1/64. 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.- - - - - - - - - - ranking = 1keywords = ms (Clic here for more details about this article) |
2/64. apoptosis of T lymphocytes in acute disseminated encephalomyelitis.apoptosis has been shown to be an efficient mechanism involved in clearance of T lymphocytes from the brains of animals with acute experimental autoimmune encephalomyelitis (EAE), an animal model for human multiple sclerosis. In this report we describe a case of acute disseminated encephalomyelitis following general measles infection. In this disease, which closely mimics the pathology of acute EAE we found a high percentage (30%) of apoptotic T cells. This indicates that in both rodent and human brain clearance of T cell-mediated inflammation follows similar mechanisms.- - - - - - - - - - ranking = 1977.0079191233keywords = multiple sclerosis, sclerosis, ms (Clic here for more details about this article) |
3/64. 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.- - - - - - - - - - ranking = 1keywords = ms (Clic here for more details about this article) |
4/64. An 11-year-old girl with syndrome of inappropriate antidiuretic hormone secretion.An 11-year-old girl presented with a syndrome of inappropriate antidiuretic hormone secretion, which was transitory and, initially, of obscure origin. Subsequently, the patient's hypothalamic disorder emerged as a component of a steroid-responsive relapsing encephalomyelitis with cerebral pathology restricted to the basal ganglia and brainstem. Where such a disorder fits in the spectrum from acute disseminating encephalomeylitis to multiple sclerosis is discussed.- - - - - - - - - - ranking = 1976.0079191233keywords = multiple sclerosis, sclerosis (Clic here for more details about this article) |
5/64. ADEM: literature review and case report of acute psychosis presentation.Acute disseminated encephalomyelitis is a monophasic, immune-mediated disorder that produces multifocal demyelinating lesions within the central nervous system. It is characterized clinically by the acute onset of neurologic abnormalities, including varying degrees of mental state changes ranging from drowsiness to coma. It is unusual for the illness to present as an isolated acute psychosis. The case of a 14-year-old female with biopsy-confirmed acute disseminated encephalomyelitis, who was initially diagnosed with an acute psychiatric disorder, is presented, and published reports on this unusual manifestation are reviewed. A medline database search was performed from 1965 to 1999, using the terms acute disseminated encephalomyelitis, postvaccinal encephalomyelitis, postinfectious encephalomyelitis, and measles encephalomyelitis, combined with the terms psychosis, psychiatric disorder, and behavioral disorder. Selected cross-referenced reports were also reviewed. Nine patients were identified who presented with acute psychosis. We conclude that, although rare, acute disseminated encephalomyelitis can present as an acute psychosis. This immune-mediated condition should be included in the differential diagnosis of neurologic disorders presenting as a psychiatric illness.- - - - - - - - - - ranking = 2keywords = ms (Clic here for more details about this article) |
6/64. Acute disseminated encephalomyelitis after live rubella vaccination.We report here a case involving a 14-year-old boy who developed acute disseminated encephalomyelitis following live rubella vaccination. The patient became febrile and began to experience nuchal pain 16 days after the immunization. By 22 days after immunization, he experienced difficulty in walking. By 24 days, he had developed tetraparesis with retention of urine, and total sensory loss below the Th1 dermatomal level. He was febrile at this point and showed nuchal rigidity and Lhermitte's sign. cerebrospinal fluid examination revealed elevated cell counts, protein level, and myelin basic protein. T2-weighted magnetic resonance imaging detected high intensity lesions in the bilateral cerebral white matter and cervical spinal cord. Following the administration of intravenous corticosteroids, the patient's clinical symptoms improved rapidly.- - - - - - - - - - ranking = 1keywords = ms (Clic here for more details about this article) |
7/64. susac syndrome.PURPOSE: To report the first Japanese case of the susac syndrome characterized by microangiopathy of the brain, retina, and cochlea. methods: Case report. A 36-year-old Japanese man underwent extensive clinical and laboratory examination for encephalopathy, bilateral recurrent branch retinal arterial occlusion, and hearing loss. RESULTS: This case was initially diagnosed as acute disseminated encephalomyelitis. Despite exhaustive clinical and laboratory examination of the patient, no evidence of a known systemic disease was found. The bilateral recurrent branch retinal arterial occlusions were widely disseminated. Remission occurred 1 year after symptom onset. This patient demonstrated all of the clinical features associated with the susac syndrome. CONCLUSION: susac syndrome has been considered rare, but it is probably more common than previously thought and seems to occur in any race. This disorder usually occurs in young women, but it can affect men.- - - - - - - - - - ranking = 1keywords = ms (Clic here for more details about this article) |
8/64. Acute disseminated encephalomyelitis associated with poliomyelitis vaccine.A 6-year-old female patient with acute disseminated encephalomyelitis associated with poliomyelitis vaccine virus is reported. She had a history of high fever, headache, and gait disturbance. neurologic examination confirmed spastic triparesis, urinary incontinence, diminution of tactile sensation, and vision deterioration. Hemography, serum laboratory findings, and urinalysis were normal. The cerebrospinal fluid was clear, with normal pressure, 9 leukocytes/mm(3), and 27 mg/dL protein, but the myelin basic protein was elevated to 10.7 ng/mL. T(2)-weighted magnetic resonance imaging disclosed multifocal high-intensity lesions of the spinal cord. The serum polio virus type 2 antibody titer was raised in the acute phase, and polio vaccine virus type 2 was detected in viral cultures of the cerebrospinal fluid and pharynx swab and had undergone an A-G neurovirulence mutation at nucleotide 481. Finally, she had human leukocyte antigen (HLA)-Cw3 and HLA-DR2, to which multiple sclerosis is related in japan. Thus the cause of ADEM may have been related to her HLA type.- - - - - - - - - - ranking = 1976.0079191233keywords = multiple sclerosis, sclerosis (Clic here for more details about this article) |
9/64. 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.- - - - - - - - - - ranking = 1keywords = ms (Clic here for more details about this article) |
10/64. 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.- - - - - - - - - - ranking = 1keywords = ms (Clic here for more details about this article) |
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