Neuromuscular Diseases

Question 1

signs of upper motor neuron lesions

Answer 1

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

Question 2

signs of lower motor neuron lesions

Answer 2

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

Question 3

clinical presentation of peripheral nerve disease

Answer 3

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

Question 4

major cranial nerve symptom of myasthenia gravis

Answer 4

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

Question 5

diagnostic test used to diagnose myasthenia gravis

Answer 5

tensilon test: inhibits ACh esterase

Question 6

general principles of ALS

Answer 6

sensory is unaffected, random weakness, CNS involvement, hypereflexia

Question 7

general principles of polyradiculoneuropathy

Answer 7

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

Question 8

general principles of myasthenia gravis

Answer 8

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

Question 9

general principles of myopathy

Answer 9

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

Question 10

risk factors for sporadic ALS

Answer 10

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

Question 11

sites of motor neuron loss in ALS

Answer 11

cortex, brainstem, spinal cord

Question 12

sensory or autonomic changes in ALS?

Answer 12

NO

Question 13

a way that ALS can be fatal

Answer 13

motor neurons to diaphragm and chest muscles leads to respiratory distress

Question 14

oral sign of ALS

Answer 14

atrophic tongue

Question 15

ALS medication, what it does

Answer 15

Riluzole: antiglutamate agent; prolonged survival

Question 16

diseases that can evolve into ALS

Answer 16

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

Question 17

most common form of inherited motor neuron disease

Answer 17

spinal muscular atrophy (aut. recessive)

Question 18

examples of mononeuropathy

Answer 18

carpal tunnel, peroneal palsy at the fibular head

Question 19

large vs small sensory nerves: myelination level?

Answer 19

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

Question 20

function of large sensory neurons

Answer 20

relay vibration and proprioception info (dorsal column)

Question 21

function of small sensory neurons

Answer 21

relay pain and temp- form spinothalamic tracts

Question 22

symptoms of polyneuropathy

Answer 22

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

Question 23

autonomic symptoms of polyneuropathy

Answer 23

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

Question 24

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

Answer 24

Guillain-Barre syndrome

Question 25

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

Answer 25

porphyria

Question 26

most common cause of rapidly progressing weakness, demyelination

Answer 26

Guillain-Barre syndrome

Question 27

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

Answer 27

ascending type weakness, symmetric areflexia, large fiber sensory loss

Question 28

causes of death due to Guillain-Barre syndrome

Answer 28

respiratory failure, autonomic instability, DVT, infection

Question 29

most common autoimmune disease involving post synaptic ACh receptors

Answer 29

myasthenia gravis

Question 30

pathology of myasthenia gravis

Answer 30

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

Question 31

50% of myasthenia gravis presenting symptoms is..

Answer 31

ocular symptoms (ptosis, diplopia)

Question 32

weakness in myasthenia gravis patients is described as..

Answer 32

fatigueable weakness

Question 33

the antibody found is myasthenia gravis functions to..

Answer 33

bind, block, modulate the ACh receptor

Question 34

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

Answer 34

arms > leg weakness

Question 35

treatment of myasthenia gravis

Answer 35

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

Question 36

botulism involves which part of the NMJ?

Answer 36

presynaptic part

Question 37

pathology of lambert eaton syndrome

Answer 37

voltage gated Ca2+ channel antibodies impede the release of ACh

Question 38

cancer associated with lambert eaton syndrome and calcium channel antibodies*

Answer 38

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

Question 39

the dystrophy muscle diseases

Answer 39

Duchenne's MD, myotonic dystrophy

Question 40

the congenital myopathies

Answer 40

glycogenoses, mitochondrial

Question 41

the acauired myopathies

Answer 41

polymyositis, dermatomyositis, inclusion body myositis, drug related

Question 42

Duchenne's MD is cause dby..

Answer 42

absence of dystrophin protein/ its X-linked recessive

Question 43

clinical sign of duchenne's muscular dystrophy

Answer 43

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

Question 44

most common form of adult dystrophy

Answer 44

myotonic dystrophy

Question 45

describe the myotonia of myotonic dystrophy

Answer 45

failure to relax muscle after contraction (hands get stuck)

Question 46

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

Answer 46

polymyositis

Question 47

30% of polymyositis patients experience

Answer 47

cardiac disturbance: conduction issues, tachyarrhythmia, CHF

Question 48

treatment of polymyositis

Answer 48

immunosuppression: prednisone, methotrexate, azathioprine

Question 49

*distinctions for dermatomyositis

Answer 49

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

Question 50

dermatomyositis is also this type of syndrome..

Answer 50

paraneoplastic

Question 51

the most common idiopathic inflammatory myopathy in adults

Answer 51

inclusion body myositis

Question 52

typical pattern of weakness of inclusion body myositis

Answer 52

asymmetric, wrist and finger flexors, quadriceps

Question 53

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

Answer 53

dysphagia (more than dermatomyositis)

Question 54

Duchenne's MD CK levels are ____ that of normal

Answer 54

100 times

Question 55

this family of drugs can cause myopathy

Answer 55

cholesterol agents (statins)

Question 56

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

Answer 56

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