1. Neuropathy Morning Report January 5 th, 2010

2.  Recognizing neuropathy  Classifying neuropathy  Myriad neuropathies  CIDP  Diagnosis  Spectrum of disease  Treatment

3.  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

4.  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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7.  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.  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

11. Diabetes Hypothyroidism Metabolic Alcohol Lead Platinum chemo, tacrolimus amiodarone, chloroquine. Toxic Sarcoidosis Vasculitis: RA, SLE Non-systemic vasculitis InflammatoryInfectious B12 (cyanocobalamine), B6 (pyridoxine), B1 (thiamine) E (alpha-tocopherol) Nutritional Deficiency Carcinoma-associated Lymphoma-associated Monoclonal gammopathy Paraneoplastic SMA CMT ALS HereditaryCompressive Leprosy Borreliosis (Lyme) HIV Carpal tunnel, ulnar, … Myxedema, RA, Acromegaly

12. Guillain-Barré CIDP Demyelinating

13.  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

14.  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.

15.  Distal Acquired Demyelinating Symmetric (DADS) Neuropathy  Men over 50  Mostly distal sensory loss, mild distal weakness, unsteady gait. 2/3 rds 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).

16.  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

17.  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 15 th, 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.