Sensory chronic inflammatory demyelinating polyneuropathy: An under-recognized entity?
Article first published online: 30 AUG 2013
Copyright © 2013 Wiley Periodicals, Inc.
Muscle & Nerve
Volume 48, Issue 5, pages 727–732, November 2013
How to Cite
Ayrignac, X., Viala, K., Koutlidis, R. M., Taïeb, G., Stojkovic, T., Musset, L., Léger, J.-M., Fournier, E., Maisonobe, T. and Bouche, P. (2013), Sensory chronic inflammatory demyelinating polyneuropathy: An under-recognized entity?. Muscle Nerve, 48: 727–732. doi: 10.1002/mus.23821
- Issue published online: 28 OCT 2013
- Article first published online: 30 AUG 2013
- Accepted manuscript online: 20 FEB 2013 03:07AM EST
- Manuscript Accepted: 12 FEB 2013
- chronic idiopathic axonal polyneuropathy;
- chronic immune demyelinating polyneuropathy;
- sensory CIDP;
- chronic immune sensory polyradiculopathy;
- somatosensory evoked potentials
Sensory chronic inflammatory demyelinating polyneuropathy (CIDP) can be difficult to diagnose.
We report 22 patients with chronic sensory polyneuropathy with ≥1 clinical sign atypical for chronic idiopathic axonal polyneuropathy (CIAP) but no electrodiagnostic criteria for CIDP.
Clinical signs atypical for CIAP were: sensory ataxia (59%), generalized areflexia (36%), cranial nerve involvement (32%), rapid upper limb involvement (40%), and age at onset ≤55 years (50%). Additional features were: normal sensory nerve action potentials (36%), abnormal radial/normal sural pattern (23%), abnormal somatosensory evoked potentials (SSEPs) (100%), elevated cerebrospinal fluid (CSF) protein (73%), and demyelinating features in 5/7 nerve biopsies. Over 90% of patients responded to immunotherapy. We conclude that all patients had sensory CIDP.
Sensory CIDP patients can be misdiagnosed as having CIAP. If atypical clinical/electrophysiologic features are present, we recommend performing SSEPs and CSF examination. Nerve biopsy should be restricted to disabled patients if other examinations are inconclusive. Muscle Nerve 48:727–732, 2013