CH43: Peripheral Nerves Flashcards

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1
Q

Differentiate central and peripheral nerve (p. 1310)

A

Myelin of centrally located fibers: oligodendrocyte
Myelin of peripheral: Schwann cells

Support central:astroyctes
Supprt peripheral: Fibroblasts

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2
Q

Disease mechanism is producing toxin that directly acts on the membrane of the Schwann cells near the DRG(p. 1311)

A

Diphteria

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3
Q

Disease mechanism is occlusion of vasa nerosum (p. 1311)

A

Polyarteritis nodosa

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4
Q

Disease mechanism is treponemal meningoradiculitis (p. 1311)

A

Tabes dorsaleis

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5
Q

Disease mechanism is combination of toxin with axoplasm of the largest sensory and motor nerves via sulfhydryl bonds (p. 1311)

A

Arsenic

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6
Q

Disease mechanism is damaging the microtubular transport (p. 1311)

A

Vincristine toxicity

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7
Q

Retrograde changes consisting of swwelling of the cell cytoplasm and marginalization and dissolution of the NIssl substance (p. 1312)

A

Chromatolysis

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8
Q

Exception wherein an axonal process is seen presenting as proximal weakness (p. 1312)

A

Porphyria

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9
Q

Compare polyneuropathies vs multifocal. (p. 1313)

A

The multifocal nature of lesions and blocakge of electrical conduction often leads to weakness of proximal limb and muscles before or at the same time as distal parts are affected.

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10
Q

Disuse vs Denervation atrophy (p. 1313)

A

In dususe, the muscle does not reduce its size by ore than 25 to 30%.

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11
Q

As a rule, neuropathies are associated with a reduction or loss of tendon reflexes EXCEPT (p. 1314)

A

small fiber neuropathies; in which DTR may be retained even with marked loss of perception of painful stimuli.

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12
Q

Characteristic of sensory ataxic gait (p. 1315)

A

brusque, flinging, slapping movements

NO NYSTAGMUS, DYSARTHRIA

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13
Q

Two most frequent manifestation of autonomic failure (p. 1315)

A

Anhidrosis

Orthostatic hypotension

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14
Q

Is there dysautonomia in radicular diseases? (p.1316)

A

No. Because the autonomic fiebrs join the spinal nerves from the sympathetic chain and parasympathetic ganglia more distally.

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15
Q

Fasciculations and cramps are NOT prominent features in polyneuropathies (they are in anterior horn cell diseases) EXCEPT (p. 1316)

A

Chronic spinal motor root compression leads to fasciculations or painful spams in the innervated muscles

TREATMENT: Carbamazepine, Phenytoin

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16
Q

Differentiate an L5 vs peroneal nerve lesion. (p. 1318)

A

Check INVERSION. If spared, lesion is in the peroneal nerve.

Check sensory:
L5 extends almost up to the knee on the anterior surface of the foreleg
Peroneal: it ends a limited distance above the ankle

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17
Q

Infections associated with GBS(p. 1321)

A

C jejuni
Herpes family (CMV EBV HIV)
Campylobacter- Mycoplasmae and Lyme

Not infection: lymphoma and systemic autoimmune

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18
Q

GBS: Antibody (p. 1323)

A

acute motor axonal- GM1
PCB- GT1b, GD1a
MFS- GQ1B

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19
Q

Pathologic findings in GBS (p.1323)

A

endoneural perivascular lymphocytic infiltrates

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20
Q

In GBS, the CSF is under normal pressure or contains only a few lymphocytes in all but __%of patients (p. 1323)

A

`10%

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21
Q

Very earliest changes in pathology of GBS(p. 1325)

A

deposition of compleemnt on the innner layer of myelin

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22
Q

Differentiate Tick paralysis vs GBS (p.1325)

A

Sensory loss is not a usual feature of tick paralysis and CSFprotein is normal

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23
Q

MG vs GBS (p. 1326)

A

The mandibular muscles remain relatively stron in GBS

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24
Q

Botulism vs GBS (p.1326)

A

Bradycardia in botulism

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25
Q

The ability to reach a count of 20 generally corresponds to a vital capacity of greater than (p.1326)

A

1.5L

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26
Q

Dysautonomia in how many percent of GBS patients (p. 1326)

A

10%

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27
Q

Bizarre waking dreams or hallucinations after weeks of immobilization (p. 1326)

A

Oneiric hallucinations

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28
Q

Dose of plasma exchange (p. 1327)

A

200 to 250ml/kg in 4 to 6 treatments on alternate days; onset less than 2 weeks

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29
Q

Dose of IvIg (p. 1327)

A

0.4g/kg per day for 5 consecutive days

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30
Q

How many percent of GBS who intiially improve will relapse with treatment (p. 1327)

A

10%

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31
Q

Differentiate between acute sensory neuronopathy and GBS (p. 1329)

A

In GBS, there is usually some degree of proximal wekaness and sensory changes do not extend to the face and trunk

