Seven children with acute disseminated encephalomyelitis (ADEM) after treatment with Japanese B encephalitis vaccine (JBEV) (Nakayama-Yoken strain 1968-88 and Beijing strain 1989-93) were identified by mailed questionnaires and by compilation of previously published case reports. It was considered that encephalomyelitis might have been related to vaccine treatment as the vaccine is derived from mouse brain tissue infected with Japanese B encephalitis virus, a potentially cross reactive antigen. The incidence of severe neurological complications associated with the newer Japanese B encephalitis Beijing strain vaccine seemed to be less than one case per 1 000 000, which is similar to the incidence of neurological complications associated with the older NakayamaYoken strain vaccine. (J Neurol Neurosurg Psychiatry 1995;59:316-317) Keywords: acute disseminated encephalomyelitis; Japanese B encephalitis vaccine due to JBEV Beijing and Nakayama-Yoken strains in an effort to update the study by Okinaka et al.5Patients and methods The questionnaire was intended to screen for potential patients with neurological complications after treatment with JBEV. Forms were mailed to 162 medical institutions including paediatric departments in medical colleges, schools of medicine, children's hospitals, and rehabilitation facilities for children, all facilities that might be served by paediatic neurologists. Acute disseminated encephalomyelitis was defined, for the purposes of the questionnaire, as an acute inflammatory disease with perivenous lymphocytic infiltration and scattered demyelinating foci in the white matter of the CNS. The presence of characteristic CNS lesions was confirmed by CT or MRI. The symptoms of ADEM must have appeared abruptly within one month after vaccination. Other similar neurological conditions must have been ruled out. We also identified cases of neurological complications associated with JBEV from the Japanese and English medical literature.
Seven muscle biopsies from patients with the clinical characteristics of Marinesco-Sjögren syndrome (MSS) revealed myopathic changes of two types; muscle fiber necrosis followed by regeneration and focal myofibrillar degeneration inducing autophagocytosis with rimmed vacuole formation. In two young patients, massive muscle fiber necrosis with phagocytic invasion was the predominant feature and autophagic phenomenon was minimal, resembling the findings in progressive muscular dystrophy. Myofibrillar degeneration with autophagic phenomenon was prominent in five adult patients. The coexistence of these two degenerative processes and the secondarily induced reactive changes of muscle fiber hypertrophy, interstitial fibrosis, occasional ragged-red fibers and type 1 fiber predominance, are responsible for the wide spectrum of muscle pathology in MSS. The dense double-membrane structure surrounding myonuclei, previously reported as being specific to MSS, was present in only one biopsy.
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