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Phase 3A Neurology > Neuropathy/myopathy > Flashcards

Neuropathy/myopathy Flashcards

1
Q

Antibodies

a) Miller-Fisher
b) MMN with conduction block
c) Neuromyelitis optica
d) MG
e) LEMS

A

a) Anti- GQ1b
b) Anti- GM1
c) Anti- AQP-4
d) Anti- nAChR
e) Anti- voltage-gated presynaptic calcium channel

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2
Q

In patients with neuromuscular disease, what serial tests must be performed?

A
  • Spirometry - FVC is particularly important

- may also do ABG

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3
Q

Scan to perform in:

a) LEMS
b) MG
c) GBS (if autonomic involvement)

A

a) CT thorax (SSLC)
b) CT thorax (thymoma)
c) ECG

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4
Q

Curative management of:

a) MG - acute, symptomatic, long-term
b) GBS - acute

A

a) - Acute/severe: IVIG or Plasmapheresis.
- Symptomatic: pyridostigmine.
- Long-term: DMARDs, or intermittent infusions of IVIG

b) IVIG or plasmapheresis
(note: steroids do NOT help recovery)

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5
Q

Supportive care for neuromuscular diseases (eg. MG, MND, GBS, muscular dystrophies)

A
  • Airway and breathing - intubate and ventilate (NIV) if necessary
  • Circulation - BP control (hypo - fluid bolus, hyper - labetalol/nitroprusside
  • DVT prophylaxis, prevent pressure sores
  • Pain management - gabapentin for neuropathic pain
  • Rehabilitation - physio, SALT, mobilise
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6
Q

GBS: diagnosis

a) 2 clinical features required
b) Supporting clinical features
c) Investigations and findings
d) What syndrome may coexist?
e) What are the commonest causes of death in GBS?
f) Prognosis

A

a) Weakness and areflexia

b) - Onset over days to 4 weeks
- Recovery within weeks of progression stopping (if longer course, may be CIDP)
- Symmetrical, sensorimotor, CNs affected
- Autonomic dysfunction
- Preceding GI/resp infection (eg. campylobacter, EBV)

c) - U+Es (rule out differentials; may have SIADH)
- Antibody screen (eg. anti-GQ1b)
- LP: elevated CSF protein with no cell count elevation
- NCS: features of nerve conduction slowing or block
- Spirometry: low FVC predicts need for ITU admission and intubation/ventilation
- ECG: heart block, other arrhythmias

d) SIADH

e) - Respiratory failure
- Pulmonary embolism
- Pneumonia

f) ~ 75% recover fully
~ 20% have some persisting disability
~ 5% mortality

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7
Q

Peripheral neuropathy screen
a) Initial tests: FLUTE FUR

b) Others depending on the clinical scenario (go through VITAMIN C DEF)

A

a) FBC, Liver, U+E, Thyroid, ESR.
Folate (and B12), Urine (protein, glucose, ?toxicology, ?Bence Jones) Renal, Serology (coeliac, syphillis/HIV)

b) +/-Vasculitic screen, +/-EMG/NCS, +/-CSF study, +/-nerve biopsy

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8
Q

Differentiating Bell’s palsy from Ramsay-Hunt syndrome

A

RHS - pain, shingles rash

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9
Q

Myopathy (stair, chair and hair) causes:

a) Inherited
b) Inflammatory
c) Infective
d) Metabolic
e) Toxic
f) Drug-induced

A

a) Dystrophies, mitochondrial disease, glycogen storage

b) Poly/dermatomyositis, IBM, SLE, vasculitis;
PMR and RA (more pain than weakness)

c) HIV, toxoplasmosis, Coxsackie viruses, influenza, Lyme disease
d) Hyper/hypothyroidism, Cushing’s, diabetes, hypo/hyperparathyroidism, electrolyte disturbances (hypercalcaemia, hypokalaemia)
e) Alcoholic myopathy with myoglobinuria, paraneoplastic myopathy, protein malnutrition
f) Steroids, statins, zidovudine, clofibrate, colcichine

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10
Q

Two clinical syndromes where signs appear before symptoms

A

Myelopathy

Peripheral neuropathy

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11
Q

3rd nerve palsy:

a) What is the earliest sign in raised ICP? Why?
b) What is it compressed against?
c) Classical cause of pupil-sparing 3rd nerve palsy?

A

a) Fixed, dilated pupil. PSNS fibres on outside of the nerve
b) Apex of petrous part of temporal bone

c) Diabetes, other microvascular;
- Motor fibres are more central in the nerve axon - affected more by vascular injury (vs. compression of outer PSNS fibres)

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12
Q

Brain vs. spinal lesion:

- differentiating rules of thumb (pain, weakness, etc.)

A

Brain - headache, visual field defect, contralateral weakness

Spine - back pain, radiating leg pain, bilateral weakness

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13
Q

Spinal nerve roots: dermatome, myotome, +/- reflex

a) C5/C6
b) C7
c) C8/T1
d) L5
e) S1

A

a) Reg badge/thumb, shoulder abduction/elbow flexion, pronator/biceps reflex
b) Middle finger, elbow extension, triceps reflex
c) Medial hand/forearm, finger flexion/abduction
d) Big toe and dorsum, dorsiflexion
e) Little toe and sole, plantarflexion, calcaneal reflex

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14
Q

Three reasons to refer to neurosurgeon in context of back pain

A
  • CES
  • Progressive or severe neurological deficits
  • Failure of conservative/medical management
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15
Q

Abnormal posturing:

a) DeCorticate - Explain. GCS motor score? Lesion site?
b) DecerEbrate - Explain. GCS motor score? Site?

