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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

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neurology case

Neurology Case

Omar Jawdat, MD

9/14/2012

cc and hpi
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
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
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+
examination1
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.
work up
Work Up
  • CT Chest: -ve for masses or LAP
  • CT Abd: -ve for masses or LAP
slide7

Where?

  • What?
ncs emg
NCS/EMG
  • Severe axonal demyelinatingsensorimotor neuropathy. There is marked demyelination

2. Right peroneal neuropathy at the knee

differential dx
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
clinical spectrum of cidp
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
variants of cidp
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
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
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
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