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11/29. Delayed acute measles inclusion body encephalitis in a 9-year-old girl: ultrastructural, immunohistochemical, and in situ hybridization studies.

    A 9-yr-old girl developed delayed acute measles inclusion body encephalitis, which was different from subacute sclerosing panencephalitis (SSPE) in clinical course. measles virus was demonstrated by electron microscopy, immunohistochemistry, and in situ hybridization. Contrary to the most previous reports, matrix (M) protein was present in the brain, cerebrospinal fluid, and serum and was demonstrated by Western blot analysis and in situ hybridization. The hybridization was performed by a nonradioactive digoxigenin method.
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keywords = encephalitis
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12/29. Opsoclonus-myoclonus-dysequilibrium syndrome: cytological and immunological dynamics in the serial cerebrospinal fluid in two patients.

    A peculiar clinical presentation characterized by the triad of opsoclonus,myoclonus and ataxia, mainly in a form of dysequilibrium, is usually associated with infectious or paraneoplastic processes. Serial cerebrospinal fluid (CSF) analysis in two patients with opsoclonus-myoclonus-dysequilibrium syndrome suggestive of viral encephalitis were performed from disease onset for up to 8 months. A cell count, cytology, total protein and glucose concentrations in CSF, the blood-CSF barrier function, intrathecal synthesis of immunoglobulins (Ig) in class M, G and A expressed as IgM, IgG and IgA indices and oligoclonal IgG bands were monitored. Cellular and humoral alterations in both patients were slight at the onset becoming more pronounced a month later. The kinetics of the CSF changes mirrored the subacute clinical deterioration and subsequent recovery. The delayed response in the CSF measures and the gradual clinical deterioration suggest the development of subacute brain inflammation. A mononuclear pleocytosis, including macrophages and plasma cells, increased within the first month and then normalized during the following weeks. Intrathecally synthesized IgM occurred only transiently after one month of illness, whereas intrathecal IgG production increased during the first month and persisted for at least eight months. An increasing number of oligoclonal IgG bands during the course, indicative of expanding local intrathecal synthesis, was noted. The dynamics of these CSF changes supports the hypothesis that opsoclonus-myoclonus syndrome is a post-infectious immune- mediated condition.
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ranking = 0.16666666666667
keywords = encephalitis
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13/29. adult post-infectious thalamic encephalitis: acute onset and benign course.

    We report on two young patients with an encephalitic syndrome and bilateral thalamic lesions following a presumably viral or mycoplasma respiratory tract infection with the main clinical symptoms of organic psychosis in the first and a prolonged amnestic syndrome and ataxia in the second case. Four months later the patients had recovered clinically and the thalamic lesions had resolved on magnetic resonance imaging in one case and almost completely in the other. We interpret the patients' illness as rare cases of a post-infectious acute thalamic encephalitis in adults. The cases and their relationship to possible post-infectious autoimmune inflammatory or toxic pathophysiological mechanisms are discussed and a review of the literature is provided.
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ranking = 0.83333333333333
keywords = encephalitis
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14/29. Brainstem encephalitis and acute disseminated encephalomyelitis following mumps.

    A previously healthy female, aged 4 years 3 months, developed brainstem encephalitis with clinical manifestations of fever, decreased level of consciousness, and left facial and abducens paralysis 1 week after bilateral parotitis. Twenty days after remission of encephalitis, she manifested new symptoms of ataxia, dysarthria, and fever. magnetic resonance imaging revealed multiple hyperintense lesions which were increased in size when compared with the first magnetic resonance imaging. She was treated with glucocorticoids and intravenous immunoglobulin. Forty-eight days after therapy, she was able to walk with support and recovered completely on follow-up. Brainstem encephalitis and acute disseminating encephalomyelitis are discussed as rare complications of mumps.
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ranking = 1.1666666666667
keywords = encephalitis
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15/29. recurrence of acute disseminated encephalomyelitis after a 12-year symptom-free interval.

    We report a patient with recurrent acute meningoencephalitis who had three episodes of headache, fever and unconsciousness; the first episode was at age 6 and the second, at age 7. After a 12-year symptom-free interval, she had a relapse, exhibiting the same symptoms as those in the previous two episodes. Head magnetic resonance imaging also revealed the recurrence of lesions in the basal ganglia and medial portion of the temporal lobe. The occurrences of stereotyped symptoms with meningoencephalitis and the same lesions in the basal ganglia observed in each episode favor the diagnosis of recurrent acute disseminated encephalomyelitis (ADEM) rather than multiple sclerosis or multiphasic disseminated encephalomyelitis. The occurrence of this rare case suggests that ADEM can relapse after a very long symptom-free interval.
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ranking = 0.33333333333333
keywords = encephalitis
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16/29. Hypersomnia and low cerebrospinal fluid hypocretin levels in acute disseminated encephalomyelitis.

