Sensory chronic inflammatory demyelinating polyneuropathy: An under-recognized entity?

Autor: Emmanuel Fournier, Xavier Ayrignac, Karine Viala, L. Musset, Thierry Maisonobe, Guillaume Taieb, Régine Morizot Koutlidis, Jean-Marc Léger, Tanya Stojkovic, Pierre Bouche
Rok vydání: 2013
Předmět:
Zdroj: Muscle & Nerve. 48:727-732
ISSN: 0148-639X
DOI: 10.1002/mus.23821
Popis: Introduction Sensory chronic inflammatory demyelinating polyneuropathy (CIDP) can be difficult to diagnose. Methods 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. Results 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. Conclusions 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
Databáze: OpenAIRE