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1/29. Acute disseminated encephalomyelitis in childhood: report of 10 cases.

    We report 10 children with the diagnosis of acute disseminated encephalomyelitis. diagnosis was based on clinical and radiologic findings, and after acute encephalitis was excluded by negative culture and antibody results. The most common presenting symptom was ataxia, followed by optic neuropathy, cranial nerve palsy, convulsions, motor dysfunction, and loss of consciousness. Brain magnetic resonance imaging showing bilateral symmetrical hyper-intense lesions of the same age in brain stem, subcortical white matter, thalamus, basal ganglia, or cerebellum was the mainstay of the diagnosis. The presence of a preceding event (either an infection or vaccination) was present in 8 of 10 patients. Brain computed tomographic scans were abnormal in 3 of 10, and electroencephalogram was normal in all patients. High-dose corticosteroids were given to six patients, one received low-dose steroids, and the other three had symptomatic follow-up. Those who relapsed were mainly from the symptomatic follow-up group. Only one patient (the youngest) receiving high-dose methylprednisolone relapsed. Therefore, early high-dose steroid treatment seems to be the most effective treatment in acute disseminated encephalomyelitis and can prevent relapses.
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ranking = 1
keywords = encephalitis
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2/29. Fulminant form of acute disseminated encephalomyelitis: successful treatment with hypothermia.

    We described herein a case of the fulminant form of acute disseminated encephalomyelitis (ADEM) that developed after mycoplasma pneumonia. A 28-year-old man who presented with fever, headache, and writing difficulty was admitted to our hospital in August 1997. He developed hernia on the 3rd hospital day. Surgical decompression and intravenous prednisolone failed to halt his progressive deterioration. We introduced systemic hypothermia and he has shown marked recovery; despite having Broca's type aphasia, he could comprehend spoken language and communicate with others by gesture. Head MRI demonstrated diffuse high signals over the white matter on fluid attenuated inversion recovery (FLAIR) images, which suggested extensive demyelination. The clinical course, imaging studies and presence of polymorphonuclear dominant leucocytosis in the blood and CSF in the patient are somewhat similar to findings in acute hemorrhagic leukoencephalitis, however, the result of a brain biopsy was inconclusive. The fulminant form of ADEM is usually fatal. Treatments such as corticosteroids, intravenous immunoglobulin, and surgical decompression have been performed to improve the prognosis. Our case results indicate that hypothermia, which suppresses both brain edema and immune response, may be included in the repertoire of treatment for the fulminant form of ADEM.
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ranking = 1
keywords = encephalitis
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3/29. Intravenous immunoglobulin therapy in acute disseminated encephalomyelitis.

    Three children ranging in age from 2 to 5 years with acute disseminated encephalomyelitis (ADEM) were successfully treated with high-dose intravenous immunoglobulin (IVIG). Their symptoms were somnolence, fever, headache, vomiting, and resting tremor. In all of these patients, it was difficult to distinguish the condition from viral encephalitis before analyzing the myelin basic protein. ADEM was diagnosed because of increased levels of myelin basic protein in their cerebrospinal fluid and abnormal high-signal intensity on T2-weighted magnetic resonance imaging. All patients were given IVIG at a dose of 400 mg/kg/day for 5 consecutive days. The patients rapidly regained consciousness in 14 hours, 2 days, and 4 days and demonstrated a complete clinical improvement within 18 days, 10 days, and 7 days of the initiation of the treatment, respectively. IVIG may prove useful as an alternative treatment to corticosteroids for ADEM.
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ranking = 1
keywords = encephalitis
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4/29. Spontaneous recovery from severe encephalitis involving cerebellar gray matter.

    We report a case of cerebellitis displaying a severe neurologic onset but with spontaneous recovery, challenging the use of immunomodulatory treatment.
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ranking = 4
keywords = encephalitis
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5/29. A residual cystic lesion in acute disseminated encephalomyelitis.

    We report a case of acute disseminated encephalomyelitis (ADEM) with a residual cystic lesion on MRI. This seemed to be induced by Japanese encephalitis vaccination. Despite complete clinical improvement with high-dose steroid therapy, the cystic lesion has persisted for 3 years on MRI. There have been no previous reports of residual cystic lesions in ADEM.
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ranking = 1
keywords = encephalitis
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6/29. Acute disseminated encephalomyelitis developed after acute herpetic gingivostomatitis.

