Journal
JOURNAL OF NEUROIMMUNOLOGY
Volume 317, Issue -, Pages 32-36Publisher
ELSEVIER SCIENCE BV
DOI: 10.1016/j.jneuroim.2018.02.006
Keywords
Combined central and peripheral demyelination (CCPD); Multiple sclerosis (MS); Neuromyelitis optical spectrum disorders (NMOSD); Chronic inflammatory demyclinating polyneuropathy (CIDP); Guillain-Barre syndrome (GBS)
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Background: Combined central and peripheral demyelination (CCPD) is rare and has never been reported as a spectrum disease in Han Chinese population. Objectives: To study the clinical features of CCPD in Han Chinese patients. Methods: Twenty-two CCPD patients were selected from 788 demyelination cases. We reviewed and compared the clinical manifestation, laboratory data, electrophysiological examination, MRI and the prognosis. Results: CCPD 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 (08) 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. Conclusions: CCPD 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.
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