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32
Q

Important pathologic change in diphtheria (p. 1330)

A

segmental demyelination without inflammatory reaction of spinal roots, sensory ganglia, and adjacent spinal nerves

Anterior horn cells, axons, peripheral nerves distally and muscle fibers remain normal

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33
Q

Treatment for diphtheria (p. 1330)

A

Diphtheria antitoxin within 48 hours

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34
Q

This disease is due to a metabolic defect in the liver marked by increased production and urinary excretion of porphobilinogen and porphyrin precurose alpha aminolevulenic acid (p. 1330)

A

Porphyria

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35
Q

Characteristics of porphyric neuropathy (p. 1330)

A
relapsing nature
acute onset
abdominal pain
psychotic symtoms
predominant motor neuropathy- often bibrachial distrubution of weakness
truncal sensory loss
tachycardia
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36
Q

Treatment for porphyria (p. 1331)

A

IV glucose and IV hematin

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37
Q

Lung tumors polyneuropathy (p. 1332)

A

Anti-Hu

38
Q

This carcinoma accoujnts approx 50 percent of cases of paraneoplastic sensorimotor polyneuropathy and 75% of those with pure sensory neuropathy (p. 1333)

A

Lung Ca

39
Q

White transverse banding of the nails (p. 1333)

A

Mee’s lines in arsenic

40
Q

Chronic exposure to lead paint or fumes (p. 1333)

A

Lead neuropathy

41
Q

Antineoplastic drugs and neuropathy (p. 1334)

A

Cisplatin, Carboplatin, Bortezomib
- sensory neuropathy and acrodynia and episodic color changes in fiber tips

Paclitaxel [ovarian]
- sensory neuropathy

Vincristine [lymphomas and leukemias]
paresthesias

42
Q

How much pyridoxine to give to prevent neuropathy from INH (p. 1335)

A

150 to 450mg daily

43
Q

Most important factor in the development of DM neuropathy (p. 1336)

A

Duration of diabetes

44
Q

Isolated involvement of practically all the major peripheral nerves has been described in diabetes but the msot frequently affected are the __ (p. 1337)

A

femoral
sciatic
peroneal

45
Q

Most prominent pathologic finding in DM neuropathy (p. 1338)

A

loss of myelinated nerve fibers

46
Q

Persistent hypergycemia inhibits this transport (p. 1339)

A

sodium- dependent myoinopsitol transport

47
Q

Treatment for DM neuropathy (p. 1339)

A

Amitryptilline
Duloxitine
Gabapentin
Pregabalin

48
Q

Nerve biopsy findings of polyarteritis nodosa (p. 1340)

A

necrotizing arteritis in medium- size vessles with infiltrating eosinophils and occlusion of vessels

TX: Rituximab, Cyclophosphamide

49
Q

Vasculitis that involves the lungs in contrast to renal and bowel infarctions of Polyarteritis Nodosa (p. 1340)

A

Churg-Strauss

50
Q

Finging relatively specific for Wegener and Churg Strauss that helps differentiate with polyarteritis (p. 1341)

A

circulating c-ANCA

TX: Corticosteroids, rituximab, cyclophosphamide

51
Q

Distinguishes the neuropathy of sarcoidosis from other forms of mononeuropathy multiplex (p. 1343)

A

`Occurence of large, irregular zones of sesnory loss over the trunk

52
Q

Triad of Lyme disease (p. 1343)

A

cranial nerve palsies
radiculitis
aseptic meningitis

53
Q

Painful lumbosacral radiculitis of lyme disease (p. 1344)

A

Bannwarth syndrome
- sparing of the proximal or distal part of the limb while the adjacent part is weakend

Dx: Western blot
Tx:Ceftriaxone 2g for 1 month

54
Q

Chronic slowly progressive autoimmune disease characterized by lymphocytic infiltration of exocrine glands, particularly of parotid and lacrimal glands hence keratoconjunctivitis and sicca (p. 1344)

A

Sjogren Disease

Tx: Prednisone, Cyclophosphamide, Rituximab

55
Q

Serologic abnormalities in Sjogren (p. 1345)

A

AntiRo SSA; Ani La SSB

56
Q

The defining feature of this unusual syndrome are searing and pulling sensations involving small cutaneous areas that are evoked by exetending or stretching the limb (p. 1346)

A

Wartenberg syndrome

57
Q

One exception to the rule that all chronic neuropathies are more or less symmetrical in pattern (p. 1348)

A

Leprous neuritis

58
Q

In polyneuropathy with associated paraproteinemia, Roberg sign, propioceptive sensory loss with gait imbalance, tremor are typical fidngins in teh group with ___activity (p. 1349)

A

Anti-MAG

59
Q

Hyperviscosity in Walderstrom hyperproteinemia manifesting as diffuse slowign of retianl and cerebral circulations, episodic confusion, coma, impairment of vision and strokes (p. 1350)

A

Bing- Neel syndrome

60
Q

Chian in myeloma (p. 1351)

