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NMSP 2 > Peripheral Nervous System And Skeletal Muscles Pathology Dr. Martin > Flashcards

Peripheral Nervous System And Skeletal Muscles Pathology Dr. Martin Flashcards

1
Q

motor unit consists of

A

axon,
lower motor neuron,
muscle fibers

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2
Q

peripheral nerve consists of

A

nerve fiber + myelin, schwann cells ( have nodes of ranvier, each schwann cell covers 1 internode)

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3
Q

Epineurium

A

encloses entire peripheral nerve

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4
Q

Perineurium

A

multilayered concentric CT that encloses each fascicle

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5
Q

Endoneurium

A

surrounds each nerve fiber

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6
Q

Axonal degeneration involves and does what

A

axon and neuron , can cause axon regeneration and reinnervation of muscle

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7
Q

Segmental Demyelination

A

myelin breakdown in segments

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8
Q

denervation atrophy vs myopathy

A
  1. denervation atrophy : loss of axon causes atrophy of muscle
  2. Myopathy : abnormal muscle fiber itself
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9
Q

segmental demyelination of schwann cell vs myelin sheath

A
  1. schwann cell : hereditary or sensory neuropathy

2. myelin : guillain barre

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10
Q

denuded axon

A

remyelination signal (new myelin in shorter in length then normal myelin)

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11
Q

cross section of thin myelin on a nerve looks like

A

onion cut (onion bulb)

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12
Q

Traumatic neuroma

A

axons that cant find their distal target (muscle) = pseudotumor made (non-neoplastic whorled proliferation of axonal processes and
= CAUSES PAINFUL NODULE

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13
Q

Wallerian degeneration

A

focal lesion in axon (distal part of axon is damaged

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14
Q

Axon damage

A

from ischemia or trauma = whole neuronal body or its axon

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15
Q

axonal degeneration

A

primary destruction of axon and myelin

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16
Q

axonal degeneration steps of what happens after

A
  1. Myelin Ovoids : schwann cells catabolize myelin + later engulf axon fragments = oval compartment made
  2. M clean them up
  3. proximal part of severed nerve degenerates in 2-3 distal internodes
  4. regenerates
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17
Q

axonal degeneration shows what SIGNS on histology of nerve

A
  1. Denervation Atrophy
  2. Angulated ends (smaller and triangle shaped muscle fibers
  3. Target Fibers
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18
Q

what determines the fiber type

A

motor neuron

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19
Q

group atrophy

A

a type group (muscle fiber type) gets denervated

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20
Q

type 2 fiber atrophy can happen from

A

= inactivity
= diffuse (limb fracture, pyramidal tract degeneration, neurodegenerative dz)
= STERIOID MYOPATHY (from glucocorticoids)

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21
Q
  1. hypertrophy does what to muscle fiber

and 2. segmental necrosis

A
  1. splitting of fiber (from increased exercise = fibers split)
  2. deposits of collagen and fat
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22
Q

Regenerated nerves stain how

A

cytoplasm laden with RNA = RED trichrome stain

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23
Q

somatic motor function travel how

A
  1. lower motor N on ANTERIOR HORN

2. to muscle fiber

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24
Q

Somatic Sensory nerve travels how

A
  1. distal nerve ending (specialized sensory structures) pick up signal
  2. travels with peripheral N to DORSAL ROOT GANGLION
  3. go to brain to synapse at SPC and brainstem
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25
Q

Autonomic nerve fiber problems involves what disease

A

Diabetic neuropathy

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26
Q

unmyelinated fibers

A

temp and pain = slow conduction

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27
Q

large axon with myelin

A

light touch, motor signals

= fast conduction

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28
Q

Axonal Neuropathy

  1. targets what never usually and causes what
  2. physiologic hallmark
A
  1. longest neuron = dying back progression

2. reduction of amplitude, PRESERVATION of conduction velocity

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29
Q

electrophysiologic hallmark of demyelinating neuropathy

A

SLOWED nerve conduction,

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30
Q

Neuronopathies

A

destruction of neurons (infection Herpes Zoster, Toxins platinum)
= effects proximal and distal body equally (HALLMARK)

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31
Q

peripheral neuropathy SX

A
  1. tingling, stabbing, buring, or pins and needles
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32
Q

peripheral neuropathy : polyneuropathies

A
  1. many nerves usually symmetric
  2. starts distal and ascends
  3. hands involved usually at level of knee (stocking and glove)
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33
Q

Mononeuritis Multiplex :

