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Keywords:

  • chronic idiopathic axonal polyneuropathy;
  • chronic immune demyelinating polyneuropathy;
  • sensory CIDP;
  • chronic immune sensory polyradiculopathy;
  • somatosensory evoked potentials

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. Muscle Nerve 48:727–732, 2013