Chronic progressive steroid responsive axonal polyneuropathy: A CIDP variant or a primary axonal disorder?

Antonino Uncini, Mario Sabatelli, Teresa Mignogna, Alessandra Lugaresi, Rocco Liguori, Pasquale Montagna

Research output: Contribution to journalArticlepeer-review

Abstract

Five patients presented with chronic, progressive, predominantly motor polyneuropathy, CSF protein content was increased in 4 patients. Motor conduction velocities and EMG were consistent with axonal involvement. Sural nerve conductions were normal in all cases and sural nerve biopsy performed in 1 patient was normal. Serum antibodies to GM1, GD(1a), GD(1b), and GM2 were negative. All patients improved after steroid treatment and 3 completely recovered. Because of therapeutic implications it is important to differentiate these patients from those with chronic idiopathic axonal neuropathies. It is unclear whether this is a primary axonal, probably immune-mediated, polyneuropathy, or whether it represents one extreme of the chronic inflammatory demyelinating polyradiculoneuropathy spectrum characterized by severe axonal loss. We suggest that the term 'chronic inflammatory polyneuropathy,' encompassing cases from pure demyelinating to pure axonal neuropathies responsive to steroids, should be reinstated and that, like in Guillain-Barre syndrome, different subtypes should be individuated.

Original languageEnglish
Pages (from-to)365-371
Number of pages7
JournalMuscle and Nerve
Volume19
Issue number3
DOIs
Publication statusPublished - Mar 1996

Keywords

  • antiganglioside antibodies
  • chronic axonal polyneuropathy
  • chronic idiopathic neuropathy
  • chronic inflammatory demyelinating polyradiculoneuropathy
  • steroid treatment

ASJC Scopus subject areas

  • Clinical Neurology
  • Neuroscience(all)

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