A

a) Flexion towards Cord (C-shaped):
- lesion in Cortex or C-spine
- GCS motor score = 3

b) Extension
- Lesion in midbrain/pons (brainstem - more severe)
- GCS motor score = 2

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16
Q

Radiculopathies.

a) Differentiating between L5 and common peroneal nerve palsy
b) A C6/C7 disc would cause what root lesion?
c) Patellar reflex - tests what roots?

A

a) L5 - more severe (weak foot inversion and hip abduction)
b) C7
c) L2 - L4

17
Q

3 gold standard tests for a myopathy

- 1 extra if dystrophy suspected

A

CK, EMG, muscle biopsy

Genetic testing

18
Q

Causes of proximal myopathy.

A 
Drugs eg steroids, statins
Low vitamin D
Cushings/ hypothyroid
Alcohol 
Other?
19
Q

Myasthenia gravis (MG).

a) Clinical features (symptoms, and signs o/e)
b) Examination findings
c) Initial investigations
d) Management
e) Main differential and management
f) Myasthenic crisis - clinical features, criteria and management

A

a) - Ocular symptoms: ptosis, diplopia
- Bulbar/facial muscles: dysarthria, facial droop, dysphagia
- Proximal myopathy
- Fatiguability - symptoms worse at end of day

b) Ice test.
- Crushed ice applied to eye for 3 minutes leads to improvement of ptosis in 90% patients

Fatiguability.

  • Focus on finger above forehead level (cannot sustain for long due to extraocular muscle weakness)
  • Count up to 50 (voice gets quieter)

Neurological.
- NO wasting, fasciculations or sensory loss; reflexes and tone are normal

c) - Bedside: FVC and negative inspiratory force
- Autoantibody screen: ACh-R (+ MuSK if ACh-R negative)
- Nerve conduction studies (normal)
- CT thorax (Thymoma)
- Edrophonium (Tensilon) test:
IV administration of short-acting cholinesterase inhibitor leads to transient improvement in symptoms; rarely done now due to risk of life-threatening bradycardia

d) Symptomatic:
- cholinesterase inhibitor (pyridostigmine)

Immune suppression:

  • steroid, azathioprine
  • plasmapheresis / IVIg
  • Thymectomy

e) LEMS: usually associated with SCLC, treat the cause

f) Clinical features:
- Increasing SOB and use of accessory muscles (inspiratory weakness)
- Weak cough (expiratory weakness)
- ABG - hypercapnia (T2RF)

Criteria:

  • FVC < 1 L, or
  • negative inspiratory force (NIF) < 20 cm H2O

Management:
- Intubation and ventilation

20
Q

Syringomyelia

a) What is it?
b) Causes
c) Typical presentation
d) If extending into brainstem - known as…?
e) What are Chiari malformations 1 and 2?

A

a) Tubular cyst (syrinx) within the central spinal cord (usually cervical);

b) CSF blockage:
- Main cause = Chiari malformation (note: type 2 malformations are associated with spina bifida)
- Other causes - SOL, arachnoiditis (following trauma, infection, inflammation, etc.)

c) - Usually presents in 20s - 30s
- Pain and temperature loss in cape distribution (upper chest and arms) - may lead to Charcot joints
- Later, affects vibration, light touch and motor nerves (generally LMN signs as affecting the anterior horn cells)
- Rarely, causes autonomic disturbance (eg. Horner’s) and bladder/bowel dysfunction

d) Syringobulbia
- Can cause CN palsies

e) - Type 1: cerebellar tonsils extend into spinal canal
- Type 2: cerebellar tonsils + brainstem + IVth ventricle

f) - Peripheral neuropathy screen
- MRI - shows syrinx +/- associated cause (eg. Chiari malformation)
- Do NOT do an LP - risk of coning

g) - Physio
- Symptom management - eg. neuropathic analgesics
- Surgery is only curative treatment

21
Q

Footdrop: causes

A
  • Central: ?MS, ?CVA
  • Nerve root: L4/L5 lesion? (sparing of eversion?)
  • Peripheral nerve: common peroneal nerve lesion (causes:
  • Muscle: tibialis anterior
22
Q

CMT.

a) What is it?
b) Classic pnset and features
c) Signs o/e
d) Investigations and findings
e) Management
f) What other inherited disease causes progressive sensory ataxia, ascending weakness and cardiac arrhythmias/ cardiomyopathy?

A

a) A group of inherited (usually autosomal dominant) sensorimotor neuropathies - caused by production of abnormal myelin and subsequent demyelination

b) - Onset usually in childhood
- Muscle weakness and wasting; distal muscles first (intrinsic muscles of foot), then hands, legs, thighs, etc.
- Glove and stocking sensory neuropathy; often causes sensory ataxia and steppage gait

c) Inspection.
- Pes cavus, hammer toes, finger curling, contractures, scoliosis
- Wasting of distal muscles
- Inverted champagne bottle leg appearance

Neurological.

  • Gait - steppage common-
  • Glove and stocking sensory neuropathy
  • Areflexia (LMN signs)
  • Peripheral weakness

d) - Peripheral neuropathy screen (FLUTE FURS)
- NCS - slowing (demyelination), reduced amplitude (axonal loss), conduction block (severe demyelination)
- ?genetic testing and nerve biopsy if uncertain

e) No cure. Symptomatic MDT management:
- Pain - especially neuropathic analgesia
- PT - tendon stretches, muscle strengthening, etc.
- Orthotic devices - ankle-foot orthoses, splints, etc.
- Orthopaedic surgery for contractures, scoliosis, etc.
- Genetic counselling

f) Friedreich’s Ataxia

23
Q

MS.

A

-

Phase 3A Neurology (9 decks)

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