1/82. SSPE following neonatal measles infection.The authors report a case of subacute sclerosing panencephalitis in a child who had measles during the neonatal period. At 3 years, 6 months of age, over a period of a few weeks, the patient lost the ability to sit unaided as a result of progressive truncal ataxia, without apparent cognitive changes, simulating acute cerebellar ataxia. His symptoms improved in 1 month, and he was able to walk again with support, but mental alteration and periodic mild head nodding on awakening followed. His illness was diagnosed as subacute sclerosing panencephalitis on the basis of the elevated titers of measles antibodies in the cerebrospinal fluid. Measles infection before 1 year of age is a risk factor of subacute sclerosing panencephalitis, but reports about patients with neonatal measles infection are rare. Immaturity of the brain at the time of measles infection may not only be a risk factor but may also influence the clinical course of the disease.- - - - - - - - - - ranking = 1keywords = brain (Clic here for more details about this article) |
2/82. Subacute encephalopathy in a 5-year-old boy.A 5-year-old boy presented with an acute ataxia and altered mental status. Although he initially recovered from these symptoms, he presented a second time with myoclonus and seizures and rapidly became vegetative. cerebrospinal fluid studies, magnetic resonance imaging, and brain biopsy all confirmed the presence of subacute sclerosing panencephalitis. Despite courses of therapy with cimetidine, amantadine, ribavirin, and inosine, no clinical improvement has been seen. Clinicians need to be alert to the possibility of subacute sclerosing panencephalitis even in the vaccinated child in the appropriate clinical setting.- - - - - - - - - - ranking = 1keywords = brain (Clic here for more details about this article) |
3/82. The value of serial auditory brainstem response in patients with subacute sclerosing panencephalitis.A total of 98 serial auditory brainstem responses from 17 patients (11 boys and 6 girls) with subacute sclerosing panencephalitis were compared with their clinical course and stages. These patients were exposed to measles early in life (at 1.8 /- 1.4 years old) with the average onset of the disease at 8.8 /- 0.7 years of age. The main abnormalities of their auditory brainstem responses were a prolongation of waves I, III, and V, and of the I to V interpeak interval. Wave V thresholds were also elevated. These effects on the auditory brainstem responses started 1 to 2 years after onset of neurologic signs. I to V interpeak latency became prolonged with the progress of clinical stages, especially in Jabbour's clinically advanced stage IV. In two patients with an acute progressive type of subacute sclerosing panencephalitis, very rapid deterioration with a distorted wave pattern of later components was observed. These findings suggest rostrocaudal progression in the central nervous system of both long-term and fulminant cases.- - - - - - - - - - ranking = 7.620763718361keywords = brain, central nervous system, nervous system (Clic here for more details about this article) |
4/82. subacute sclerosing panencephalitis : CT and MR imaging in a rapidly progressive case.We report the findings on CT and MR imaging in a patient with rapidly progressive subacute sclerosing panencephalitis (SSPE), which correlated with the clinical progression of the disease. In view of the rapid neurological deterioration and CSF pleocytosis, a brain biopsy was done and this confirmed the diagnosis.- - - - - - - - - - ranking = 1keywords = brain (Clic here for more details about this article) |
5/82. subacute sclerosing panencephalitis with atypical onset: clinical, computed tomographic, and magnetic resonance imaging correlations.Clinical, computed tomographic (CT), and magnetic resonance imaging (MRI) correlations of subacute sclerosing panencephalitis with an atypical onset are presented in three children. In all three patients, the disease began similarly, with unilateral neurologic deficit followed by gnosis, praxis, and memory dysfunction corresponding to massive one-sided lesions. The first patient demonstrated right frontal-lobe syndrome and polymorphic extrapyramidal hyperkinesias; MRI showed a large high-signal lesion in the right frontal lobe, while CT was normal. The second patient displayed a disease onset with left-sided hemiparkinsonism and involuntary movements correlating to the MRI finding, ie, a massive rightsided occipitotemporoparietal subcortical lesion. An acute, stroke-like episode represented the first symptom in the third child. CT visualized cerebral, mainly left-sided cortical atrophy. In all three children, CT and MRI revealed significantly progressing brain atrophy at the disease's latest stages. We discuss the role of MRI in detecting early pathologic changes in children with subacute sclerosing panencephalitis.- - - - - - - - - - ranking = 1keywords = brain (Clic here for more details about this article) |