    A child with acute disseminated encephalomyelitis (ADEM) developed after acute herpetic gingivostomatisis was described. Inspite of the improvement of his gingivostomatitis, his consciousness gradually deteriorated and he was admitted to Nakadori General Hospital. His consciousness level was drowsiness and increased bilateral patellar reflexes were shown. Because magnetic resonance imaging (MRI) T2-weighted scan showed areas of high signal intensity disseminated in superior portion of medulla oblongata, dorsal portion of pons, basal nuclei and thalamus, he was suspected as having ADEM. Anti-herpes simplex virus (HSV) 1 IgG and IgM antibodies elevated in both blood and cerebrospinal fluid. From these results, HSV1 infection was thought to be the preceding infection of ADEM. methylprednisolone therapy (20 mg/kg daily) for 3 days, followed by prednisolone (2 mg/kg) was started, with an excellent response. In addition, administration of acyclovir was also continued, considering the complication of HSV encephalitis. MRI T2-weighted scan performed at 2 months later after the onset of ADEM revealed disappearance of the lesions. He was discharged without remaining disorders. It is difficult to distinguish between ADEM and HSV encephalitis because both of these diseases show various neurological symptoms. In our case, MRI was the most useful method for correct diagnosis of ADEM. We concluded that ADEM is important as a disease of central nervus system due to HSV1 infection, in addition to encephalitis.
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ranking = 3.0006690619948
keywords = encephalitis, herpes
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7/29. Acute disseminated encephalomyelitis following aseptic meningoencephalitis.

    A previously healthy 50-year-old man developed aseptic meningoencephalitis with clinical manifestations including fever, headache, seizure, Wernicke aphasia, right hemiplegia, and blindness in the left eye. One and one-half months after remission of meningoencephalitis, marked ataxia and psychiatric symptoms became apparent. magnetic resonance imaging revealed multiple new lesions involving the basal ganglia, thalamus, white matter, and cerebellum. Despite these developments, cerebrospinal fluid findings continued to improve except for excessive content of myelin basic protein. Within 2 weeks, steroid therapy dramatically resolved the ataxic symptoms and disseminated lesions.
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ranking = 6
keywords = encephalitis
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8/29. Severe depression as an initial symptom in an elderly patient with acute disseminated encephalomyelitis.

    We report a 63-year-old man with acute disseminated encephalomyelitis (ADEM), initially showing depression for one and a half months but subsequently meningoencephalitis followed by acute-onset myelopathy. Neuroradiological examinations of the brain demonstrated no focal lesion causative for his depression, while cerebrospinal fluid revealed elevated levels of inflammatory cytokines in parallel with disease activity. Because depression is usually a rare initial symptom for patients with ADEM, an increased production of inflammatory cytokines in the central nervous system as well as age-related alterations of immune response might have played an important role in the development of depression in this elderly patient.
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ranking = 1
keywords = encephalitis
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9/29. diffusion- and perfusion-weighted MR imaging in a patient with acute demyelinating encephalomyelitis (ADEM).

    To monitor changes of brain tissue metabolism in acute demyelinating encephalitis (ADEM), we examined a patient with suspected ADEM by serial MRI including diffusion- and perfusion-weighted imaging (DWI, PWI). Within the inflammatory tissue, the apparent diffusion coefficients were reduced, normal, and increased. perfusion varied between reduced and normal values, except for small hyperperfused regions. Combining standard MRI with DWI and PWI may elucidate different overlapping phases in cerebral inflammation.
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ranking = 1
keywords = encephalitis
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10/29. Acute disseminated encephalomyelitis after Japanese B encephalitis vaccination.

    A 6-year-old girl (Patient 1) and a 5-year-old boy (Patient 2) with acute disseminated encephalomyelitis after Japanese B encephalitis vaccination are reported. Drowsiness, paresthesias, and gait disturbance were observed at 14 days (Patient 1) and 17 days (Patient 2) after the vaccination; however, transient impairment of visual acuity was only found in Patient 1. Laboratory examinations revealed slow theta waves on electroencephalography and elevated myelin basic protein in the cerebrospinal fluid in both patients. The most striking feature on magnetic resonance imaging was the combination of white matter lesions and abnormal intensity signals of the thalamus. The administration of oral prednisolone (2 mg/kg/day) markedly improved the clinical findings and abnormal magnetic resonance imaging findings. A similar magnetic resonance imaging finding of abnormal intensity of the thalamus with deep white matter lesions has been reported in patients with Japanese B encephalitis; therefore, thalamic lesions may be related to the naturally occurring encephalitis.
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ranking = 7
keywords = encephalitis
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