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  • Title: Sensory chronic inflammatory demyelinating polyneuropathy: an under-recognized entity?
    Author: Ayrignac X, Viala K, Koutlidis RM, Taïeb G, Stojkovic T, Musset L, Léger JM, Fournier E, Maisonobe T, Bouche P.
    Journal: Muscle Nerve; 2013 Nov; 48(5):727-32. PubMed ID: 23424105.
    Abstract:
    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.
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