The clinical features of combined central and peripheral demyelination in Chinese patients

Abstract

BackgroundCombined central and peripheral demyelination (CCPD) is rare and has never been reported as a spectrum disease in Han Chinese population. ObjectivesTo study the clinical features of CCPD in Han Chinese patients. MethodsTwenty-two CCPD patients were selected from 788 demyelination cases. We reviewed and compared the clinical manifestation, laboratory data, electrophysiological examination, MRI and the prognosis. ResultsCCPD patients presented with sensory disturbance (86.4%), plegia (77.3%), cranial nerve involvement (77.3%), abnormal deep tendon reflexes (72.7%). CSF data showed increased CSF protein in 81% patients. Oligoclonal IgG bands (OB) were negative. Cortical or juxtacortical, periventricular, infratentorial lesions, thoracic and cervical spinal cord were mostly affected. Visual evoked potentials indicated optic nerves demyelinating in 50% cases. 21 CCPD patients were treated with intravenous immunoglobulin or steroids or both of them, and the efficacy was 33.3%, 54.5%, 71.4%, respectively. One case that showed no response to steroids plus intravenous immunoglobulin treatment was improved significantly after using cyclophosphamide. ConclusionsCCPD is a spectrum disease that can't be regarded as a simple combination of MS and CIDP. A suspected CCPD should receive brain and spinal MRI as well as electrophysiological examination to obtain a precise diagnosis.