CH43: Peripheral Nerves Flashcards
Differentiate central and peripheral nerve (p. 1310)
Myelin of centrally located fibers: oligodendrocyte
Myelin of peripheral: Schwann cells
Support central:astroyctes
Supprt peripheral: Fibroblasts
Disease mechanism is producing toxin that directly acts on the membrane of the Schwann cells near the DRG(p. 1311)
Diphteria
Disease mechanism is occlusion of vasa nerosum (p. 1311)
Polyarteritis nodosa
Disease mechanism is treponemal meningoradiculitis (p. 1311)
Tabes dorsaleis
Disease mechanism is combination of toxin with axoplasm of the largest sensory and motor nerves via sulfhydryl bonds (p. 1311)
Arsenic
Disease mechanism is damaging the microtubular transport (p. 1311)
Vincristine toxicity
Retrograde changes consisting of swwelling of the cell cytoplasm and marginalization and dissolution of the NIssl substance (p. 1312)
Chromatolysis
Exception wherein an axonal process is seen presenting as proximal weakness (p. 1312)
Porphyria
Compare polyneuropathies vs multifocal. (p. 1313)
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.
Disuse vs Denervation atrophy (p. 1313)
In dususe, the muscle does not reduce its size by ore than 25 to 30%.
As a rule, neuropathies are associated with a reduction or loss of tendon reflexes EXCEPT (p. 1314)
small fiber neuropathies; in which DTR may be retained even with marked loss of perception of painful stimuli.
Characteristic of sensory ataxic gait (p. 1315)
brusque, flinging, slapping movements
NO NYSTAGMUS, DYSARTHRIA
Two most frequent manifestation of autonomic failure (p. 1315)
Anhidrosis
Orthostatic hypotension
Is there dysautonomia in radicular diseases? (p.1316)
No. Because the autonomic fiebrs join the spinal nerves from the sympathetic chain and parasympathetic ganglia more distally.
Fasciculations and cramps are NOT prominent features in polyneuropathies (they are in anterior horn cell diseases) EXCEPT (p. 1316)
Chronic spinal motor root compression leads to fasciculations or painful spams in the innervated muscles
TREATMENT: Carbamazepine, Phenytoin
Differentiate an L5 vs peroneal nerve lesion. (p. 1318)
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
Infections associated with GBS(p. 1321)
C jejuni
Herpes family (CMV EBV HIV)
Campylobacter- Mycoplasmae and Lyme
Not infection: lymphoma and systemic autoimmune
GBS: Antibody (p. 1323)
acute motor axonal- GM1
PCB- GT1b, GD1a
MFS- GQ1B
Pathologic findings in GBS (p.1323)
endoneural perivascular lymphocytic infiltrates
In GBS, the CSF is under normal pressure or contains only a few lymphocytes in all but __%of patients (p. 1323)
`10%
Very earliest changes in pathology of GBS(p. 1325)
deposition of compleemnt on the innner layer of myelin
Differentiate Tick paralysis vs GBS (p.1325)
Sensory loss is not a usual feature of tick paralysis and CSFprotein is normal
MG vs GBS (p. 1326)
The mandibular muscles remain relatively stron in GBS
Botulism vs GBS (p.1326)
Bradycardia in botulism
The ability to reach a count of 20 generally corresponds to a vital capacity of greater than (p.1326)
1.5L
Dysautonomia in how many percent of GBS patients (p. 1326)
10%
Bizarre waking dreams or hallucinations after weeks of immobilization (p. 1326)
Oneiric hallucinations
Dose of plasma exchange (p. 1327)
200 to 250ml/kg in 4 to 6 treatments on alternate days; onset less than 2 weeks
Dose of IvIg (p. 1327)
0.4g/kg per day for 5 consecutive days
How many percent of GBS who intiially improve will relapse with treatment (p. 1327)
10%
Differentiate between acute sensory neuronopathy and GBS (p. 1329)
