Neuropathy, Myopathy, and Motor Neuron Disease Flashcards

1
Q

mononeuropathy

A

involvement of a single major named nerve usually by trauma or compression (median n at wrist, ulnar n at elbow, common peroneal n at fibular head)

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2
Q

polyneuropathy (peripheral neuropathy)

A

d/o of multiple major and small nerves or branches

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3
Q

what happens in the most common polyneuropathies

A

s/s are symmetrical and sensory loss or impairment occurs early and often remains prominent

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4
Q

where does numbness and tingling usually befin

A

distally in the toes and feet, later affecting the fingers and hands

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5
Q

what happens if autonomic nerves are involved

A
  • orthostatic hypotension
  • incontinence
  • impotence
  • sweating abnormalities
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6
Q

what are the basic pathological processes in neuropathy?

A

demyelination and axonal degeneration

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7
Q

demyelination

A
  • characterizes a mononeuropathy from focal compression such as carpal tunnel syndrome where median n is compressed at the wrist
  • also primary process of G-Barre (acute polyneuropathy)
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8
Q

role of axonal degeneration

A

-if neuropathy progresses over time or is initially several axonal degen may also occur with less chance for recovery

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9
Q

Wallerian degeneration

A

when there is a more severe crush or penetrating focal nerve injury, axonal loss occurs via wally
-axons and myelin degenerate distal to the point of nerve injury

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10
Q

polyneuropathies from toximetabolic causes

A

mostly have axonal degeneration as the primary pathology

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11
Q

multiple mononeuropathy

A

can be d/t systemic illness which is inflamm/ai (SLE), infiltrative (sarcoidosis), or infectious (leprosy)

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12
Q

causes of polyneuropathy

A
  • meds (chemo)
  • neurotoxins
  • alcoholism
  • malnutrition and vitamin deficiencies
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13
Q

role of EMG in diagnosis

A

-tests the electrical activity and function of nerves and muscles

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14
Q

sural (sensory nerve) biopsy

A
  • used when the clinical pic or EMG suggests a chronic polyneuropathy d/t an inflamm, immune-mediated, or vasculitic cause
  • leaves permanent numbness along lateral foot
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15
Q

oral medications for neuropathic pain

A
  • anticonvulsants (Gabapentin, Pregabalin, Carbamazepine)

- antidepressants (Duloxetine, Amitriptyline)

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16
Q

Guillain-Barre

A
  • one of the most rapidly progressive polyneuropathies
  • usually s/p recent viral illness
  • immune system targets peripheral nerve myelin
  • ascending areflexic paralysis (lower limbs affected first)
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17
Q

how can you hasten recovery for G-B

A

plasmapheresis or IVIG infusion

18
Q

what is one of the most common chronic polyneuropathies (occurs over months to yrs)

A

DM-which may also cause:

  • isolated or multiple mononeuropathies
  • autonomic neuropathies (diabetic gastroparesis or orthostatic hypoTN)
  • cranial neuropathies (diabetic third nerve palsy)
19
Q

what are some other causes of polyneuropathies?

A
  • metabolic or endocrine disorders
  • rheumatologic disease
  • cancer or myeloma
  • infection
  • nutritional deficiencies
  • toxins
20
Q

characteristics of most myopathies

A

-proximal weakness or fatigue, nml sensation, and late loss of reflexes after significant atrophy has occurred

21
Q

CK creatine kinase

A

muscle enzyme often nonspecifically elevated in disease of muscle

22
Q

polymyositis

A

inflammation of multiple muscle, may be d/t infections or drug reactions
-refers to ai dz affecting muscle

23
Q

dermatomyositis

A

-both skin (rash involving periorbital areas and knuckles) and muscle are involved

24
Q

Duchenne’s (X-linked) muscular dystrophy

A

deficiency of muscle dystrophin (important structural protein)

  • Gower’s manuever
  • calf muscles: pseudohypertrophy (fat and connective tissue)
25
Q

myotonic dystrophy type 1 (AD)

A
  • d/t excessive trinucleotide DNA repeats on c. 19; producing an abnml protein kinase in muscle fibers
  • weakness affects the distal limbs, neck, face, and jaws
26
Q

myotonia

A
  • peculiar impaired relaxation of muscle after volitional contraction
  • difficulty letting go of handshake or doorknob
  • also: cataracts, frontal baldness, infertility, cardiac arrhythmias
27
Q

UMN (CS+CB)

A

-UMN comprise the corticospinal tract (control anterior horn cells) and corticobulbar tract (CN motor nuclei-LMN)

28
Q

BULB

A

-refers to lower brain stem, location of the CN motor nuclei that innervate muscles of the jaws, face, palate, pharynx, larynx, and tongue

29
Q

spinal muscular atrophy

A
  • group of disorder involving just anterior horn cells
  • clinical findings: LMN signs
  • Werdnig-Hoffman=example of infantile onset
30
Q

primary lateral sclerosis

A
  • familial degeneration of corticospinal tract in lateral columns of the spinal cord not due to structural (spinal stenosis from degenerative arthritis) or metabolic (vit b12 deficiency) lesions
  • weakness + UMN signs
31
Q

pseudobulbar palsy

A

-encompasses several d/o where only the corticobulbar tract is involved, causing facial wealness, impaired chewing dysarthria, dysphagia and hoarseness

32
Q

what is the most common motor neuron dz

A

ALS

33
Q

motor neuron dz diagnosis

A

should be made over a long period of time

34
Q

ALS epi

A

-40-70 yo , M>W

35
Q

ALS early s/s

A
  • may start with focal weakness and atrophy in a limb such as a shoulder or leg (foot drop)
  • spreads and becomes bilateral
36
Q

ALS later

A
  • both UMN and LMN degenerate + widespread fasciculations
  • extraocular muscles and sphincters of bladder and bowel are spared
  • rarely familial–SOD enzyme defective; more commonly sporadic
37
Q

ALS pathological findings

A
  • degeneration of corticospinal and corticobulbar tracts
  • gliosis and loss of anterior horn cells and puramidal neurons
  • neurogenic atrophy of muscle
38
Q

ALS mimics

A
  • cervical radiculomyelopathy (LMN in UE, UMN in LE)
  • stroke
  • rare ai neuromuscular disorders
39
Q

ALS drugs

A

-Riluzole-opposes excitotoxic effect of glutamate at the NMDA receptors of motor neurons

40
Q

most common site of ulnar nerve entrapment

A

elbow where the ulnar nerve lies at the medial epicondylar groove

41
Q

ulnar nerve syndrome

A

-dec sensation over 4th and 5th fingers and weak grip d/t involvement of interosseous muscles of the hand