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 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

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

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+ SAbEFEEWFWEFEFFFAbHFKFKEADFAPFTE

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, -ve jaw jerk. Coordination and gait: too weak to perform.

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

Where? What?

NCS/EMG 1.Severe axonal demyelinating sensorimotor neuropathy. There is marked demyelination 2. Right peroneal neuropathy at the knee

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

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, Barré and Strohl described Acute ascending paralysis Classification is based on clinical features, lab findings and associated systemic disease

Classification by clinical pattern Clinical featureClassic CIDPDADSMADSAMMMN Weakness and Sensory loss Symmetric, distal and proximal Symmetric, mild distal Asymmetric, mostly distal Asymmetric, purely motor, mostly distal Elevated CSF Protein Yes No M ProteinUncommon, IgG or IgA Usually IgM, rarely IgG UncommonUncommon, rarely IgM Antineural Antibodies UncommonAnti MAG in 50% of patients UncommonAnti GM1 Motor NCSDemyelination Sensory NCSAbsent or small SNAPs Normal TreatmentPrednisone, IVIG, PLEX, Immunosuppre ssants Possibly Rituximab, IVIG for patients without Anti- MAG IVIG, Prednisone, Immunosupress ants IVIG, possibly Cyclophospham ide

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

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

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

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