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

Muscle Nerve. 2013 Nov;48(5):727-32. doi: 10.1002/mus.23821. Epub 2013 Aug 30.


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.

Keywords: chronic idiopathic axonal polyneuropathy; chronic immune demyelinating polyneuropathy; chronic immune sensory polyradiculopathy; sensory CIDP; somatosensory evoked potentials.

MeSH terms

  • Adult
  • Aged
  • Diagnosis, Differential
  • Evoked Potentials, Somatosensory / physiology*
  • Female
  • Humans
  • Male
  • Middle Aged
  • Polyneuropathies / diagnosis
  • Polyneuropathies / pathology
  • Polyneuropathies / physiopathology*
  • Polyradiculoneuropathy, Chronic Inflammatory Demyelinating / diagnosis*
  • Polyradiculoneuropathy, Chronic Inflammatory Demyelinating / pathology
  • Polyradiculoneuropathy, Chronic Inflammatory Demyelinating / physiopathology*
  • Retrospective Studies
  • Severity of Illness Index