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Med School Year 2 Flash Cards > Peripheral Nerve Disease > Flashcards

Peripheral Nerve Disease Flashcards

1
Q

What is segmental demyelination?

A

Damage to the myelin or Schwann cell disease

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2
Q

What is the process of recovery after demyelination?

A
  1. Disintegrating myelin is engulfed by Schwann cells, then by macrophages
  2. Axon stimulates endoneurium for remyelination
  3. New myelinated internodes are shorter and thinner
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3
Q

What are onion bulbs?

A

Concentric lays of Schwann cell cytoplasm and BM from sequential demyelination
Will eventually cause axonal injury

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4
Q

What is Guillan-barre?

A

Inflammation and demyelination of the peripheral nerves and spinal nerve roots

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5
Q

What are the sx of Guillan-Barre?

A

Decreased DTRs
Ascending paralysis
Increase CSF protein

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6
Q

What is the histo of Guillan-Barre?

A

Inflammation of nerves
Macrophage processes penetrate the BM of Schwann cells
Segmental demyelination

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7
Q

What is Guillan-Barre preceded by?

A

An acute flulike illness
Pathogens associated: campylobacter, CMV, EBV, mycoplasma
Mediated by T cells

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8
Q

What is seen in chronic inflammatory demyelinating polyradiculoneuropathy?

A

Symmetric sensorimotor polyneuropathy
Onion bulbs

Tx : steroids, plasmapheresis

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9
Q

What is seen in lepromatous infectious peripheral nerve disease?

A

M. Leprae invades the Schwann cells causing demyelination
Endoneurium fibrosis
Thickening of peri neural sheaths
Symmetric polyneuropathy — ulcers, wounds
Granulomatous inflammation of the dermis

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10
Q

What is seen in diphtheria?

A

Paresthesias
Weakness
Loss of proprioception and vibratory sense
Demyelination of axons in the anterior and posterior roots

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11
Q

What is Walllerian degeneration?

A

Degeneration of distal ends of fibers on a focally damaged axon

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12
Q

What is denervation atrophy?

A

When muscle fibers lose neural input

Will see angulated cells and target fibers (rounded zone of reorganization)

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13
Q

What is the cause of axonal degeneration and muscle fib atrophy?

A

Trauma

Ischemia

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14
Q

What happens in reinnervation?

A

Neighboring unaffected motor unit sprouts over to innovate the damaged segment— loss of checkerboard, get type grouping

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15
Q

What is the inheritance pattern of muscular dystrophy?

A

X linked

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16
Q

What is the mutation in muscular dystrophy?

A

Frameshift or point mutation in X that codes for dystrophin

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17
Q

What is dystrophin?

A

Cytoplasmic protein of z band that forms the interface between intracellular contractile apparatus and extra cellular connective tissue matrix

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18
Q

What is the histo of muscular dystrophy?

A

Variation in fiber size,
Muscle replaces by fat and CT
Hyper contracted fibers

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19
Q

What are hyper contracted fibers?

A

Enlarged, rounded, hyaline fiber with no cross striation in DMD

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20
Q

What do females who are carriers of DMD often have?

A

Dilated cardiomyopathy

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21
Q

What are the sx of DMD?

A

Delay in walking
Pseudo hypertrophy of calf muscles - increase in fat and CT tissue
Cardiac arrhythmias
Cognitive impairment

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22
Q

What are the causes of death in DMD?

A

Respiratory insufficiency
Cardiac decomp
Pulmonary infection

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23
Q

What is dystrophy is autosomal dominant in inheritance and autosomal recessive?

A

Limb girdle

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24
Q

What is the mutation in limb girdle dystrophy?

A

In sacroglycan complex which interacts with dystrophin via b-dystroglophin

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25
Q

What is the inheritance pattern of myotonic dystrophy?

A

Auto Dom

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26
Q

What is the mutation in myotonic dystrophy?

A

CTG repeat on ch.19 that affects DMPK

27
Q

What dystrophy has ring fibers, change in intrafusal fibers, sarcoplasmic mass

A

Myotonic

28
Q

What are the sx of myotonic dystrophy?

A 
Abnormal gait
Ptosis
Cataracts
Frontal balding
Gonadal atrophy
Dementia
Decrease IgG
Sustained involuntary contraction
29
Q

What myopathy is due to vigorous exercise, cold, or high carb meals?

A

Ion channel myopathies

30
Q

Which gene is mutated in the hypokalemic kind?

A

Gene for voltage gated calcium channel

31
Q

What gene is mutated in the hyperkalemic ion channel myopathy?

