1 / 15

Neurology Case

Neurology Case. Omar Jawdat , MD 9/14/2012. CC and HPI. CC: Elderly woman with weakness in all 4 extremities Initial complaint : right biceps myalgia followed by right shoulder and hand weakness 2 wk later right foot drop

effie
Download Presentation

Neurology Case

An Image/Link below is provided (as is) to download presentation Download Policy: Content on the Website is provided to you AS IS for your information and personal use and may not be sold / licensed / shared on other websites without getting consent from its author. Content is provided to you AS IS for your information and personal use only. Download presentation by click this link. While downloading, if for some reason you are not able to download a presentation, the publisher may have deleted the file from their server. During download, if you can't get a presentation, the file might be deleted by the publisher.

E N D

Presentation Transcript

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

  2. CC and HPI • CC: Elderly woman with weakness in all 4 extremities • Initial complaint: right biceps myalgia followed by right shoulder and hand weakness • 2 wk later right foot drop • 2 months later: 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

More Related