In GBS, there is usually some degree of proximal wekaness and sensory changes do not extend to the face and trunk
Important pathologic change in diphtheria (p. 1330)
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
Treatment for diphtheria (p. 1330)
Diphtheria antitoxin within 48 hours
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)
Porphyria
Characteristics of porphyric neuropathy (p. 1330)
relapsing nature acute onset abdominal pain psychotic symtoms predominant motor neuropathy- often bibrachial distrubution of weakness truncal sensory loss tachycardia
Treatment for porphyria (p. 1331)
IV glucose and IV hematin
Lung tumors polyneuropathy (p. 1332)
Anti-Hu
This carcinoma accoujnts approx 50 percent of cases of paraneoplastic sensorimotor polyneuropathy and 75% of those with pure sensory neuropathy (p. 1333)
Lung Ca
White transverse banding of the nails (p. 1333)
Mee’s lines in arsenic
Chronic exposure to lead paint or fumes (p. 1333)
Lead neuropathy
Antineoplastic drugs and neuropathy (p. 1334)
Cisplatin, Carboplatin, Bortezomib
- sensory neuropathy and acrodynia and episodic color changes in fiber tips
Paclitaxel [ovarian]
- sensory neuropathy
Vincristine [lymphomas and leukemias]
paresthesias
How much pyridoxine to give to prevent neuropathy from INH (p. 1335)
150 to 450mg daily
Most important factor in the development of DM neuropathy (p. 1336)
Duration of diabetes
Isolated involvement of practically all the major peripheral nerves has been described in diabetes but the msot frequently affected are the __ (p. 1337)
femoral
sciatic
peroneal
Most prominent pathologic finding in DM neuropathy (p. 1338)
loss of myelinated nerve fibers
Persistent hypergycemia inhibits this transport (p. 1339)
sodium- dependent myoinopsitol transport
Treatment for DM neuropathy (p. 1339)
Amitryptilline
Duloxitine
Gabapentin
Pregabalin
Nerve biopsy findings of polyarteritis nodosa (p. 1340)
necrotizing arteritis in medium- size vessles with infiltrating eosinophils and occlusion of vessels
TX: Rituximab, Cyclophosphamide
Vasculitis that involves the lungs in contrast to renal and bowel infarctions of Polyarteritis Nodosa (p. 1340)
Churg-Strauss
Finging relatively specific for Wegener and Churg Strauss that helps differentiate with polyarteritis (p. 1341)
circulating c-ANCA
TX: Corticosteroids, rituximab, cyclophosphamide
Distinguishes the neuropathy of sarcoidosis from other forms of mononeuropathy multiplex (p. 1343)
`Occurence of large, irregular zones of sesnory loss over the trunk
Triad of Lyme disease (p. 1343)
cranial nerve palsies
radiculitis
aseptic meningitis
Painful lumbosacral radiculitis of lyme disease (p. 1344)
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
Chronic slowly progressive autoimmune disease characterized by lymphocytic infiltration of exocrine glands, particularly of parotid and lacrimal glands hence keratoconjunctivitis and sicca (p. 1344)
Sjogren Disease
Tx: Prednisone, Cyclophosphamide, Rituximab
Serologic abnormalities in Sjogren (p. 1345)
AntiRo SSA; Ani La SSB
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)
Wartenberg syndrome
One exception to the rule that all chronic neuropathies are more or less symmetrical in pattern (p. 1348)
Leprous neuritis
In polyneuropathy with associated paraproteinemia, Roberg sign, propioceptive sensory loss with gait imbalance, tremor are typical fidngins in teh group with ___activity (p. 1349)
Anti-MAG
Hyperviscosity in Walderstrom hyperproteinemia manifesting as diffuse slowign of retianl and cerebral circulations, episodic confusion, coma, impairment of vision and strokes (p. 1350)
