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Block 9 - Neuro > CM: Neuromuscular Dz > Flashcards

CM: Neuromuscular Dz Flashcards

1
Q

What does a lesion in a UMN cause?

A

weakness, increased tone (spasticity), hyperreflexia

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2
Q

What does a lesion in a LMN cause?

A

weakness, hypotonia, hyporeflexia, atrophy, fasciculations

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3
Q

What general characteristics characterize myopathies?

A

symmetric proximal weakness with normal sensory fxn

reflexes normal or decreased in proportion to weakness

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4
Q

What tests can be used to evaluate the NM system?

A

electrodiagnostic testing can determine which part of unit is damaged
test blood for Abs or genetic abnormalities
muscle or nerve biopsy

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5
Q

Injuries to the corticospinal tracts have effects where?

A

if above medullary pyramids - contralateral side

below - ipsilateral side

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6
Q

What diagnostic tests might be necessary with myopathies?

A

EMG, CK, aldolase, Ab with connective tissue disorders, muscle biopsy

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7
Q

What are general features of inflammatory myopathies?

A

slowly progressive symmetric proximal weakness
ocular muscles spared, but dysphagia common
may have pain or tenderness

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8
Q

What are distinctive features that can differentiate dermatomyositis from polymyositis?

A

dermato - skin rash (over eyelids, knuckles)

poly - associated w other connective tissue disorders

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9
Q

What are features common to dermatomyositis and polymyositis?

A

increased risk of cancer (dermato more)
high CK levels (>1000)
EMG w short small motor units w early recruitment

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10
Q

What is Gowers’ manuever?

A

children get on hands and knees, elevate posterior,then walk hands up to legs to raise upper body
seen in Duchenne

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11
Q

What is myotonic dystrophy?

A

AD, CTG repeat on ch 19 - shows anticipation, inherited from mom = imprinting
most common in adults
facial, neck and distal extremity weakness (hatchet face = ptosis, long thin, temporal wasting)
inability to relax muscles after sustained contraction or percussion

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12
Q

What is facioscapulohumeral dystrophy?

A

can be mild and progress slowly, may develop foot drops and leg weakness, CK normal or slightly elevated, EMG shows myopathic features

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13
Q

What is oculopharyngeal dystrophy?

A

onset after 50, ptosis and dysphagia

AD, defective PABPN1 gene causes protein to clump together in muscle cell nuclei, interferes w fxn

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14
Q

What does the evaluation of metabolic myopathies include?

A

ischemic forearm exercise testing - doesn’t show increase in lactate in pts w glycogen disorders

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15
Q

What is an example of a metabolic myopathy?

A

McArdle’s = disorder of glycogen

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16
Q

What symptoms can accompany mitochondrial myopathies?

A

retinitis pigmentosa, cardiac conduction defects, short stature, seizures or strokes
serum and CSF lactic acid levels elevated
muscle biopsy shows ragged red fibers

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17
Q

What is Kearns-Sayre syndrome?

A

mitochondrial dz associated w progressive external opthalmoplegia, retinitis pigmentosa and heart block
unilateral ptosis becomes bilateral
lactate and pyruvate elevated

18
Q

What are MELAS and MERRF?

A

mitochondrial ecephalomyopathy, lactic acidosis, stroke
myoclonic epilepsy and ragged red fibers
mitochondrial dizs associated w myopathy

19
Q

What is myasthenia gravis?

A

immune mediated - Abs against postsynaptic Ach receptor, usually progressive

20
Q

What are conditions associated w myasthenia gravis?

A

thymoma, thymic hyperplasia

other autoimmune conditions

21
Q

What are signs and symptoms of myasthenia gravis?

A

fatigue fluctuating throughout day
symptoms progress as day goes on
prolonged use of muscles worsens, rest improves
proximal muscle weakness, ocular symptoms, dysphagia
no sensory involvement

22
Q

What can help diagnose myasthenia gravis?

A

CMAP declines in amplitude in MG w/i first 4-5 stimuli, single fiber jitter on EMG, Ab, tensilon test results in temporarily restored muscle fxn

23
Q

What is Lambert-Eaton myasthenic syndrome?

A

weakness due to Abs blocking voltage gated Ca channels on presynaptic side of NMJ
DTR diminished or absent, but may be elicited after brief exercise
*postexercise or postactivation facilitation of CMAPs
paraneoplastic and autoimmune forms seen

24
Q

How is LEMS different than MG?

A

never starts w ocular involvement, MG can

LEMS more common in men, MG presents earlier in women

25
Q

What other conditions are associated w LEMS?

A

small cell lung cancer

26
Q

How do pts w peripheral neuropathy present?

A

distal symmetric sensory complaints first
feet first, then hands and knees
sensory and motor findings on exam
ankle reflexes absent first

27
Q

What diagnostic tests should be done with peripheral neuropathies?

A

EMG/NCV to see if axonal or demyelinating features present

based on findings, lab tests

28
Q

What is Guillain-Barre syndrome?

A

acute immune demyelinating polyneuropathy that causes ascending weakness over days to weeks - postinfectious targeting gangliosides
may also have back pain and paresthesias
variants have opthalmoplagia and ataxia or pure motor nerve damage
decreased DTR

29
Q

peripheral neuropathies are commonly ________

A

axonal

30
Q

What are pts with Guillain-Barre at risk for?

A

respiratory failure and dysautonomia as weakness ascends

31
Q

What does testing in pts with Guillain-Barre show?

A

albuminocytological dissociation = normal WBC w elevated total protein in CSF
nerve conduction velocities slowed early on and only after >7 days do changes reflect demyelination

32
Q

What is chronic inflammatory demyelinating polyradiculoneuropathy (CIDP)?

A

chronic form of Guillain-Barre - consider when pt w GBS relapses

33
Q

What endocrine disorders are associated w neuropathies?

A

diabetes, thyroid dz

34
Q

What connective tissue dzs are associated w neuropathies?

A

SLE,SS, RA

35
Q

What malnutrition syndromes are associated w neuropathies?

A

alcoholic neuropathy, vit B12 def

36
Q

What are some drugs that can cause neuropathy?

A

antineoplastic agents: cisplatin, vincristine
Antimicrobials: isoniazid
Antivirals: anything ending in -dine
phenytoin

37
Q

What are examples of motor neuron dzs where there is UMN signs alone or LMN signs alone?

A

UMN: primary lateral sclerosis (PLS)
LMN: progressive muscular atrophy (PMA), polio (ant horn cells)

38
Q

Where is there degeneration and loss of motor neuron cells in ALS?

A

cerebral cortex, brainstem motor nuclei, ant horn cells

39
Q

What bulbar symptoms can be seen in ALS?

A 
bulbar symptoms (LMN) due to facial, palatal or tongue weakness: slurred speech, hoarseness, dysphonia, dysphagia
psuedobulbar palsy (UMN): emotional lability
40
Q

What causes ALS?

A

cause unknown

might be SOD1 (super oxide dismutase) or mutation on ch 9

Block 9 - Neuro (26 decks)

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