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NeuropathyMorning Report January 5th, 2010
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Summary
Recognizing neuropathy Classifying neuropathy Myriad neuropathies CIDP
Diagnosis Spectrum of disease Treatment
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Clues a neuropathy is present: History:
Distal motor neuropathy: ▪ tripping over toes, dropping coffee cups.
Proximal motor neuropathy:▪ trouble climbing stairs, getting up from sitting,
raising arms to brush teeth and hair. Sensory neuropathy: ▪ burning, lancinating pain, paresthesias, dysesthesias,
a tight band-like sensation around wrists or ankles, hypesthesia, restless legs, numbness.
Acute vs. chronic
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Clues a neuropathy is present: Physical Exam:
Motor Strength: ▪ 5= full strength, 4= falls to moderate resistance, 3= falls
to minimal resistance, 2= falls to gravity, 1= muscle contraction, 0= no movement.
Sensation: ▪ vibration/proprioception (large myelinated axons) vs.
pain/temperature (small myelinated axons)▪ Light touch shared by both fiber types.
Trophic Changes: ▪ pes cavus, kyphoscoliosis, loss of hair, ulceration,
thinning of phalanges, charcot joints (neurogenic arthropathy).
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Mda.org
Ordesignslv.com
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Myfootshop.com
Thefootblog.org
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What type of neuropathy? Neuronopathy
Cell body damage of the motor neuron or dorsal root ganglion.
SMA, ALS, CMT (sometimes). Axonopathy
Traumatic, toxic, metabolic Often length dependent- “die back”: symmetric,
distal. Myelinopathy
Hereditary or inflammatory Non-length dependent. ▪ Patchy or segmental (inflammatory)▪ Diffuse (hereditary)
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Electromyelogram (EMG) andNerve Conduction Studies (NCS)
Axonopathy Loss of amplitude of
AP Evidence of
denervation- fasciculation
Myelopathy Slowed conduction Prolonged distal
latency Conduction block Temporal dispersion Prolonged minimum
f-wave latency.
Ispub.com
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Ddx: Neuropathy
•Diabetes•Hypothyroidism
Metabolic
•Alcohol•Lead•Platinum chemo, tacrolimus
amiodarone, chloroquine.Toxic
•Sarcoidosis•Vasculitis: RA, SLE•Non-systemic vasculitis
Inflammatory
Infectious
•B12 (cyanocobalamine), B6 (pyridoxine), B1 (thiamine)
•E (alpha-tocopherol)
Nutritional Deficiency
•Carcinoma-associated•Lymphoma-associated•Monoclonal gammopathy
Paraneoplastic
•SMA•CMT•ALS
Hereditary
Compressive• Leprosy• Borreliosis (Lyme)• HIV
• Carpal tunnel, ulnar, …• Myxedema, • RA, Acromegaly
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•Guillain-Barré•CIDP
Demyelinating
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Chronic Inflammatory Demyelinating Polyneuropathy
C = progresses over >1 month. I = presumed to have an autoimmune
origin D= demyelinating P = neuropathy that is poly-
Motor and sensory Symmetrical (or more than one limb) Non-length-dependent
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CIDP Diagnosis
American Academy of Neurology Criteria Clinical: 2 more more months,
hypo/areflexia, motor/sensory involvement, >1 limb.
EMG/NCS: 1. Conduction block, 2. decr conduction velocity, 3. incr distal latency, 4. incr F-wave latency. (Must have 3 of 4)
Pathologic: unequivocal evidence of demyelination, remyelination.
CSF: WBC < 10, VDRL neg, elevated protein.
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The Larger World of Demyelinating Polyneuropathies Distal Acquired Demyelinating Symmetric (DADS) Neuropathy
Men over 50 Mostly distal sensory loss, mild distal weakness, unsteady gait. 2/3rds
have a IgM paraproteinemia. Multifocal Motor Neuropathy (MMN)
Asymmetric, all weakness, no sensory loss Some have antiganglioside antibodies
Multifocal Acquired Demyelinating Sensory and Motor (MADSAM) Neuropathy (Lewis-Sumner Syndrome) Motor and sensory, elevated CSF protein, asymmetric, some with
antiganglioside antiboties. Axonal chronic inflammatory demyelinating polyneuropathy,
pure sensory chronic inflammatory demyelinating polyneuropathy, multifocal acquired sensory and motor axonopathy (MASAM).
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Treatment
Goal: block the immune process Course: continue until maximal
improvement and then maintain. IVIg: 0.4g/kg qday x 5 days, then
monthly x 3 Plasma exchange Corticosteroids Mycophenolate mofetil, azothioprine,
cyclophosphamide, etanercept, rituximab
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Sources Koller, H. et al. (2005) Chronic Inflammatory
Demyelinating Polyneuropathy. NEJM 352(13): 1343-1356.
Poncelet, AM. (1998) An Algorithm for the Evaluation of Peripheral Neuropathy. AFP, Feb 15th, 1998.
Said, G. (2006) Chronic Inflammatory Demyelinating Polyneuropathy. Neuromusc Disorders 16:293-303.
Sander, HW et al. (2003) Research Criteria for defining patients with CIDP. Neurology 60(Suppl 3):S8-S15.