Chronic immune sensory neuropathy
J Peripher Nerv Syst.2004 Sep;9(3):132-7.
Sensory CIDP presenting as cryptogenic sensory polyneuropathy.Chin RL; Latov N: Sander HW: Hays AP: Croul SE: Magda P: Brannagan TH 3rd
of this study was to report that patients with
chronic inflammatory demyelinating
polyneuropathy (CIDP) can present with a
clinical picture of cryptogenic sensory
neuropathy. Patients with distal sensory
neuropathy and electrodiagnostic studies that
are minimally abnormal or consistent with an
axonal pathology are usually diagnosed as having
cryptogenic sensory neuropathy if no cause for
neuropathy can be found.
Some of these patients, however, may have sensory CIDP. We reviewed the records of eight patients with CIDP, diagnosed by sural nerve biopsy, who presented with sensory neuropathy and electrodiagnostic studies that were minimally abnormal or revealed changes consistent with axonal neuropathy. All patients reported distal numbness and paresthesias and, on examination, had predominantly large fiber distal sensory loss and normal muscle strength. In most patients, deep tendon reflexes were reduced or absent. Sural nerve biopsies in all patients were consistent with chronic myelinopathy, with quantitative teased fiber analysis revealing segmental remyelination in 13-40% of the fibers. The four patients who received IVIg therapy had improved sensation and gait. Of the remaining four patients, one is being followed, one had spontaneous remission, one was lost to follow-up, and one, with contraindications to therapy, reported disease progression.
Sensory CIDP may present as cryptogenic sensory polyneuropathy with normal or axonal electrophysiologic features. Sural nerve biopsy should be considered in patients with progressive, predominantly large fiber sensory neuropathy of otherwise unknown etiology, as they may have sensory CIDP that responds to therapy.