Bing- Neel syndrome
Chian in myeloma (p. 1351)
Kappa
Lambda can be in amyloid neuropathy or osteosclerotic type
Pathologic findings in CIPD (p. 1353)
interstitial and perivascular infiltates of inflammatory cells
Tx: IvIg, Plasmapharesis, Cyclophosphamide, Mycophenolate mofetil, Rituximab
MMN antibody associated (p. 1355)
Angi-GM1
Description of uremic polyneuropathy (p. 1355)
painless, progressive, symmetrical sensorimotor paralysis of legs and arms
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)
tuberculoid leprosy
Type of leprosy: proliferation of hematogenous spreads of bacilli and the diffuse infiltration of skin, ciliary bodies, testes, lymph nodes and nerves (p. 1356)
lepromatous leprosy
Idiopathic small fiber sensory polyneuropathy gene assocation (p. 1358)
SCN9A encoding voltage gated sodium channel NaV1.7
Most common type of Charcot Marie Tooth (p. 1361)
CMT1A- due to 17p duplication
Muscle deformity due to CMT (p. 1361)
inverted champagne bottle
Difference between CMT1 and CMT2 (p. 1361)
Enlargement of nerves in Type 1, most easily appreciated by palpation of the great auricular and peroneal nerves
Electrographic features of CMT (p. 1361)
slow (less than 38m/s) in CMT1
normal in CMT2
If slow nerve velocities, check for these genes in CMT (p. 1362)
PMP22, CMTX1
Pathologic findings in CMT (p. 1362)
depletion of population of large sensory and motor fibers, leaving only the condensed endoneural connective tissue
Hereditary Neuropathy with Pressure Palsies vs CMTY (p. 1362)
both PMP22A gene is affected.
In CMT1A- gene is duplicated
In HNPP- gene is deleted
Hypertrophy neuropathy of infancy (p. 1362)
Dejerine-Sottas Disease
In HSAN1, a consequence of loss of function of this enzyme which is the rate- limiting step in the biosynthesis of sphingolipids (p. 1363)
serine palmitoyltransferase
Symmetrical lipomas of the neck and shoulders that are associated with polyneuropathy and sometimes, deafness (p. 1364)
Launois- Bensaude disease
Familial dysautonomia after birth. Tongue lacks fungiform papillae (p. 1364)
Riley-Day Familial dysautonomia
DX: check amounts of homovanillic acid and decreased vanillylmandelic acid and methoxyhydroxyphenylglycol
Retinitis pigmentosa, ataxia, chronic polyneuropathy with increase in blood phytanic acid (p. 1366)
Refsum disease
Finding of red blood cell acanthocyts, low serum cholesterol and b lipoprotein (p. 1366)
Bassen- Kornzweig Syndrome
Mutation elimiantes the function of ATP casette transport protein (p. 1367)
Tangier Disease
alpha-galactosidase deficiency; angiokeratomas, painful neuropathies (p. 1367)
Fabry disease
Metaphyseal dysplasia that resembles acromegaly (p. 1367)
Pyle disease
Amyloid is derived from an inherited abnormality of serum protein __ (p. 1468)
transthyretin
Two novel treatment for amyloid (p. 1369)
Tafamidis
Interfering RNA therapy
Ischemic condition resulting from thrombosis of the subclavian artery or vein (p. 1372)
Paget- Schrotter syndrome
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)
TRUE
Nerve damaged in meatpackers neuropathy (p. 1376)
Suprascapular nerve
from carrying objectes on teh shoulder
Sign that consists of hyperflexion of the wrist for 30 to 60s (p. 1377)
Phalen maneuver
lighlty tapping the volar aspect of the rist ast the transverse carpal ligament (p. 1377)
Tinel sign
Uppper vs Lower lumbar plexus (p. 1378)
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
Most common cause of femoral neuropathy (p. 1381)
diabetes
Most frequently compressed nerves in order (p. 1382)
median ulnar peroneal tibial plantar