A

many nerves damaged in haphazard distribution

VASCULITIS —> Polyarteritis nodosum PAN

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34
Q

mononeuropathies

A

single nerve effected

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35
Q

Bell’s Palsy

  1. effects what
  2. age
  3. SXX
A
  1. CN 7 mononeuropathy (facial muscle paralysis)
  2. 15-60yo (resolves spontaneously)
  3. one side facial droop , facial tingling, headache + neck pain, memory prob, balance issues, SAME side limb paresthesias, low tears tasting saliva)
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36
Q

Bell’s Palsy associated with what and can look like what conditions

A
  1. DM and URI

2. stroke, brain tumor, Ramsay Hunt Syndrome (type 2 herpes zoster) , Lyme disease

37
Q

Neurologenic bladder

  1. is what
  2. can be caused by degenerative dz
  3. can be caused by what else
A
  1. lack of bladder control from spinal cord or nerve problem
  2. Parkinsons, DM, MS
  3. infection brain or SC , heavy metal, stroke, SC or pelvic injury
38
Q

Neurologenic bladder can be seen in what congenital condition

A

spina bifida

39
Q

Neurologenic bladder 4 types of things what nerves are damaged for the bladder

  1. Hypogastric nerve
  2. pelvic nerve
  3. pudendal nerve
A
  1. overactive bladder
  2. underactive bladder
  3. incontinence (cant hold)
  4. obstructive bladder (can empty)
40
Q

Guillain Barre

  1. first sx seen
  2. nerve effected and what happens
A
  1. Loss of deep tendon reflexes or flu illness after recovery (Campylobacter jejuni, CMV, EBV, Mycoplasma pneumonia, vaccine)
  2. multifocal destruction of myelin (Demyelination + inflammation) of SPINAL nerve ROOTS + PERIPHERAL NERVES = Radiculoneuropathy
41
Q

Guillain Barre

  1. type of neuropathy
  2. histology seen
  3. DX by
A
  1. ascending polyneuropathy
  2. perivenular + endoneurial infiltration of inflammatory cells
    (SEGMENTAL DEMYELINATION)
  3. Anti-Myelin ABs
42
Q

Guillain Barre :

  1. where to M like to eat
  2. CSF findings
  3. TX
A
  1. right by the Nodes of Ranvier (it can go under myelin and strip it away)
  2. HIGH protein (from permeability increase) LOW WBCs they stay at the roots
  3. Plasmapheresis IVIg
43
Q

Chronic Inflammatory Demyelinating Poly(radiculo)neuropathy:

  1. what type of neuropathy
  2. dx criteria
  3. caused by
  4. different then Guillan Barre how
A
  1. most common acquired, symmetric
  2. persists for at least 2mo
  3. T cells and B cells (ABs) against myelin, IgG and IgM found
  4. responds to steroids
44
Q

Chronic Inflammatory Demyelinating Poly(radiculo)neuropathy:
histology looks like and how to dx

A

Biopsy of sural nerve : ONION BULBS seen (many schwann cell layers look like onion rings (recurrent demyelination and remyelination)

45
Q

systemic autoimmune diseases causing peripheral neuropathy and what type

A

SLE*, RA, Sjögrens

= distal sensory or sensorymotor

46
Q

Vasculitis causing neuropathy :

  1. type neuropathy
  2. type of vasculitits
  3. most common vasculitis causing this
  4. histo
A
  1. mononeuritis multiplex
  2. MPO-ANCA (microscopic PA, Eosinophilic granulomatosis, Churg-Strauss) + PAN + PR3-ANCA (GPA)
  3. Nonsystemic vasculitic neuropathy
  4. patchy axonal degeneration
47
Q

Leprosy and neuropathy : Lepromatous Leprsy

  1. caused by
  2. what happens
  3. histo
  4. involves what
A
  1. Mycobacterium leprae attacking myelin
    2 . Symmetric Segmental demyelination + remyelination (LOSS of myelinated + unmyelinated axons
  2. Endoneurial fibrosis + multilayerd thickening of perineural sheaths
  3. pain fibers , loss of sensation (likes COOL extremitis)
48
Q

Leprosy and neuropathy : Tuberculoid Leprosy

  1. what happens
  2. sx + location
  3. what histo
A
  1. active cell mediated immune response
  2. granulomatous nodules on dermis, localized nerve involvement (Cutaneous nerves)
  3. fibrosis of perineurium and endoneurium
49
Q

Lyme dz and neuropathy

A

BILATERAL facial nerve palsy

(polyradiculoneuropathy

50
Q

HIV and neuropathy

  1. type of neuropathy (early stage)
  2. SX and what happens
  3. late stage
A
  1. Mononeuritis multiplex
  2. Demyelination looking like Guillain Barre, chronic inflammatory demyelinating polyradiculopathy
  3. Distal sensory neuropathy
51
Q

