Neuropsychological performance of children with Duchenne muscular dystrophy and spinal muscle atrophy.

Neuropsychological examinations were conducted on 39 boys with Duchenne muscular dystrophy (DMD) and 12 children with spinal muscular atrophy (SMA). There were no significant group differences in the pattern or level of test-score profiles, indicating that the performances of the two patient groups were very similar. However, pattern analysis indicated that while scores on verbal and non-verbal intelligence tests were within the average range for the children with DMD, there were deficits on tests of immediate memory.

Neuropsychological deficits in Cushing's syndrome.

In contrast to well documented reports of medical symptoms in Cushing's syndrome (an endocrine disorder with the hallmark of corticoid overproduction), vague and infrequent references to associated mental changes reflect the subjective nature of previous clinical studies of psychological symptoms. This report presents neuropsychological test findings of 35 Cushing's syndrome patients before initiation of medical therapy, and documents the frequency, nature, and severity of deficits. Performances on the Michigan Neuropsychological Test Battery revealed equivocal or no signs of neuropsychological deficits in 13 patients. Ten other patients had few and mild deficits, eight had moderate and more frequent signs of impairment, and four had frequent and marked deficits in language and nonlanguage tests of higher (cognitive) and/or lower (sensory and/or motor) level cerebral functions. The findings thus indicate varying degrees of diffuse bilateral cerebral dysfunction in two thirds of this population. Impairment was more frequent and severe in nonverbal visual-ideational and visual memory functions. This pattern of deficits is similar to that in patients with other types of diffuse bilateral neuropathological processes such as toxicity, anoxia, and infectious cerebral disorders.