    A 7-year-old Japanese female diagnosed as having acute disseminated encephalomyelitis presented seizures, visual symptoms, and hypersomnia with bilateral lesions in the white matter, basal ganglia, and hypothalamus. Her clinical findings and demonstrated lesions in neuroimages were similar to those of Von Economo's encephalitis lethargica. Her hypocretin, the hypothalamic neuropeptide controlling sleep-awake cycle, was significantly low in the cerebrospinal fluid (146 pg/mL) on admission. Successive measures resulted in the gradual recovery of cerebrospinal fluid hypocretin to the normal range (263 pg/mL) as her excessive daytime sleepiness was reduced. Decreased hypothalamic hypocretin neurotransmission may be involved in this symptomatic case of hypersomnia associated with acute disseminated encephalomyelitis.
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ranking = 0.16666666666667
keywords = encephalitis
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17/29. Fulminant acute disseminated encephalomyelitis mimicking acute bacterial menigoencephalitis.

    Most patients with acute disseminated encephalomyelitis (ADEM) recover quickly under corticosteroid treatment and have a favourable long-term prognosis. We report on a young woman with acute onset of an extensive and solitary white-matter lesion in the left hemisphere. fever, high pleocytosis and elevated protein in cerebrospinal fluid initially suggested bacterial meningoencephalitis. The patient died from brain herniation despite maximal conservative therapy. Histological changes in necropsy were consistent with the diagnosis ADEM. Treatment options of fulminant ADEM are discussed.
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ranking = 0.83333333333333
keywords = encephalitis
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18/29. Postinfectious immune-mediated encephalitis after pediatric herpes simplex encephalitis.

    We report a 3-year-old patient who presented a secondary acute neurological deterioration clinically characterized by a partial kluver-bucy syndrome, 1 month after the onset of herpes simplex encephalitis. This episode is unlikely due to continuation or resumption of cerebral viral replication but might be related to an immune-inflammatory process. In children, postinfectious immune-mediated encephalitis occurring after HSE are usually clinically characterized by choreoathetoid movements. This type of movement disorder was, however, not observed in this patient. On the basis of this case and a review of the literature, we hypothesize the existence of a spectrum of secondary immune-mediated process triggered by herpes simplex virus cerebral infection ranging from asymptomatic cases with diffuse white matter involvement to secondary acute neurological deteriorations with or without extrapyramidal features.
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ranking = 1.6673357286615
keywords = encephalitis, herpes
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19/29. epilepsia partialis continua: acute disseminated encephalomyelitis or Rasmussen's encephalitis?

    This report describes a 15-year-old male presenting with headaches and seizures after a viral illness progressing to intractable seizures of the right hand and face. This patient presented with diffuse white matter lesions on magnetic resonance imaging which disappeared with treatment. A relapse 6 months later involving the left temporal and insular regions produced epilepsia partialis continua involving the right face and hand. The relevant literature is reviewed with an emphasis on possible etiologies, including both acute disseminated encephalomyelitis and Rasmussen's encephalitis.
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ranking = 0.83333333333333
keywords = encephalitis
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20/29. Initial DWI and ADC imaging may predict outcome in acute disseminated encephalomyelitis: report of two cases of brain stem encephalitis.

    Two young patients with acute disseminated encephalomyelitis (ADEM) of the brain stem are described. In spite of similar lesion sites in the brain stem, reaching from the upper medulla to the mesencephalon, the outcomes of the patients were very different: one made a full clinical recovery within three weeks while the other remained in a locked-in state more than a year after the disease episode. Both patients also differed in magnetic resonance imaging (MRI) findings on admission. The patient who remained in a locked-in state had pathological diffusion weighted imaging (DWI) scans and decreased apparent diffusion coefficient maps initially, with severe tissue destruction on follow up computed tomography, while the patient who recovered fully showed initially increased apparent diffusion coefficient values and almost complete resolution of MRI changes on follow up. Thus a comparison of these two cases may indicate differences in the underlying pathology in ADEM (vasogenic v cytotoxic oedema) that may be crucial for estimating tissue damage and clinical outcome.
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ranking = 0.66666666666667
keywords = encephalitis
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