Clinical Analysis Of Acute Disseminated Encephalomyelits And Its Variants In Adults

BackgroundAlthough Acute disseminated encephalomyelitis?ADEM?like diseases have been described by Clifton as early as 1724,there are no uniform criterion for their definition,classification,diagnoses and treatment strategies.Traditionally,ADEM is considered to be a monophasic inflammatory demyelinating disease of the central nervous system in children after infection or vaccination.However,more and more cohort studies have found that the proportion of adult ADEM,idiopathic ADEM and multiphasic ADEM are increased gradually in recent years,so the definition is not completely consistent with that of adult patients.In 2013,Marchioni E et al.^[2]reported the results of two-year follow-up of 176 adult patients with postinfectious nervous system syndrome?PINSs?.ADEM was considered to be a subgroup of PINSs,including peripheral nervous system involvement,heterogeneity and chronic progressive variants.ObjectiveOur study aims to understand the clinical characteristics of adult ADEM and its variants,to compare the differences between monophasic and multiphase ADEM in order to find out the recurrence factors of multiphasic patients,and to explore the electrophysiological characteristics of ADEM combined with peripheral neuropathy.MethodsNinety-one adult patients with ADEM who visited the Department of Neurology of Henan Provincial People's Hospital from January 2015 to January 2018 were recruited,and a total of 56 patients were included after re-evaluation by an experienced clinical associate chief physician in our hospital according to the inclusion criteria.Demographic data,clinical manifestations,laboratory characteristics,imaging findings of all patients,and electrophysiological characteristics of some patients were analyzed.All data were statistically analyzed using SPSS 19.0.Count data were expressed as percentages,and measurement data were expressed as medians;?2 test for two independent samples or?2 test for row×list were performed for each count data?percentage?,when comparing monophasic and multiphase acute disseminated encephalomyelitis data;Mann-Whitney U test was used for comparison of continuous variables,and statistical significance was considered when P<0.05.ResultsIn 56 patients,the male:female ratio was 1:0.8,multiphase ratio was about 23%,and multiphase cases were more common in female patients,and the difference was statistically significant.In the first course,twenty-nine cases?52%?had precursor events before onset,the commonest presenting symptoms and signs were fever?36%?,disturbance of consciousness?52%?,mental disorder?38%?,seizure?14%?,headache and dizziness?43%?,optic neuritis?34%?,autonomic nervous symptom?52%?,Limb paralysis or abnormal sensation?73%?,unilateral or bilateral pyramidal tract sign?48%?and cerebellar ataxia?23%?.The incidence of optic nerve and/or autonomic nerve damage in multiphasic patients was higher than that in uniphasic patients,and the difference was statistically significant.Immunotherapy was given to all patients at the first onset.At the first follow-up,38 patients?68%?were fully recovered,9 patients?16%?had neurological deficits,6 patients?11%?died of severe pulmonary infection,and 3 patients?5%?died of brain hernia in acute hemorrhagic leukoencephalitis.Cerebrospinal fluid examination showed that oligoclonal bands were transient positive,and oligoclonal bands were mostly positive in patients with heterogeneity,the difference was statistically significant.Magnetic resonance examination showed that ADEM was the most common brain type in adults.Gray matter and spinal cord of multiphasic patients were more vulnerable to damage than that of uniphasic patients,and multiphasic patients had a wider range of involvement.Most of the lesions involved both supratentorial and infratentorial tissue.Neurophysiological examination was perfected in 23 patients?41%?,of whom 6 patients showed polyradiculoneuropathy?2of whom had no clinical symptoms of peripheral nerve damage?and 2 showed neurogenic damage;4 patients underwent reexamination,2 patients showed improvement in electromyography,and 2 patients showed normal electromyography.Conclusion1.In clinical practice,it is not difficult to diagnose ADEM in typical adults,but it is relatively difficult to diagnose ADEM in those cases with clinical manifestation variants,so detailed and complete auxiliary examination and regular follow-up are very important for diagnosis and classification.2.Giving multiphasic ADEM immunotherapy as early as possible can reduce the risk of recurrence,so for patients with relapse-related clinical features,such as optic nerve damage,autonomic nerve damage,transient positivity of oligoclonal bands,and lesions appearing simultaneously in cortical gray matter,spinal cord,and supratentorial and infratentorial part on cranial MRI,which suggest that conventional immunotherapy should be given.3.The electrophysiology of ADEM case with peripheral neuropathy showed mostly demyelinating lesions,suggesting that the central and peripheral nervous systems have some common components.