Clinically and electrodiagnostically pure sensory demyelinating polyneuropathy

A 35-year-old woman presented with a 4 year history of stepwise sensory loss which progressed in an asymmetrical fashion (mononeuropathy multiplex) and developed into a relatively symmetric polyneuropathy which was purely sensory by clinical and electrodiagnostic criteria. Sural nerve biopsy revealed demyelination with axonal sparing. Extensive laboratory evaluation failed to reveal a definite cause. This case is unique among well-documented cases of chronic inflammatory demyelinating polyneuropathy (CIDP) in that it is purely sensory by both clinical and electrodiagnostic criteria, and may represent a distinct entity rather than an extension of the spectrum of CIDP.