A

Kappa

Lambda can be in amyloid neuropathy or osteosclerotic type

61
Q

Pathologic findings in CIPD (p. 1353)

A

interstitial and perivascular infiltates of inflammatory cells

Tx: IvIg, Plasmapharesis, Cyclophosphamide, Mycophenolate mofetil, Rituximab

62
Q

MMN antibody associated (p. 1355)

A

Angi-GM1

63
Q

Description of uremic polyneuropathy (p. 1355)

A

painless, progressive, symmetrical sensorimotor paralysis of legs and arms

64
Q

Type of leprosy:epithelioid granuloma that involves cutaneous and subcutaneous nerves and results in characteristic hypopigmented patch of sperficial numbness and sensory loss (p. 1356)

A

tuberculoid leprosy

65
Q

Type of leprosy: proliferation of hematogenous spreads of bacilli and the diffuse infiltration of skin, ciliary bodies, testes, lymph nodes and nerves (p. 1356)

A

lepromatous leprosy

66
Q

Idiopathic small fiber sensory polyneuropathy gene assocation (p. 1358)

A

SCN9A encoding voltage gated sodium channel NaV1.7

67
Q

Most common type of Charcot Marie Tooth (p. 1361)

A

CMT1A- due to 17p duplication

68
Q

Muscle deformity due to CMT (p. 1361)

A

inverted champagne bottle

69
Q

Difference between CMT1 and CMT2 (p. 1361)

A

Enlargement of nerves in Type 1, most easily appreciated by palpation of the great auricular and peroneal nerves

70
Q

Electrographic features of CMT (p. 1361)

A

slow (less than 38m/s) in CMT1

normal in CMT2

71
Q

If slow nerve velocities, check for these genes in CMT (p. 1362)

A

PMP22, CMTX1

72
Q

Pathologic findings in CMT (p. 1362)

A

depletion of population of large sensory and motor fibers, leaving only the condensed endoneural connective tissue

73
Q

Hereditary Neuropathy with Pressure Palsies vs CMTY (p. 1362)

A

both PMP22A gene is affected.
In CMT1A- gene is duplicated
In HNPP- gene is deleted

74
Q

Hypertrophy neuropathy of infancy (p. 1362)

A

Dejerine-Sottas Disease

75
Q

In HSAN1, a consequence of loss of function of this enzyme which is the rate- limiting step in the biosynthesis of sphingolipids (p. 1363)

A

serine palmitoyltransferase

76
Q

Symmetrical lipomas of the neck and shoulders that are associated with polyneuropathy and sometimes, deafness (p. 1364)

A

Launois- Bensaude disease

77
Q

Familial dysautonomia after birth. Tongue lacks fungiform papillae (p. 1364)

A

Riley-Day Familial dysautonomia

DX: check amounts of homovanillic acid and decreased vanillylmandelic acid and methoxyhydroxyphenylglycol

78
Q

Retinitis pigmentosa, ataxia, chronic polyneuropathy with increase in blood phytanic acid (p. 1366)

A

Refsum disease

79
Q

Finding of red blood cell acanthocyts, low serum cholesterol and b lipoprotein (p. 1366)

A

Bassen- Kornzweig Syndrome

80
Q

Mutation elimiantes the function of ATP casette transport protein (p. 1367)

A

Tangier Disease

81
Q

alpha-galactosidase deficiency; angiokeratomas, painful neuropathies (p. 1367)

A

Fabry disease

82
Q

Metaphyseal dysplasia that resembles acromegaly (p. 1367)

A

Pyle disease

83
Q

Amyloid is derived from an inherited abnormality of serum protein __ (p. 1468)

A

transthyretin

84
Q

Two novel treatment for amyloid (p. 1369)

A

Tafamidis

Interfering RNA therapy

85
Q

Ischemic condition resulting from thrombosis of the subclavian artery or vein (p. 1372)

A

Paget- Schrotter syndrome

86
Q

If the denervatin changes are found in the paraspina lmuscles, the source of weaknes and pain is in the intraspinal roots, proximal to the plexus (p. 1372)

A

TRUE

87
Q

Nerve damaged in meatpackers neuropathy (p. 1376)

A

Suprascapular nerve

from carrying objectes on teh shoulder

88
Q

Sign that consists of hyperflexion of the wrist for 30 to 60s (p. 1377)

A

Phalen maneuver

89
Q

lighlty tapping the volar aspect of the rist ast the transverse carpal ligament (p. 1377)

A

Tinel sign

90
Q

Uppper vs Lower lumbar plexus (p. 1378)

A

ULP:
weakness of flexion and adduction of the thigh and extension of the leg
sensory of anterior thigh and leg

LLP
weakness of posterior thigh, leg and foot muscles and abolish sensations over the first and second sacral segments

91
Q

Most common cause of femoral neuropathy (p. 1381)

A

diabetes

92
Q

Most frequently compressed nerves in order (p. 1382)

A
median
ulnar
peroneal
tibial
plantar