Diphtheria and vasculitis

  1. SX
  2. from what
  3. location effected
  4. Associated SX**
A
  1. paresthesia and weakness, loss of proprioception and vibratory sensation
  2. bacteria exotoxin (not vaccinated or waning immunity)
  3. demyelination of axons extending to Anterior Root + Posterior Root + Mixed sensorimotor nerves
  4. PROMINENT BULBAR + RESPIRATORY MUSCEL DYSFUNCTION = DEATH or long term disability
52
Q

Varicella Zoster Virus and Neuropathy :

  1. effects what
  2. can cause what type of problem
  3. SXs
A
  1. sensory ganglia degeneration+ large motor neurons in ANTERIOR HORN + cn nuceli
  2. reactivation = shingles + painful vesicular skin eruptions (trigeminal or thoracic nerve dermatomes)
  3. regional necrosis, hemorrhage, loss of effected ganglia (burning, tingling, itching)
53
Q

most common cause of peripheral neuropathy

A

DM2 and DM1

54
Q

Diabetes and neuropathy

  1. type and how it acts
  2. SX
  3. can cause what to lead to morbidity
A
  1. ascending distal symmetric sensorimotor polyneuropathy (loss of axons)
  2. numbness, tingling, loss of pain, difficult balance
  3. low sensation in feet causing ulcers and amputation + diffuse vascular injury
55
Q

Diabetic neuropathy

  1. histo and nerves effected
  2. ANS dysfuntion condition
  3. peripheral neuropathy condition
A
  1. loss of myelinated and unmyelinated small fibers + large , ENDOONEURIAL ARTERIOLES thick and hyalinized, PAS+ and duplicated BM**
  2. postural hypotension, cant empty bladder (uti), sexual dysfunction
  3. ASYMMETRIC mononeuropathy, cranial neuropathy, radiculoplexus neuropathy
56
Q

Diabetes and neuropathy progresses due to what 2 things

A

vascular and metabolic changes contribute

57
Q

Cartilage forming tumors : Osteochondroma

  1. location
  2. age
  3. morphology
  4. B or M
  5. B
A
  1. metaphysis of long bones
  2. 10-30 yo
  3. bony excrescence with cartilage
58
Q

Cartilage forming tumors : Chondroma

  1. location
  2. age
  3. morphology
  4. B or M
A
  1. small bones of feet and hands
  2. 30yo-50yo
  3. circumscribed hyaline cartilage nodule in medulla
  4. B
59
Q

Cartilage forming tumors : Chondrosarcoma

  1. location
  2. age
  3. morphology
  4. B or M
A
  1. shoulder and pelvis
  2. 40yo-60yo
  3. extends from medulla through cortex into soft tissue, atypic chondrocytes
60
Q

Bone forming tumors : Osteoid osteoma

  1. location
  2. age
  3. morphology
  4. B or M
A
  1. Metaphysis of long bone
  2. 10-20yo
  3. cortical, interlacing microtrabeculae of woven bone
  4. B
61
Q

Bone forming tumors : Osteosarcoma

  1. location
  2. age
  3. morphology
  4. B or M
A
  1. metaphysis of distal femur, proximal tibia
  2. 10yo-20yo
  3. Extends from medulla to lift periosteum, malignant cells producing woven bone
62
Q

unknown orgin tumors : Ewing Sarcoma

  1. location
  2. age
  3. morphology
  4. B or M
A
  1. Diaphysis of long bone
  2. 10-20yo
  3. sheets of primitive small round cells
  4. M
63
Q

Uremic Neuropathy

  1. who gets this
  2. what happens SX
  3. what happens to nerves
A
  1. renal failure pts
  2. peripheral neuropathy = Distal Symmetric Neuropathy (asymptomatic, muscle cramps, low reflex, distal dysesthesias)
  3. axon degeneration (after dialysis recovers and regenerated)
64
Q

Thyroid Dysfunction : hypothyroidism

  1. causes what
  2. rare association
A
  1. compression mononeuropathies (carpal tunnel) , distal symmetric sensory neuropathy
  2. Guillain Barre
65
Q

Vit12 deficiency :

  1. what happens
  2. other name for this vitamine
A
  1. subacute combined degeneration with damage to long tracts in the spinal cord + peripheral nerves
  2. cyanocobalamin
66
Q

Vitamines associated with peripheral neuropathy

A
  1. B1 (Thiamine)
  2. B6 (pyridoxine)
  3. Folate
  4. copper
  5. Zinc
67
Q

metals and chemotx associated with peripheral neuropathy

A
  1. EtOH, Pb, Mercury, arsenic, thallium)

