Combined central and peripheral demyelination: Clinical features, diagnostic findings, and treatment

A. Cortese, D. Franciotta, E. Alfonsi, N. Visigalli, E. Zardini, L. Diamanti, P. Prunetti, C. Osera, M. Gastaldi, G. Berzero, A. Pichiecchio, G. Piccolo, A. Lozza, G. Piscosquito, E. Salsano, M. Ceroni, A. Moglia, G. Bono, D. Pareyson, E. Marchioni

Research output: Contribution to journalArticle

Abstract

Combined central and peripheral demyelination (CCPD) is rare, and current knowledge is based on case reports and small case series. The aim of our study was to describe the clinical features, diagnostic results, treatment and outcomes in a large cohort of patients with CCPD. Thirty-one patients entered this retrospective, observational, two-center study. In 20 patients (65%) CCPD presented, after an infection, as myeloradiculoneuropathy, encephalopathy, cranial neuropathy, length-dependent peripheral neuropathy, or pseudo-Guillain-Barré syndrome. Demyelinating features of peripheral nerve damage fulfilling European Federation of Neurological Societies/Peripheral Nerve Society (EFNS/PNS) electrodiagnostic criteria for CIDP were found in 23 patients (74%), and spatial dissemination of demyelinating lesions on brain MRI fulfilling the 2010 McDonald criteria for multiple sclerosis (MS) in 11 (46%). Two thirds of the patients had a relapsing or progressive disease course, usually related to the appearance of new spinal cord lesions or worsening of the peripheral neuropathy, and showed unsatisfactory responses to high-dose corticosteroids and intravenous immunoglobulins. The clinical presentation of CCPD was severe in 22 patients (71%), who were left significantly disabled. Our data suggest that CCPD has heterogeneous features and shows frequent post-infectious onset, primary peripheral nervous system or central nervous system involvement, a monophasic or chronic disease course, inadequate response to treatments, and a generally poor outcome. We therefore conclude that the current diagnostic criteria for MS and CIDP may not fully encompass the spectrum of possible manifestations of CCPD, whose pathogenesis remains largely unknown.

Original languageEnglish
Pages (from-to)182-187
Number of pages6
JournalJournal of the Neurological Sciences
Volume363
DOIs
Publication statusPublished - Apr 15 2016

Keywords

  • Acute disseminated encephalomyelitis
  • Chronic inflammatory demyelinating polyneuropathy
  • Combined central and peripheral demyelination
  • Guillain-Barré syndrome
  • Multiple sclerosis

ASJC Scopus subject areas

  • Clinical Neurology
  • Neurology

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