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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
4 causes of slowed saccades
PSP, Parkinsons disease (hypometric - "bunny hops") Huntingtons Spinocerebellar ataxia II and VII CPEO
26
Cause of ocular dysmetria
Cerebellar disorders ("overshoot")
27
Which cranial nerves converge in the cerebellar pontine angle
CN V, VI, VII, VIII
28
Which cranial nerves converge in the cavernous sinus
CN III, IV, V, VI
29
Which cranial nerves converge in the jugular foramen
CN IX, X, XI, XII
30
Features of bulbar palsy Upper or lower motor neuron? 5 causes
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
Features of pseudobulbar palsy Upper or lower motor neuron? 5 causes
Upper motor neuron Spastic tongue Slow grunting speech Brisk jaw jerk Emotional lability MND, PSP, parkinsons disease, MS, post-stroke
32
What is "split hand"
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
Causes of bilateral foot drop
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
What is an RAPD and where is the lesion
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
If visual acuity improves with pin hole what does this indicate
Refractive error
36
Changes in fundoscopy with diabetic retinopathy
Non-proliferative - cotton wool spots (soft exudates), hard exudates, intraretinal hemorrhages Proliferative - neovascularization, fibrosis
37
Changes in fundoscopy with hypertensive retinopathy
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
Changes in fundoscopy with papilloedema
venous engorgement, blurring of disc margins
39
One and a half syndrome Where is the lesion?
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
Binocular vs monocular diplopia
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
What is a small fibre neuropathy?
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
Causes of a small fibre (painful) neuropathy
Infective - HIV, leprosy Toxins - heavy metals, drugs (isoniazid, cisplatin, metronidazole) Autoimmune - sjogrens, primary biliary cirrhosis Metabolic - diabetes, hypothyroidism, Infiltrative - amyloidosis, sarcoid
43
Cranial nerve VI palsy
Ipsilateral esotropia Impaired abduction Horizontal diplopia on ipsilateral lateral gaze that improves with cover testing
44
CNIV palsy
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
Differential for an extraocular muscle palsy NOT fitting a specific cranial nerve lesion (i.e. MR palsy)
Neuromuscular - MG, miller fisher syndrome | Muscular - muscular dystrophy, myopathies (i.e. Graves), orbital myositis, trauma, CPEO (mitochondrial )
46
INO vs MR palsy
INO - impaired adduction resolves when unaffected eye is covered MR palsy - impaired adduction regardless
47
Differentials for up gaze palsy
Central - Dorsal midbrain lesions (parinaud), PSP Neuromuscular - MG, miller fisher Muscular - muscular dystrophy, myopathies (i.e. Graves), CPEO
48
Findings in chronic progressive external ophthalmoplegia
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
4 patterns seen in wrist drop
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
Gertman Syndrome Where is the lesion?
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
Lateral medullar syndrome Artery?
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
Causes of a sensorimotor neuropathy
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
Causes of a bilateral cerebellar syndrome
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
Causes of a unilateral cerebellar syndrome
``` Ischemia (vertebrobasilar disease) Space occupying lesions (tumor, abscess, granuloma) Paraneoplastic MS Trauma ```
55
Anterior cord syndrome 3 causes
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
Posterior cord syndrome 3 causes
Dorsal column sensory loss + UMN pattern of weakness below level of the lesion Ataxia Urinary incontinence Tabes dorsalis, B12 deficiency, cervical myelopathy
57
Central cord syndrome 3 causes
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
Femoral neuropathy
Motor - weak hip flexion and knee extension Reflexes - absent knee jerk Sensation - loss over anterior and medial aspect of thigh extending to foot
59
Optic neuropathy causes
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
Testing for MG
``` repetitive nerve stimulation single fiber EMG anti-AcHR anti-MUSK CT chest ```
61
Findings bilateral internuclear ophthalmoplegia Causes?
``` 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

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