1/168. 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. ( info) |
| The patient is a 10-year-old male who experienced somnolence and incomplete quadriplegia after headache and vomiting, without exanthema, for 3 days. The clinical course and magnetic resonance imaging findings of the brain and spinal cord were compatible with acute disseminated encephalomyelitis. The serologic examination revealed that the patient had rubeola because titers of IgM and IgG antibody to measles virus measured by enzyme immunoassay were 0.91 and 40 (cutoff = 0.80 and 2), respectively, at 5 weeks after the onset, the IgM titer had become negative (0.56), and the IgG titer had decreased to 17.7 at 13 weeks after the onset. Because the patient had received a measles-mumps-rubella vaccine at 12 months of age, the acute disseminated encephalomyelitis was thought to be attributed to the modified measles resulting from measles vaccine failure. ( info) |
| 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. ( info) |
4/168. 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. ( info) |
| A 12-year-old boy developed a convulsion, hemiparesis, and acquired aphasia with paroxysmal electroencephalogram (EEG) abnormalities consisting of repetitive spikes and waves in the left centro-parietal region. T2-weighted magnetic resonance imaging disclosed high intensity lesions in the left pre-Sylvian and right frontal areas. He was diagnosed as having acute disseminated encephalomyelitis, and thus the oral administration of phenytoin and steroid pulse therapy were begun. With these treatments, his hemiparesis disappeared and the aphasia also improved gradually. magnetic resonance imaging examination revealed the disappearance of the previously noted abnormalities, and the EEG abnormalities disappeared as well. This patient is a rare case of acute disseminated encephalomyelitis presenting an acquired aphasia. A focal lesion of acute disseminated encephalomyelitis may be responsible for the acquired aphasia. The distinction from landau-kleffner syndrome is also discussed. ( info) |
6/168. Intravenous immunoglobulin therapy in acute disseminated encephalomyelitis. We describe 4 patients with acute disseminated encephalomyelitis (ADEM) who were treated with intravenous immunoglobulins (IVIg) after getting no immediate response from a 3-5 day course of high dose intravenous methylprednisolone. All had clinical features to suggest poor prognosis and MRI findings to indicate extensive white matter changes in the brain. Two patients who had spinal cord involvement as well, required ventilatory support during acute phase of the illness. All the 4 patients recovered dramatically. Recovery pattern suggested that IVIg might be useful in fulminant ADEM. Further trials are needed to look for the efficacy of IVIg alone and in combination with methylprednisolone in the treatment of ADEM. ( info) |
7/168. 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. ( info) |
| Although acute disseminated encephalomyelitis has been observed after a variety of viral infections and an occasional bacterial infection, it has not been reported in association with rickettsial infections. Reported is a 7-year-old male with magnetic resonance images and clinical manifestations suggestive of acute disseminated encephalomyelitis after a tick bite and serologically proven rocky mountain spotted fever. ( info) |
9/168. 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. ( info) |
10/168. 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. ( info) |