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.
Subject(s)
Anemia, Sickle Cell/complications , Nervous System Diseases/etiology , Adolescent , Child , Cognition Disorders/etiology , Humans , Intelligence TestsSubject(s)
Dementia/etiology , Subacute Sclerosing Panencephalitis/psychology , Adolescent , Age Factors , Child , Child, Preschool , Follow-Up Studies , Humans , IntelligenceABSTRACT
Subacute sclerosing panencephalitis is a central nervous system degenerative disease that rapidly progresses to death in most untreated cases. In this study we compare the level of neurological disability longitudinally in a group of SSPE patients receiving inosiplex (Isoprinosine) treatment (12) to a historical control group of untreated patients (15). The mean ND did not differ between the groups from onset of SSPE through 21 months. From two years through four and one-half years the inosiplex group had significantly lower ND compared to the nontreatment group. The subjects were then divided into four subgroups: Group 1, rapidly progressing SSPE, not treated; Group 2, slowly progressing SSPE, not treated; Group 3, rapidly progressing SSPE, inosiplex treated; and Group 4 slowly progressing SSPE, inosiplex treated. The rapidly developing groups did not differ in ND at any time. The slowly developing treated group had significantly lower ND than the slowly developing untreated group from two and one-half years to four and one-half years after onset of SSPE. These findings suggest that inosiplex is effective in the slowly developing or chronic form of SSPE.
