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Abstract Details

Atypical CISP: A New Variant of Chronic Immune Sensory Demyelinating Polyradiculoneuropathy
Neuromuscular and Clinical Neurophysiology (EMG)
P13 - Poster Session 13 (5:30 PM-6:30 PM)
1-001

To define and compare atypical-CISP to typical-CISP through examination, imaging, CSF testing and electrodiagnostics features.
 Sensory neuropathy with ataxia and normal nerve conduction from selective sensory nerve root inflammatory demyelination is the hallmark of chronic immune sensory polyradiculoneuropathy (CISP). In practice, many patients have broader involvement including some motor or post-ganglionic sensory nerves supported by clinical, electrophysiological, and imaging finding, i.e. atypical-CISP. 
We identified typical and atypical CISP patients utilizing a unified data platform from 1985 to 2019 of which 16,542 Mayo Clinic medical records with CIDP, polyganglionopathy or ataxia. 
We identified 24 CISP and 50 atypical-CISP cases. Median onset age for CISP=59 years (range; 30-79; 13 males) and atypical-CISP=59 years (range; 40-80; 29 males). Presenting symptoms of sensory ataxia occurred in 70% (17/24) in CISP and 40% (17/43) in atypical-CISP. Large-fiber proprioception loss was present in 95% (21/22) of CISP and 97% (41/42) of atypical-CISP. Nerve conductions were normal in all CISP cases (24/24) and minimally abnormal in 95% (40/42) of atypical-CISP, all patients having preserved sural sensory responses. Median CSF protein for CISP was 67 g/dl (range; 31-161) and 63.5 g/dl (range; 19-268) in atypical-CISP. Slowed tibial or median SSPE occurred in 74% (14/19) of CISP and in 77% (27/35) in atypical CISP. MRI nerve root thickening occurred in 22% (5/23) for CISP and in 37% (13/35) in atypical-CISP. The majority of patients improved with immune-therapy.  

Pure and atypical-CISP are clinically similar and present with sensory ataxia, large fiber abnormalities, elevated CSF proteins and are responsive to immunotherapy.  Chronic immune sensory polyradiculopathies present as a clinical spectrum with isolated involvement of sensory nerve roots (represented by pure CISP) at one end and primary involvement of peripheral nerve (represented by sensory CIDP) at the other end and atypical CISP bridging the two in the middle.

Authors/Disclosures

PRESENTER 		No disclosure on file
Peter J. Dyck, MD, FÂ鶹´«Ã½Ó³»­ (Mayo Clinic) Dr. Dyck has nothing to disclose.
Pritikanta Paul, MD (University of California, San Francisco) The institution of Dr. Paul has received research support from ZS Associates.
Christopher J. Klein, MD, FÂ鶹´«Ã½Ó³»­ (Mayo Clinic) Dr. Klein has received personal compensation in the range of $500-$4,999 for serving as a Consultant for NMD Pharma.
P. James B. Dyck, MD, FÂ鶹´«Ã½Ó³»­ (Mayo Clinic) Dr. Dyck has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Akcea/Ionis.