Abstract Details

Title Diagnostic Value of MRI in CIDP

Topic Peripheral Nerve

Presentation(s) P07 - (-)

Poster/Presentation
Number 055

Objective

Background BACKGROUND: CIDP is a treatable, relapsing, immune mediated disorder, which can be challenging to diagnose. Traditionally, clinical presentation, CSF analysis, and electro-diagnostic studies are sufficient to evaluate patients with peripheral sensorimotor deficits. Recently, imaging has gained importance in providing supplemental information to identify CIDP. Hypertrophy and gadolinium enhancement have been noted in multiple nerve roots, with variable clinical correlation. We present a patient with undiagnosed CIDP and massive hypertrophy of multiple nerve roots.

Design/Methods DESIGN/METHODS: A 62 y/o male presented with progressive weakness in all four extremities, and bilateral foot drop over an 8 year period. On exam, he had normal cranial nerve function, significant atrophy, paresis in all extremities (distal > proximal, legs > arms), symmetrically decreased ankle jerks, no ataxia, and no plantar responses with stimulation. Clinical evaluation was consistent with a severe length dependent sensorimotor peripheral neuropathy.

Results RESULTS: Labs for etiologies of neuropathy were all within normal limits. Initial EMG/NCS was limited due to severe distal nerve damage. MRIs showed thickened and enhancing CN V1-V3 segments, mastoid segments of CN VII, the jugular nerve complex, the entire brachial plexus, as well as the intradural, foraminal, and extraforaminal segments of cervical and lumbar nerve roots. His extensive lumbar root hypertrophy prevented a successful lumbar puncture for CSF protein analysis. Two rounds of 2 grams/kg of IVIG were administered with minimal improvement. Repeat EMG/NCS 6 months later, with dedicated testing of the cranial nerves, confirmed the diagnosis of CIDP with predominantly demyelinating sensorimotor polyneuropathy.

Conclusions CONCLUSIONS: CIDP can be challenging to diagnose, making MRIs necessary for appropriate diagnosis, especially if classic diagnostic tools (CSF and EMG/NCS) are initially unyielding. The identification of multiple hypertrophied nerve roots may also be indicative of refractoriness to immunotherapies.

Authors/Disclosures
Forough Ghavami, DO (NuVasive Clinical Services) PRESENTER	No disclosure on file
Rebecca O'Dwyer, MD (Rush University Medical Center)	Dr. O'Dwyer has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for UCB Pharma. Dr. O'Dwyer has received personal compensation in the range of $5,000-$9,999 for serving on a Speakers Bureau for SK Life Sciences.
Marshall S. Balish, MD, PhD	No disclosure on file
Ping Zhai, MD (VA Medical Center)	No disclosure on file
Alexander U. Brandt, MD (Charite - Universitatsmedizin Berlin)	No disclosure on file

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