| Background: Generally,inflammatory demyelinating diseases occur in two categories: those that affect only the central nervous system(CNS),and those that affect only the peripheral nervous system(PNS).However,there is a demyelinating condition involving both the CNS and the PNS simultaneously or temporarily separated,namely,combined central and peripheral demyelination(CCPD).In these patients,there may be antibodies against both the central nerves and the peripheral nerves.CCPD was defined by the characteristics including brain or spinal cord T2 high-signal intensity lesions in MR images or abnormalities in visual evoked potentials,suggesting demyelination in the central nervous system,and conduction delay,conduction block,F-wave or H reflex abnormalities in nerve conduction studies,implicating demyelination in the peripheral nervous system,and ruled out secondary demyelination.In 1979,a case of multiple sclerosis with peripheral neuropathy was reported for the first time.In 1987,the combined central and peripheral demyelination was named for the first time.In 2013,it was first reported that the positive rate of anti-NF155 antibodies in CCPD patients was high.In 2016,there have been clinical studies with larger sample size.31 cases of CCPD patients were reported in Italy and 40 cases of CCPD patients were reported in Japan.In 2018,Chinese scholars conducted the first clinical research on CCPD.However,this study did not focus on the detection of nodal/paranodal antibodies and myelin-related antibodies.The study was to explicate the clinical characteristics and evaluate the existence of relavant antibodies in patients with CCPD.Methods: According to the criteria for inclusion and exclusion of CCPD,a total of 31 patients with CCPD were included in this study.We reviewed the demographic characteristics,course features,clinical manifestations and signs,laboratory examinations including hematology and cerebrospinal fluid examinations,electrophysiological examinations,and neuroimaging findings in 31 patients with CCPD.We also followed up the patients and recorded the medication status and prognosis.Using a live cell-based assay,we tested antibodies against aquaporin 4(AQP4),myelin oligodendrocyte glycoprotein(MOG),neurofascin-155(NF155),neurofascin-186(NF186),myelin-associated glycoprotein(MAG),myelin basic protein(MBP)and so on in these patients.Continuous variables are shown as the means±SD,and categorical variables are presented as counts and percentages.Results: The age of onset was 38.9 ± 14.3 years.Females accounted for 54.8% of patients.The onset mode was acute in 64.5%,subacute in 6.5%,and chronic in 29.0%of patients.For clinical courses,3(10.7%)were monophasic,9(32.1%)were relapsing-remitting,11(39.3%)were chronic progressive,and 5(17.9%)were rapidly progressive.Ten(33.3%)patients had antecedent infections.The most common symptoms were motor weakness(83.3%),hyporeflexia(63.3%),and sphincter disturbance(58.1%).Although cerebrospinal fluid protein levels were increased in 60.0% of patients,we revealed oligoclonal Ig G bands in only 36.0% of patients.While only 16.6% of patients had impaired vision symptoms,33.3% patients had abnormal VEPs.A total of 71.0% of patients had an EDSS score greater than 6.21.1%(4/19)patients were positive for anti-AQP4 antibodies,20.0%(2/10)were positive for anti-NF155 antibodies,and 10.0%(1/10)patients were positive for anti-MAG antibodies.No other antibodies were detected.In this study,patients with CCPD who were treated with intravenous corticosteroids,intravenous immunoglobulin,and rituximab were reported to be effective.At the illness peak,75%patients with CCPD had m RS score of 4 or more,and in remission,37.5% patients had m RS score of 4 or more.Conclusions: Patients suspected of having CCPD are recommended to undergo brain and spinal MRI as well as electrophysiological examinations and visual-evoked potentials examinations.The anti-AQP4 antibodies and anti-nodal/paranodal antibodies are also recommeded to test. |
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