it seen in

A

Increased rigidity of one arm with voluntary movement of the other
Seen in parkinsons disease

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21
Q

8 causes of parkinonism

A

Idiopathic parkinsons disease
Parkinsons plus syndromes (PSP, MSA, CBD, LBD)
Toxic - Wilson’s disease, Drugs (antipsychotics, valproate, metoclopramide, methyldopa)
Ischemic - Hypoxic brain injury, Vascular parkinsons
Tumor - Basal ganglia tumors

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22
Q

8 parkinsons plus symptoms

A
Poor response to dopaminergic therapies
Early falls
Early hallucinations
Early autonomic dysfunction
Cognitive impairment within <1 year of symptom onset
Symmetry
Ocular signs
Cerebellar signs
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23
Q

Features differentiating essential tremor from parkinsons tremor

A

Symmetric
Worse with voluntary movement
Associated head tremor

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24
Q

8 causes of bilateral pes cavus

A
SCA
CMT
Hereditary spastic paraperesis 
Spinomuscular atrophy
Muscular dystrophies
Cerebral palsy
Spinal cord - Syringomyelia, tumors (can also cause unilateral)
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25
Q

4 causes of slowed saccades

A

PSP, Parkinsons disease (hypometric - “bunny hops”)
Huntingtons
Spinocerebellar ataxia II and VII
CPEO

26
Q

Cause of ocular dysmetria

A

Cerebellar disorders (“overshoot”)

27
Q

Which cranial nerves converge in the cerebellar pontine angle

A

CN V, VI, VII, VIII

28
Q

Which cranial nerves converge in the cavernous sinus

A

CN III, IV, V, VI

29
Q

Which cranial nerves converge in the jugular foramen

A

CN IX, X, XI, XII

30
Q

Features of bulbar palsy

Upper or lower motor neuron?

5 causes

A

Lower motor neuron

Palatal paralysis
Wasted tongue with fasciculations
Nasal speech

MND, MG, brainstem lesions (medullary stroke/tumor,), demyelination (i.e. GBS), Kennedy disease

31
Q

Features of pseudobulbar palsy

Upper or lower motor neuron?

5 causes

A

Upper motor neuron

Spastic tongue
Slow grunting speech
Brisk jaw jerk
Emotional lability

MND, PSP, parkinsons disease, MS, post-stroke

32
Q

What is “split hand”

A

Severe wasting of first dorsal interossei + abductor pollicis brevis (lateral hand)

Sparing of abductor digiti minimi (medial)

Indicative of MND –> dissociated muscle atrophy not respecting nerve trunk/roots as ADM + FDI innervated by ulnar nerve + C8/T1

33
Q

Causes of bilateral foot drop

A

Upper motor neuron signs

  • spinal chord lesions aboe L5 (look for a sensory level)
  • parasagittal tumor
  • if mixed –> think MND

Lower motor neuron signs

  • bilateral L5 radiculopathies
  • bilateral lumbosacral plexopathy
  • UNUSUAL causes –> bilateral sciatic (loss of AJ) or peroneal nerve lesions (inv + reflexes preserved)
  • polyneuropathy (look for associated peripheral neuropathy)
  • distal hereditary motor neuropathy (i.e. CMT)
34
Q

What is an RAPD and where is the lesion

A

RAPD - on swinging light test, the unaffected eye will constrict and when swinging to the affected pupil it will dilate

Indicates an optic nerve lesion of the dilating pupil

35
Q

If visual acuity improves with pin hole what does this indicate

A

Refractive error

36
Q

Changes in fundoscopy with diabetic retinopathy

A

Non-proliferative
- cotton wool spots (soft exudates), hard exudates, intraretinal hemorrhages

Proliferative
- neovascularization, fibrosis

37
Q

Changes in fundoscopy with hypertensive retinopathy

A

Silver lining (arteriole narrowing), AV nicking (mild)

Hemorrhages (flame/dot shaped), soft (cotton wool spots) + hard exudates, micronaueyryms (moderate)

Papilloedema, optic atrophy (pale disc) venous engorgement, blurring of disc margin (severe)

38
Q

Changes in fundoscopy with papilloedema

A

venous engorgement, blurring of disc margins

39
Q

One and a half syndrome

Where is the lesion?

A

Complete horizontal gaze palsy of one eye
Impaired Adduction of the other
May have an extropia of the contralateral eye

Lesion in paramedian pontine reticular formation

40
Q

Binocular vs monocular diplopia

A

Monocular diplopia doesn’t improve with contralateral eye closure - indicates refractive error (astigmatism, cataract)

Binocular diplopia improves with contralateral eye closure - indicates a nerve/neuromusclar/muscular problem

41
Q

What is a small fibre neuropathy?

