1/224. 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. ( info) |
| subacute sclerosing panencephalitis (SSPE) is a rare progressive neurologic disorder. A 9-year-old boy was seen who had progressive neurocognitive decline, myoclonic jerking of the extremities, and an abnormal result of an electroencephalogram (EEG). Ophthalmoscopic examination revealed multifocal subretinal lesions. The diagnosis of SSPE was made on the basis of the clinical examination and elevated serum and spinal fluid measles titer. We describe subretinal lesions in a patient with SSPE. ( info) |
3/224. A 4-year-old with pica, progressive incoordination, and decreased responsiveness. This article reports a typical case of subacute sclerosing panencephalitis (SSPE). The patient contracted measles as an infant during the 1989 to 1991 united states measles epidemic. At 4 1/2 years of age, he developed behavioral changes and quickly progressed through the typical clinical stages of SSPE. His EEG was characteristic. serum and CSF measles immunoglobulin g were markedly elevated. He remains alive but is vegetative. To our knowledge, this is the first case of SSPE stemming from the 1989 to 1991 measles epidemic. Because infants--the group at highest risk to develop SSPE--were most severely affected by this measles outbreak, other cases of SSPE stemming from this epidemic may occur. ( info) |
4/224. 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. ( info) |
| We report a boy with subacute sclerosing panencephalitis (SSPE) who exhibited parkinsonian symptoms four months after onset. The symptoms improved after administration of levodopa. One year after onset, bilateral symmetric lesions appeared in the substantia nigra and the putamen, as observed using magnetic resonance imaging. After a one-year interval, the lesions migrated to the bilateral caudate and the cerebellar dentate nuclei. The series of migratory legions, each of which was connected by axonal pathways originating from the substantia nigra, suggests axonal spread of the sspe virus. ( info) |
| A defective cell-mediated immunity and inflammatory cytokines are suggested in the pathogenesis of subacute sclerosing panencephalitis. In this study we analyzed lymphocyte subsets in peripheral blood and concentrations of interleukin-1alpha (IL-1alpha), interleukin-2 (IL-2alpha), tumor necrosis factor-alpha (TNF-alpha), and platelet activating factor in plasma and cerebrospinal fluid before and after immunomodulatory therapy (interferon-alpha plus isoprinosine) in three patients with subacute sclerosing panencephalitis. Increased percentage of CD8 cells (T-suppressor/cytotoxic cell) and CD16 CD56 cells (NK cell) and reduced percentage of CD3 /HLA-DR (active T-cell) and CD3 (total T-cell) cells were found before therapy. After immunomodulatory therapy, CD3 /HLA-DR (active T-cell) cells were markedly increased and there was a slight increase in the percentages of all lymphocyte subsets in the patients. The concentrations of platelet activating factor in plasma and cerebrospinal fluid were higher than the mean value in controls. cerebrospinal fluid and plasma TNF-alpha and IL-2 levels were nondetectable in two patients who had a stationary course of disease and were markedly elevated in patient 3, who displayed a rapidly progressive course. ( info) |
| 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. ( info) |
| 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. ( info) |
| A 14-year-old boy presented with history of deterioration of scholastic performance, altered behaviour, difficulty in walking and repeated falls since last 2-3 months. He had history of delayed milestones and at 11 months developed febrile rashes. On investigations, MRI showed compensated hydrocephalus with aqueductal stenosis. EEG suggested subacute sclerosing panencephalitis (SSPE). CSF was positive for measles antibody titre. This case of aqueductal stenosis with SSPE is very interesting for its diagnostic dilemma. ( info) |
| 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. ( info) |