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1/4. A case of relapsing acute disseminated encephalomyelitis with high dose corticosteroid treatment.

    We report a 16-month-old boy with acute disseminated encephalomyelitis (ADEM) who had an early relapse despite prompt treatment with high dose methylprednisolone. The second episode responded to intravenous immunoglobulin (IVIg). This case illustrates the probability of relapses or treatment failures in ADEM after steroid treatment, and the use of alternative drugs.
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2/4. 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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3/4. recurrence of acute disseminated encephalomyelitis at the previously affected brain site.

    BACKGROUND: Acute disseminated encephalomyelitis (ADEM) is a usually monophasic demyelinating disorder of the central nervous system. Recurrences pose a diagnostic challenge because they can be overlooked or suggest an alternative diagnosis. OBJECTIVE: To examine the frequency, nature, and outcome of recurrent ADEM. DESIGN: review of the medical records of patients diagnosed in our institution as having ADEM between January 1, 1983, and May 31, 1998. Recurrences were defined as appearance of new symptoms and signs at least 1 month after the previous episode. RESULTS: Five (24%) of 21 patients with ADEM developed recurrent disease episodes. In all, diagnosis was confirmed by brain biopsy. One patient had 4 disease episodes, 2 had 3, and the other 2 each had 2. recurrence appeared 1.5 to 32 months after initial presentation and involved the same brain territory in 6 of 9 recurrences in 3 of 5 patients. In 2 patients, recurrences included neuropsychiatric signs. A good response to corticosteroid therapy was observed in 10 of 13 of treated ADEM attacks: in 3 of the 4 treated initial events and in 7 of 9 recurrences. CONCLUSIONS: Recurrent ADEM may be more prevalent than previously recognized. patients who relapse tend to have more than 1 recurrence that usually involves, clinically and radiologically, a brain territory that was affected before and can simulate a space-occupying lesion that requires histologic diagnosis. Neuropsychiatric features may be the main presentation of a relapse. Since recurrent ADEM is a corticosteroid-responsive condition, awareness and early diagnosis are mandatory.
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4/4. A high rate of neurological complications following Semple anti-rabies vaccine.

    A fatal case of encephalitis due to Semple (phenolized sheep-brain) anti-rabies vaccine prompted a search for neurological complications among 722 recipients of 2 vaccine batches administered in Bangkok, thailand in June and July 1984. A review of all patients admitted with neurological symptoms from June through August 1984 to the 5 major teaching hospitals in Bangkok found 6 cases (0.83%), including the index case, who had received the vaccine. rabies infection was ruled out in all 6 cases. 4 patients had meningitis, and 2 had meningo-encephalitis. Only the index case was fatal; the other patients recovered without neurological sequelae. The rate of neurological complications after receiving Semple vaccine was therefore a minimum of 8.31 cases per 1000 persons vaccinated (1:120). This complication rate was about 25 times higher than the overall complication rate of 0.33 per 1000 (1:3018) determined from 14 previous reports. The fatality rate was 1.39 per 1000 (1:722), about 15 times higher than the rate of 0.09 per 1000 (1:10805) calculated from the previous studies. It is urgent to find economically feasible alternatives to Semple vaccine.
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