During the past five years, 15 patients with subacute sclerosing panencephalitis (SSPE) were treated with inosiplex. Using a disability index specifically designed for the disease, this study monitored the course of SSPE in each patient before and during inosiplex therapy. Posttreatment follow-up ranged from 2 to 144 months. Inosiplex had an apparently beneficial effect on morbidity and mortality in 10 of the 15 patients tested. Eight improved immediately after treatment, 2 had a delayed improvement, and 1 patient stabilized. Four patients followed a typical course for SSPE and died a mean 9 months after onset. Treatment was not associated with adverse reactions. Due to its low risk-benefit ratio, inosiplex is recommended for continuous use in SSPE even after extended remissions.

Twenty-four computed tomographic scans of 12 patients with confirmed subacute sclerosing panencephalitis were studied using standardized techniques of radiological assessment. Abnormalities encountered were of four types--(1) lateral ventricular dilatation, (2) cerebral cortical atrophy and sylvian fissure widening, (3) low parenchymal attenuation, and (4) brainstem atrophy and cerebellar atrophy--and of varying degrees. The abnormalities correlated best with the stage and duration of disease, but not necessarily well with the patient's mental state. The fewest radiological abnormalities were encountered in the acute or early stages, whereas more signs of parenchymal disturbances in the form of low attenuations emerged during intermediate periods. Chronic periods were accompanied by atrophic changes in the form of cortical atrophy, ventricular dilatation, and brainstem cerebellar atrophy.

No abstract

No abstract

In this study, the neuropsychologic functioning of 21 children with sickle cell anemia and 21 sibling controls, age range 7 through 16 years, with no history of neurologic disease, was examined. Outcome measures included tests of intelligence, constructional praxis, memory, and academic learning. On the Wechsler Intelligence Scale for Children—Revised, the sickle cell group had a mean Full Scale IQ of 77.7 (SD 12.4) compared with 94.3 (SD 11.0) for the control group. The profile of test scores was similar for the two groups, with the sickle cell group scoring significantly lower than the control group on almost all cognitive measures. Both groups showed academic achievement to be commensurate with their measured intellectual ability. These results suggest that subtle but significant and widespread neuropsychologic deficits are associated with sickle cell anemia even in the absence of neurologic complications. When and by what process this neuropsychologic impairment is caused needs to be determined.