6/82. Immunocytochemical characterization of long-term persistent immune activation in human brain after herpes simplex encephalitis.The clinical, virological and immunocytochemical features of three children who recovered from acute herpes simplex encephalitis (HSE) before the age of 2 years, and who developed secondary severe focal epilepsy after a symptom-free period, leading to neurosurgery 3-10 years later are described. In one child, relapse of HSE occurred immediately after surgery. In all three patients, brain sample biopsies showed abundant CD3-positive T lymphocytes with a majority of CD8 cells, and abundant activated macrophage-microglial cells, a pattern similar to that found in acute HSE. herpes simplex virus dna was retrieved from the tissue biopsy in one case. The long-term persistent cerebral inflammatory process observed after HSE differed from that observed in another chronic viral disease, subacute sclerosing panencephalitis. This inflammatory reaction may be a result either of low-grade viral expression or self-induced immune activation. The role of inflammation in triggering epilepsy remains hypothetical. Solving these issues should have major therapeutic implications. herpes simplex virus dna latency in brain may be the source of replicative HSE relapse.- - - - - - - - - - ranking = 6keywords = brain (Clic here for more details about this article) |
7/82. subacute sclerosing panencephalitis presenting as optic neuritis.subacute sclerosing panencephalitis (SSPE) is a rare progressive neurologic disease affecting both grey and white matter of the brain in children and young adults. One such case which involved the visual system is described here.- - - - - - - - - - ranking = 1keywords = brain (Clic here for more details about this article) |
8/82. Combined treatment with interferon-alpha and ribavirin for subacute sclerosing panencephalitis.Two patients with subacute sclerosing panencephalitis (SSPE) are described. They were diagnosed on admission to the hospital with SSPE, as judged on cerebrospinal fluid examination involving reverse transcription followed by polymerase chain reaction, at the second stage of Jabbour's classification. They first were treated with intraventricular interferon-alpha monotherapy; however, the combination of interferon-alpha and IV ribavirin was started at 8 and 5 months after beginning the interferon-alpha monotherapy, respectively. Although slow progressive brain atrophy was observed in Patient 1 on brain magnetic resonance imaging before the ribavirin therapy, no further progression was noted 11 months after starting combination therapy with ribavirin. The event-related potential study results and audiography of the right ear improved in Patient 1 after the combination therapy was initiated. In Patient 2 the hypertonicity, neurobladder incontinence, and dysphagia improved 3 months after starting the combination treatment. Although this group of patients is small, these results suggest treatment with intrathecal high-dose interferon-alpha and IV ribavirin is effective in the treatment of SSPE. Early administration of intrathecal high-dose interferon-alpha and IV ribavirin should be considered as a possible therapy for SSPE patients, especially interferon-nonresponding ones.- - - - - - - - - - ranking = 2keywords = brain (Clic here for more details about this article) |
9/82. subacute sclerosing panencephalitis: brain stem involvement in a peculiar pattern.The most common pattern in subacute sclerosing panencephalitis, is in the cerebral hemisphere white matter on T2-weighted images with or without atrophy. Brain-stem lesions are rare. We report brain-stem involvement in two children with subacute sclerosing panencephalitis. A peculiar pattern, with involvement of the pons with extension to both middle cerebellar peduncles and substantia nigra but sparing the pontine tegmentum, is suggested.- - - - - - - - - - ranking = 5keywords = brain (Clic here for more details about this article) |
10/82. subacute sclerosing panencephalitis (SSPE): isolation of a defective variant of measles virus from brain obtained at autopsy.A cytopathic agent causing formation of syncytial giant cells was isolated by co-cultivation of human embryonic lung cells with brain cells obtained at autopsy from a patient with subacute sclerosing panencephalitis. Measles specific intracellular immunofluorescence was detected in syncytial giant cells developed by the agent. Paramyxovirus-like nucleocapsids were observed by electron microscopy in nuclei of the syncytial giant cells. Measles specific immunofluorescence was also detected on the surface of unfixed syncytial giant cells. However, the synycytial giant cells did not produce either virions or hemogglutinin, and did not show hemadsorption of African green monkey red cells. Hence, the newly isolated agent seems to be a defective variant of measles virus, and was designated as the SSPE-"BIKEN" strain.- - - - - - - - - - ranking = 5keywords = brain (Clic here for more details about this article) |
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