2. Vinca alkaloids, taxanes, microtuble inhibitors, cisplatin

68
Q

Neuropathies form malignancy

  1. brachial plexopathy
  2. Obturator Palsy
  3. Cranial palsy
  4. Polyradiculopathy of lower extremities
  5. Sensorimotor neuropathy
A
  1. Brachial plexopathy : apex of lung
  2. Obturator Palsy : pelvic tumor
  3. Cranial palsy : cranial tumor
  4. Polyradiculopathy : cauda equina involved in carcinomatosis
  5. sensorimotor neuropathy : Small cell lung Carcinoma
69
Q

sensorimotor neuropathy : Small cell lung Carcinoma presents how and DX how

A
  1. DX : anti-Hu antibodies
  2. start distally and asymmetric, mutlifocal
  3. if pt has Anti-CV2 (painful axonal asymmetric sensorimotor neuropathy
70
Q

Neuropathy associated with

1. monoclonal gammopathies , what happens, and SX

A
  1. B-cell neoplasm**
  2. IgM demyelination peripheral neuropathy, IgG and IgA also cause peripheral neuropathy
  3. POEMS (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, skin changes
71
Q

excess Ig can deposit how and cause

A

as amyloid, can lead to peripheral neuropathy, vascular insufficiency, direct toxic effect

72
Q

sympathetic chain syndrome can happen is what malignancy condition

A

Hornes Syndrome

73
Q

Traumatic neuroma

A 
regenerated slowly (axons are misaligned and continue to grow) =random oriented axons 
= can happen from trauma
74
Q

carpal tunnel syndrome what, sx, associated with

A
  1. entrappment of medial N
  2. numbness and paresthesia of tip of thumb and 1st 2 digits
  3. edema, preg, inflammatory arthritis, hypothyroidism, amyloid diabetes, wrist motions
75
Q

Saturday night palsy

A

radial nerve upper arm , fall asleep weird

76
Q

elbow and knee nerve compression

A

ulnar and peroneal

77
Q

Morton Neuroma

A

metatarsalgia, perineural fibrosis

78
Q

Inherited Peripheral Neuropathy : Hereditary Motor and Sensory Neuropathy

  1. other name
  2. sx
  3. 4 genes and there causes
A
  1. Charcot- Marie Tooth (CMT)
  2. distal muscle atrophy, sensory loss, for deformities
  3. CMT1** = AD demyelination (Chr17 PMP22 gene) most common

CMT1B = mutation in MPZ demyleination

CMTX = X-linked , GJB1 gene

CMT2 = AD axonal injury *(severe and early childhood) MFN2 gene

79
Q

Inherited Peripheral Neuropathy : Hereditary Sensory Neuropathy

  1. what happens
  2. sx
A
  1. loss sensation + other ANS (KEEPS MOTOR), axonal neuropathy
  2. Loss of pain and temperature, cant feel pain (can lead to big injuries)
80
Q

Inherited Peripheral Neuropathy : Familial Amyloid Polyneuropathies

  1. what happens
  2. mutation
  3. associated with
A
  1. Amyloid on peripheral nevers
  2. Transthyretin gene
  3. metabolic disfunctions
81
Q

NMJ problems present as

A

painless weakness and fatigue

82
Q

2 most common NMJ problem diseases

A
  1. Myasthenia Gravis

2. Lambert Eaton

83
Q

Myasthenia Gravis :

  1. what is the cause
  2. associated with what
  3. histology
A
  1. AB against AchR
  2. thymoma or thymic hyperplasia
  3. B-cell follicles in thymus
84
Q

Myasthenia Gravis :

  1. SX
  2. DX how
  3. TX how
A
  1. generalized fluctuating weakness worse with exertion and over day, repeated stimulation cause diminished response, Drooping eyelid, extraocular muscle weakness = diplopia
  2. decrease response to repeated stimuli
  3. Acetylcholinesterase Inhibitors, thymectomy, plasmapheresis
85
Q

Lambert-Eaton Myasthenic Syndrome :

  1. happens how
  2. Paraneoplastic to what
  3. SX
  4. TX
A
  1. AB against presynaptic CA channel = no Ach release
  2. Neuroendocrine carcinoma (Small Cell Carcinoma)
  3. proximal weakness in extremities, INCREASE response during repeated stimuli
  4. increase Ach release (depolarize membranes)
86
Q

Congenital Myasthenic Syndrome

  1. what happens
  2. SX
A
  1. muscle weakness due to AchR mutation

2. perinatal period, poor muscle tone, external eye muscel weakness, breathing probs

87
Q

Clostridium Botulinum can do what

A

Botox blocks Ach release

88
Q

Curare does what

A

blocks AchR = flaccid paralysis

NMSP 2 (3 decks)

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