Neuromuscular Diseases Flashcards

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1
Q

signs of upper motor neuron lesions

A

weakness, paralysis, spasticity, hypereflexia, extensor plantar Babinski sign, loss of superficial abdominal reflexes, little if any muscle atrophy

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2
Q

signs of lower motor neuron lesions

A

weakness, paralysis, wasting and fasciculations, hypotonia/flaccidity, loss of tendon reflexes, normal abdominal and plantar reflexes

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3
Q

clinical presentation of peripheral nerve disease

A

numbness, impaired vibration perception, atrophy of small muscles of hands and feet, weakness, ataxia, pain, risk for charcot foot

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4
Q

major cranial nerve symptom of myasthenia gravis

A

diplopia that fluctuates throughout the day, worsens towards the end of the day

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5
Q

diagnostic test used to diagnose myasthenia gravis

A

tensilon test: inhibits ACh esterase

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6
Q

general principles of ALS

A

sensory is unaffected, random weakness, CNS involvement, hypereflexia

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7
Q

general principles of polyradiculoneuropathy

A

sensory impairment involved, distal weakness more than proximal, CNS involvement rare, hyporeflexia

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8
Q

general principles of myasthenia gravis

A

upper extremities more affected than LE, sensory impairment involved, weakness, variable CNS involvement, normal reflexes

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9
Q

general principles of myopathy

A

more proximal weakness than distal weakness, normal or declining CNS involvement, normal reflexes

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10
Q

risk factors for sporadic ALS

A

smoking! military, trauma, physical activity (soccer players), environmental toxins: cycad nuts in Guam

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11
Q

sites of motor neuron loss in ALS

A

cortex, brainstem, spinal cord

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12
Q

sensory or autonomic changes in ALS?

A

NO

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13
Q

a way that ALS can be fatal

A

motor neurons to diaphragm and chest muscles leads to respiratory distress

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14
Q

oral sign of ALS

A

atrophic tongue

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15
Q

ALS medication, what it does

A

Riluzole: antiglutamate agent; prolonged survival

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16
Q

diseases that can evolve into ALS

A

primary lateral sclerosis, progressive bulbar palsy, other motor neuron diseases

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17
Q

most common form of inherited motor neuron disease

A

spinal muscular atrophy (aut. recessive)

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18
Q

examples of mononeuropathy

A

carpal tunnel, peroneal palsy at the fibular head

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19
Q

large vs small sensory nerves: myelination level?

A

large sensory neurons are well myelinated; small sensory neurons are poorly myelinated or unmyelinated

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20
Q

function of large sensory neurons

A

relay vibration and proprioception info (dorsal column)

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21
Q

function of small sensory neurons

A

relay pain and temp- form spinothalamic tracts

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22
Q

symptoms of polyneuropathy

A

start in FEET (tripping), move proximally, hand symptoms appear when LE symptoms are up to the knee (trouble turning keys, opening jars), pins and needles, tingling, burning

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23
Q

autonomic symptoms of polyneuropathy

A

dry eyes, mouth, changes in sweat, orthostatic hypotension, bladder dysfunction, post prandial diarrhea, constipation, ED

24
Q

name this acute polyneuropathy: acute progressive neuropathy involving myelin of the peripheral nerves

A

Guillain-Barre syndrome

25
Q

this acute polyneuropathy involves axons of nerves; can present as psychiatric disorder or have unexplained GI symptoms

A

porphyria

26
Q

most common cause of rapidly progressing weakness, demyelination

A

Guillain-Barre syndrome

27
Q

Guillain-Barre syndrome has this type of weakness and this type of hyporeflexia; what type of sensory neuron is lost?

A

ascending type weakness, symmetric areflexia, large fiber sensory loss

28
Q

causes of death due to Guillain-Barre syndrome

A

respiratory failure, autonomic instability, DVT, infection

29
Q

most common autoimmune disease involving post synaptic ACh receptors

A

myasthenia gravis

30
Q

pathology of myasthenia gravis

A

less number of receptors; weakness; ACh goes right to ACh esterase

31
Q

50% of myasthenia gravis presenting symptoms is..

A

ocular symptoms (ptosis, diplopia)

32
Q

weakness in myasthenia gravis patients is described as..

A

fatigueable weakness

33
Q

the antibody found is myasthenia gravis functions to..

A

bind, block, modulate the ACh receptor

34
Q

extremity weakness in myasthenia gravis: which is greater, legs or arms?

A

arms > leg weakness

35
Q

treatment of myasthenia gravis

A

ACh esterase inhibitors, plasma exchange, IV Ig’s, thymectomy, immunosuppressants

36
Q

botulism involves which part of the NMJ?

A

presynaptic part

37
Q

pathology of lambert eaton syndrome

A

voltage gated Ca2+ channel antibodies impede the release of ACh

38
Q

cancer associated with lambert eaton syndrome and calcium channel antibodies*

A

lung cancer, or possibly any underlying cancer. antibodies to the lung cancer result in weakness of LE, hyporeflexia

39
Q

the dystrophy muscle diseases

A

Duchenne’s MD, myotonic dystrophy

40
Q

the congenital myopathies

A

glycogenoses, mitochondrial

41
Q

the acauired myopathies

A

polymyositis, dermatomyositis, inclusion body myositis, drug related

42
Q

Duchenne’s MD is cause dby..

A

absence of dystrophin protein/ its X-linked recessive

43
Q

clinical sign of duchenne’s muscular dystrophy

A

Gower’s maneuver: getting off the floor by placing hands and feet on the floor together first

44
Q

most common form of adult dystrophy

A

myotonic dystrophy

45
Q

describe the myotonia of myotonic dystrophy

A

failure to relax muscle after contraction (hands get stuck)

46
Q

1/3 of all cases of inflammatory myopathies are..

A

polymyositis

47
Q

30% of polymyositis patients experience

A

cardiac disturbance: conduction issues, tachyarrhythmia, CHF

48
Q

treatment of polymyositis

A

immunosuppression: prednisone, methotrexate, azathioprine

49
Q

*distinctions for dermatomyositis

A

more rapid onset, very high CPK’s (creatinine phosphokinase), acute renal failure, skin changes that usually appear first

50
Q

dermatomyositis is also this type of syndrome..

A

paraneoplastic

51
Q

the most common idiopathic inflammatory myopathy in adults

A

inclusion body myositis

52
Q

typical pattern of weakness of inclusion body myositis

A

asymmetric, wrist and finger flexors, quadriceps

53
Q

inclusion body myositis patients present with this 40-60% of the time

A

dysphagia (more than dermatomyositis)

54
Q

Duchenne’s MD CK levels are ____ that of normal

A

100 times

55
Q

this family of drugs can cause myopathy

A

cholesterol agents (statins)

56
Q

factors that determine whether or not to stop administering statins causing myopathy

A

if symptoms are intolerable, if CPK > 10 x, if elevation of BUN develops