A

Neuropathy affecting thinly myelinated alpha delta nerve fibres (cold, pain, autonomic) and unmyelinated C nerve fibres (warmth) with preservation of large calibre nerve fibres

42
Q

Causes of a small fibre (painful) neuropathy

A

Infective - HIV, leprosy

Toxins - heavy metals, drugs (isoniazid, cisplatin, metronidazole)

Autoimmune - sjogrens, primary biliary cirrhosis

Metabolic - diabetes, hypothyroidism,

Infiltrative - amyloidosis, sarcoid

43
Q

Cranial nerve VI palsy

A

Ipsilateral esotropia
Impaired abduction
Horizontal diplopia on ipsilateral lateral gaze that improves with cover testing

44
Q

CNIV palsy

A

Head tilt away from the affected eye
Affected eye elevated compared with left
Vertical binocular diplopia maximum on looking vertical and away from affected side

45
Q

Differential for an extraocular muscle palsy NOT fitting a specific cranial nerve lesion (i.e. MR palsy)

A

Neuromuscular - MG, miller fisher syndrome

Muscular - muscular dystrophy, myopathies (i.e. Graves), orbital myositis, trauma, CPEO (mitochondrial )

46
Q

INO vs MR palsy

A

INO - impaired adduction resolves when unaffected eye is covered

MR palsy - impaired adduction regardless

47
Q

Differentials for up gaze palsy

A

Central - Dorsal midbrain lesions (parinaud), PSP
Neuromuscular - MG, miller fisher
Muscular - muscular dystrophy, myopathies (i.e. Graves), CPEO

48
Q

Findings in chronic progressive external ophthalmoplegia

A

Bilateral ptosis
External ophthalmoplegia (typically symmetric)
Slowed saccades (horizontal + vertical)
May have associated reduced VA from retinal degeneration

Other - cerebellar ataxia, generalised muscle weakness

49
Q

4 patterns seen in wrist drop

A
  1. Corticospinal (pyramidal pattern weakness, UMN)
  2. C7/C8 plexus/root (radial deviation on attempt at wrist ext, triceps weak + reflex loss, finger flex/ext weak, sensory loss over C7/8)
  3. Radial nerve (brachioradialis weak + reflex lost, finger extension weak, sensory loss over snuff box)
  4. PIN branch (radial deviation on attempt at wrist ext, finger extension weak, BR + sensation intact)
50
Q

Gertman Syndrome

Where is the lesion?

A

Dominant (left) inferior parietal lobe (left angular gyrus)

Acalculia - can’t do math
Agraphia - can’t write
Agnosia (of the fingers) - can’t name fingers
Right-left disorientation

51
Q

Lateral medullar syndrome

Artery?

A

Ipsilateral horners

Ipsilateral bulbar symptoms (IX/X)

Ipsilateral facial spinothalamic sensory loss in spinothalamic tract with contralateral upper and lower limb spinothalamic sensory loss

Ipsilateral falling

Cereballr signs - ataxia, nystagmus diplopia

Artery = PICA

52
Q

Causes of a sensorimotor neuropathy

A

Vascular - vasculitis

Infective - cryglobulinemia (HCV)

Toxins/drugs - ALCOHOL, amiodarone, chemotherapy (vincristine, paclitaxel, cisplatin), nitrofurantoin

Autoimmune - CIDP, GBS

Metabolic - porphyria (often motor>sensory), DM, uraemia

Infiltrative - sarcoid, amyloid

Neoplastic - paraproteinemia

Congenital - CMT

53
Q

Causes of a bilateral cerebellar syndrome

A

Hereditary - SCA, friedrichs

Drugs/toxins - phenytoin, chronic ETOH

Metabolic - hypothyroidism

Structural - large occupying lesion (midline tumor = midline cerebellar syndrome), arnold chiari (look for CN 9/10/11/12 palsies + central cord syndrome)

Paraneoplastic

MS

54
Q

Causes of a unilateral cerebellar syndrome

A
Ischemia (vertebrobasilar disease)
Space occupying lesions (tumor, abscess, granuloma)
Paraneoplastic
MS
Trauma
55
Q

Anterior cord syndrome

3 causes

A

Spinothalamic sensory loss + UMN pattern of weakness below the level of the lesion
LMN pattern of weakness at the level of the lesion (ant. horn)
Urinary incontinence

Anterior spinal artery infarction, disc herniation, radiation myelopathy

56
Q

Posterior cord syndrome

3 causes

A

Dorsal column sensory loss + UMN pattern of weakness below level of the lesion
Ataxia
Urinary incontinence

Tabes dorsalis, B12 deficiency, cervical myelopathy

57
Q

Central cord syndrome

3 causes

A

Dissociated sensory loss (preservation dorsal column, spinothalamic lost)
Suspended sensory level (sensory loss at level of lesion)
UMN weakness (arms>legs) below level of lesion, LMN at level of lesion
Urinary retention

Syringomyelia, spinal cord tumors, cervical myelopathy

58
Q

Femoral neuropathy

A

Motor - weak hip flexion and knee extension

Reflexes - absent knee jerk

Sensation - loss over anterior and medial aspect of thigh extending to foot

59
Q

Optic neuropathy causes

A

Vascular - ischaemic optic neuropathy, vasculitis (GCA),
Infective - west nile, syphysillis, cat scratch
Traumatic + Compressive - optic nerve compression (orbital tumours or dysthyroid eye disease), asymmetric glaucoma
Autoimmune - optic neuritis (i.e. MS)
Metabolic - diabetes

60
Q

Testing for MG

A
repetitive nerve stimulation
single fiber EMG 
anti-AcHR
anti-MUSK
CT chest
61
Q

Findings bilateral internuclear ophthalmoplegia

Causes?

A
Bilateral exotropia ("Wall Eyes BINO syndrome)
Loss of adduction bilaterally

If ipsilateral abducens palsy –> 1 and a half syndrome (loss of horizontal one eye, loss of add other eye)

Etiologies:

  • mid brain lesion (space occupying lesion, ischemia)
  • demyelination (MS)
  • PSP
  • infection
  • wernickes encephalopathy