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Neuropathy Morning Report January 5 th , 2010

Neuropathy Morning Report January 5 th , 2010. Summary. Recognizing neuropathy Classifying neuropathy Myriad neuropathies CIDP Diagnosis Spectrum of disease Treatment . Clues a neuropathy is present:. History: Distal motor neuropathy: tripping over toes, dropping coffee cups.

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Neuropathy Morning Report January 5 th , 2010

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  1. Neuropathy Morning Report January 5th, 2010

  2. Summary • Recognizing neuropathy • Classifying neuropathy • Myriad neuropathies • CIDP • Diagnosis • Spectrum of disease • Treatment

  3. 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, lancinatingpain, paresthesias, dysesthesias, a tight band-like sensation around wrists or ankles, hypesthesia, restless legs, numbness. • Acute vs. chronic

  4. 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: • pescavus, kyphoscoliosis, loss of hair, ulceration, thinning of phalanges, charcot joints (neurogenicarthropathy).

  5. Ordesignslv.com Mda.org

  6. Myfootshop.com Thefootblog.org

  7. 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)

  8. 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

  9. Ddx: Neuropathy • Carpal tunnel, ulnar, … • Myxedema, • RA, Acromegaly • Leprosy • Borreliosis (Lyme) • HIV

  10. Demyelinating • Guillain-Barré • CIDP

  11. 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

  12. 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.

  13. 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 IgMparaproteinemia. • 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 antigangliosideantiboties. • Axonal chronic inflammatory demyelinatingpolyneuropathy, pure sensory chronic inflammatory demyelinatingpolyneuropathy, multifocal acquired sensory and motor axonopathy (MASAM).

  14. Treatment • Goal: block the immune process • Course: continue until maximal improvement and then maintain. • IVIg: 0.4g/kg qdayx 5 days, then monthly x 3 • Plasma exchange • Corticosteroids • Mycophenolatemofetil, azothioprine, cyclophosphamide, etanercept, rituximab

  15. 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.

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