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Neurology Case

Neurology Case. Omar Jawdat , MD 9/14/2012. CC and HPI. CC: 68 yo F weakness in all 4 extremities 7/2010: right biceps myalgia followed by right shoulder and hand weakness 2 wk later right foot drop 9/2010: weakness all 4 extremities, can not walk, numbness on feet and fingers

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Neurology Case

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  1. Neurology Case Omar Jawdat, MD 9/14/2012

  2. CC and HPI • CC: 68 yo F weakness in all 4 extremities • 7/2010: right biceps myalgia followed by right shoulder and hand weakness • 2 wk later right foot drop • 9/2010: weakness all 4 extremities, can not walk, numbness on feet and fingers • Rx with IVIG with good response for 5 wks • 2-3 relapses, Rx with IVIG in 3-5 wk intervals

  3. HPI, PMH, FHx and SHx • C-Spine MRI shows severe central stenosis C6-C7  Surgical decompression 1/2011 • Post op, patient is worse, IVIG, Decadron, PLEX and then transferred to KU on 2/2011 PMH: • HTN, Hypothyroidism, GERD, right IJ venous thrombus on Coumadin FHx/SHx: • No family history of neurologic conditions, homemaker, quit smoking 2001, drinks occasionally, No recreational drugs

  4. Examination • MS: A & O x 4 • Speech: Fluent without dysarthria • CN: RERRLA, Full Fields, EOMI, normal sensation, palpebral fissure 8/9mm, no ptosis, Orb Oculi 4, Orb Oris 5-. Palatal elevation is symmetric. Sternomastoid and trapezii are 5. Normal tongue with no fasciculation, power 5. • Motor: • Normal tone, bulk, no atrophy or fasciculation. Neck Flexion 3+

  5. Examination • Sensory: diminished LT/PP in feet Vibration: R/L toes 0, ankles 3/0, lower tibia 3/2 sec fingers 3 sec. Proprioception intact • DTR: 0 all over, Flexor planter response, -ve Hoffman, -vejaw jerk. • Coordination and gait: too weak to perform.

  6. Work Up • CT Chest: -ve for masses or LAP • CT Abd: -ve for masses or LAP

  7. Where? • What?

  8. NCS/EMG • Severe axonal demyelinatingsensorimotor neuropathy. There is marked demyelination 2. Right peroneal neuropathy at the knee

  9. Differential Dx • MADSAM (Multifocal acquired demyelinating sensory motor neuropathy) • MMN (multifocal motor neuropathy) • POEMS • Mononeuritismuliplex • Classic CIDP • Infectious and infiltrative processes such as HIV, Lyme, sarcoidosis • Neoplastic invasion of spinal roots by lymphoma • HNPP

  10. Clinical Spectrum of CIDP • CIDPs form a heterogeneous group of NP that share one common feature, demyelination of peripheral nerves • First reported by James Austin, MD in 1958, over 50 years after Guillan, Barréand Strohl described Acute ascending paralysis • Classification is based on clinical features, lab findings and associated systemic disease

  11. Classification by clinical pattern

  12. Variants of CIDP • Temporal variants - SIDP (subacute presentation) • Concurrent illness variants HIV, Lymphoma, POEMS, MGUS, Hep C, IBD, connective tissue disease • Distribution variants DADS neuropathy, MADSAM neuropathy, MMN and classic CIDP

  13. MADSAM • Also called Lewis-Summer syndrome • Unlike MMN but similar to CIDP, CSF protein is elevated • Asymmetric pattern of sensory and motor deficits and like MMN, it starts distal. • Electrophysiologic evidence of sensory and motor nerve involvement, motor demyelination, TD and motor conduction block

  14. Treatment • Responds to IVIG • Responds to Corticosteroids as opposed to MMN patients • Our patient responded very well to PLEX acutely with IVIG and IV methylprednisone • Currently on Prednisone, IVIG q4wk and CellCept

  15. Conclusion • This case reports a patient with chronic progression of asymmetric numbness and weakness in four extremities. • MADSAM neuropathy was diagnosed after clinical and laboratory evaluations and to look for other possible differential • It is very important to distinguish between CIDP, MADSAM neuropathy, and MMN by clinical, laboratory, and electrodiagnostic features because of different effective therapeutic strategies

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