Subacute inflammatory demyelinating polyneuropathy

Neurology. 2003 Dec 9;61(11):1507-12. doi: 10.1212/01.wnl.0000096166.28131.4c.


Objective: To report the clinical, electrophysiologic, and histologic characteristics of subacute inflammatory demyelinating polyneuropathy (SIDP) and to present the diagnostic criteria of this disease.

Methods: For a diagnosis of "definite SIDP," there were four mandatory criteria: 1) progressive motor and/or sensory dysfunction consistent with neuropathy in more than one limb with time to nadir between 4 and 8 weeks, 2) electrophysiologic evidence of demyelination in at least two nerves, 3) no other etiology of neuropathy, and 4) no relapse on adequate follow-up. Supportive criteria included high spinal fluid protein level (>55 mg/dL) and inflammatory cells in the nerve biopsy. A diagnosis of "probable SIDP" required progression of demyelinating neuropathy over a 4- to 8-week period.

Results: Sixteen definite SIDP patients were identified among 29 probable SIDP patients. An antecedent infection was found in 38% of cases. The two most common neuropathy types were a symmetric motor-sensory neuropathy and a pure motor neuropathy. Cranial nerve deficits and respiratory failure were rare. Spinal fluid protein was high in 93% of cases. Demyelination was documented by the motor nerve conduction in 88% of cases and by the near-nerve needle sensory nerve conduction in two cases. Almost all patients were treated with prednisone and some with additional immunotherapies. Complete recovery was achieved in 69% of cases and partial recovery in others. Definite SIDP had all the characteristics of CIDP with three exceptions: a higher rate of antecedent infection, no relapse rate, and a high rate of recovery to normal.

Conclusion: Subacute inflammatory demyelinating polyneuropathy is a definite entity bridging the gap between Guillain-Barré syndrome and chronic inflammatory demyelinating polyneuropathy.

MeSH terms

  • Adolescent
  • Adult
  • Aged
  • Biopsy
  • Child
  • Child, Preschool
  • Female
  • Humans
  • Male
  • Middle Aged
  • Nerve Fibers / pathology
  • Polyradiculoneuropathy / diagnosis*
  • Polyradiculoneuropathy / pathology
  • Polyradiculoneuropathy / therapy
  • Polyradiculoneuropathy, Chronic Inflammatory Demyelinating / diagnosis
  • Treatment Outcome