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.
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 CIDPpatients 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.
Authors: Anne Louise Oaklander; Michael Pt Lunn; Richard Ac Hughes; Ivo N van Schaik; Chris Frost; Colin H Chalk Journal: Cochrane Database Syst Rev Date: 2017-01-13
Authors: Shahar Shelly; Kamal Shouman; Pritikanta Paul; JaNean Engelstad; Kimberly K Amrami; Robert J Spinner; Divyanshu Dubey; Rocio Vazquez Do Campo; Peter J Dyck; Christopher J Klein; P James B Dyck Journal: Neurology Date: 2021-03-02 Impact factor: 9.910