A

Sodium channel in skeletal muscle

32
Q

What is malignant hyperprexia?

A

Hyper metabolic state induced by halogenated anesthesias

33
Q

What is abnormal in the lipid myopathies?

A

Carnitine transport system

Deficiency of mitochondrial dehydrogenase enzyme

34
Q

What do you see in lipid myopathies?

A

Accumulation of lipid droplets within muscle
Myo fibrils separated by vacuoles that stain with oils red O or Sudan black
Occurs in type 1 fibers

35
Q

What is seen in mitochondrial myopathies?

A

Aggregate of abnormal mitochondria
Ragged red fibers
Para crystalline parking lot inclusions

36
Q

How do people with mitochondrial myopathies present?

A

With proximal muscle weakness

External ophthalmoplegia

37
Q

What is dermatomyositis?

A

Inflammatory myopathy where there is antibody attack on the microvasculature so there is foci of ischemic myocyte necrosis

38
Q

What is the morphology of dermatomyositis?

A

Inflammatory infiltrates around small blood vessels and in perimysium
Perifascicular atrophy
Necrotic muscle fibers and regeneration

39
Q

What are the sx of dermatomyositis?

A

Iliac or heliotrope rash of upper eyelids with peri orbital edema
Groton lesions= scaling erythmatous eruption or dusky red patches over knuckles, elbows, knees

40
Q

What disease has antibodies directed against tRNA synthetase?

A

Polymyositis

41
Q

What disease is ANA positive, mediated by CD8 T cells and macrophages with infiltrates in the endomysium?

A

Polymyositis

42
Q

What disease involves the distal muscles, has asymmetric weakness, amyloid deposition and is not improved by immunosuppressive therapy?

A

Inclusion inflammatory myopathy

43
Q

What is the auto dom mutation in inclusion?

A

Mutation in gene for myosin heavy chain IIa

44
Q

What is the auto recessive mutation for inclusion?

A

Mut in GNE gene

45
Q

What is seen in inclusion?

A

Rimmed vacuoles
Amyloid deposition
Hyperphosphorylated tau protein

46
Q

What disease present with proximal muscle weakness, exophthalmic ophthalmoplegia, cramping muscles, slowed reflexes, and episodic weakness with hypokalemic?

A

Thyrotoxic myopathy

47
Q

What presents with acute pain of a single muscle group and myoglobinuria?

A

Ethanol myopathy

48
Q

Which fibers are affected in steroid myopathy?

A

Type 2

49
Q

What myopathy is associated with presence of vacuoles in the myocytes, autophagic membrane, curvilinear bodies?

A

Chloroquine myopathy

50
Q

What myopathy is associated with dilation of the SR and thickening of the basal laminate and atrophy of type 2 fibers?

A

Steroid myopathy

51
Q

What disease is associated with antibodies to the acetylcholine receptor?

A

Myasthenia gravis

52
Q

What disease is associated with thymic hyperplasia, t

Type 2 atrophy, ptosis, diplopia, decreased motor response with repeated stimulation?

A

Myasthenia gravis

53
Q

Who gets myasthenia gravis?

A

Women before 40

54
Q

What is the treatment for myasthenia gravis?

A

Thymectomy
Prednisone
Plasmapheresis
Anticholinesterase agents

55
Q

What develops as a paraneoplastic process?

A

Lambert eaton myasthenic syndrome

56
Q

What disease has fewer vesicles synapse in response to an action potential?

A

Lambert eaton

57
Q

What disease has enhanced neurotransmission with repeated stimulation?

A

Lambert eaton

58
Q

What disease manifests as progressive muscle atrophy below the knee in childhood, palpable nerves and is autosomal dominant?

A

HMSN I

59
Q

What is the most common mutation of HMSN I?

A

Mut in PMP22 - transmembrane protein in myelin

60
Q

What disease has a mutation in KIF1B, is auto Dom and has no nerve enlargement?

A

HMSN II

61
Q

What disease has depressed DTRs, auto recessive, palpable nerves, presents in early childhood?

A

Dejerine soltas neuropathy

62
Q

What disease is auto recessive and destroys the anterior horn cells and CN motor neurons?

A

Spinal muscular atrophy

63
Q

What is the mutation in spinal muscular atrophy?

A

SMN1 on ch.5 required for motor neuron survival

64
Q

What is seen in spinal muscular atrophy?

A

Panfasciculad atrophy
Floppy baby
Death with first 3 years

Med School Year 2 Flash Cards (18 decks)

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