Ruth Nass

Androgen effects on cognition : congenital adrenal hyperplasia

Cognitive studies of congenital adrenal hyperplasia (CAH) patients have revealed (1) the presence of an IQ advantage in patients, siblings and parents due to socioeconomic status, genetic, hormonal, or other factors; (2) an IQ disadvantage in salt wasters compared with simple virilizers, probably due to early brain damage secondary to salt-wasting crisis; (3) a possibly increased incidence of learning disabilities, particularly in female patients and particularly for calculation abilities, due to disease-related early androgen exposure; and (4) a possible post-pubertal spatial advantage in CAH women, also due to early androgen exposure.

Differential effects of congenital left and right brain injury on intelligence.

IQ performance was compared in 28 prepubertal children with unilateral left vs. right hemisphere preperinatal injury. Results indicated statistically superior FSIQ and VIQ in the left-lesioned group. Findings were interpreted as consistent with the hypothesis of a left-right maturational gradient, whereby early right hemisphere damage has a more severe effect on overall intellectual functioning than early left injury. Findings also lend support for a greater crowding effect, as seen in the sparing of verbal skills, after early left hemisphere injury.

Acquired Aphasia With Convulsive Disorder: A Pervasive Developmental Disorder Variant

A 5½-year-old boy with language delay and behavioral problems was evaluated. His symptoms were consistent with pervasive developmental disorder, and electroencephalography showed frequent generalized spike and polyspike activity. After therapeutic levels of anticonvulsant medication were achieved, improvement was noted in communication skills and behavior, as well as on the electroencephalogram. Although the response of language dysfunction to these drugs has been generally disappointing in previously reported cases of communication disorders associated with electroencephalographic abnormalities, a trial of anticonvulsant medication is probably warranted. (J Child Neurol 1990;5:327-328).

Effects of Subependymal and Mild Intraventricular Lesions on Visual Attention and Memory in Premature Infants.

This study evaluated 10-month-old infants to determine if supependymal or mild intraventricular hemorrhage (S/IVH) affects visual attention, visual memory, or memory for location. Thirty premature infants with normal ultrasound scans, 30 premature infants with S/IVH, and 30 full-term infants were evaluated on a habituation/novelty preference task, the AB object permanence task, and the Bayley Scales of Infant Development

Landau-Kleffner syndrome: Temporal lobe tumor resection results in good outcome

Abstract A child with a left temporal lobe tumor and Landau-Kleffner syndrome (acquired aphasia with a convulsive disorder) is reported because of his good postsurgical outcome.

Temperament differences in toddlers with early unilateral right‐ and left‐brain damage

This article documents temperament differences between toddlers with early left‐ versus early right‐hemisphere injury. Consistent with the findings in adults of right‐hemisphere dominance for emotion, children 1 to 3 years old with congenital right‐hemisphere damage are already more difficult than their counterparts with left‐hemisphere damage.

FAILURE OF STEROID REPLACEMENT TO CONSISTENTLY NORMALIZE PITUITARY FUNCTION IN CONGENITAL ADRENAL HYPERPLASIA : HORMONAL AND MRI DATA

Exogenous glucocorticoid replacement in patients with congenital adrenal hyperplasia (CAH), who due to an adrenal 21-hydroxylase enzyme deficiency are unable to produce endogenous glucocorticoids, is aimed at normalizing hypothalamic-pituitary-adrenal function. Excess androgen production by the adrenals is thus decreased. Despite standard glucocorticoid replacement doses (12.5-40 mg, 10.5-27 mg/m2/day hydrocortisone equivalents) 4 of 7 patients ranging in age from 14 to 33 years had abnormalities of the pituitary on MRI. Three appeared to have microadenomas and 1 had an empty sella. Five (3 salt wasters, 2 simple virilizers) of these 7 patients had 60-min p.m. ovine corticotropin-releasing hormone (oCRH) stimulation studies. The mean (logarithm) area under the ACTH curve for 0-60 min after oCRH stimulation was significantly greater in patients than controls (p < 0.0001). Mean ACTH at each time point before and after oCRH stimulation was similarly greater in patients than controls (p < 0.05). Two of these patients had pituitary microadenomas, 1 had an empty sella; all 3 were salt wasters. Despite standard glucocorticoid replacement, adolescent and young adult patients with CAH tend to have high basal ACTH and ACTH hyperresponsiveness to oCRH, as well as structural abnormalities of the pituitary. The inevitable periods of under- and overexposure to glucocorticoids in CAH patients may over time cause abnormalities of the hypothalamic-pituitary-adrenal axis.

Epileptic facial metamorphopsia

A 14-year-old patient with a right parietal arteriovenous malformation presented with seizures characterized by metamorphopsia of faces. Unlike adults with right hemisphere pathology she performed like an age matched control on a task requiring recognition of unfamiliar faces. This likely reflects maturational changes in hemispheric dominance for face recognition.

Dichotic complex‐pitch and speech discrimination in 7‐ to 12‐year‐old children

Forty‐three normal 7 to 12‐year‐olds were tested on dichotic complex‐pitch and speech discrimination tests. For each age group (7 to 8 years, 9 to 10 years, and 11 to 12 years), the adult patterns of perceptual asymmetries were observed: a right‐ear advantage for speech and a left‐ear advantage for complex‐pitch, without significant changes either in the magnitude of perceptual asymmetries or in the frequency of occurrence of particular patterns of asymmetry as a function of age. For overall accuracy, however, a significant increase with age was found for speech but not for pitch discrimination, suggesting that despite the absences of significant age‐related changes in perceptual asymmetries in these two tasks, they may nevertheless undergo different developmental courses.

Cerebral dysfunction following infantile dietary chloride deficiency

Twenty-two children who were chloride-depleted in infancy due to a chloride-deficient diet and who had resultant hypochloremic alkalosis were analyzed in regard to their signs and symptoms, metabolic studies, and growth parameters. Deceleration of weight, linear growth, and head growth occurred in most, and persistent growth failure occurred in some. The majority had cognitive deficits at follow-up. Comparison with growth parameters in a chronically malnourished group of children who had a variety of disorders revealed a similar degree of deceleration of weight (p = 0.50) and height (p = 0.70), but more severe deceleration of head growth (p = 0.01). Comparison with follow-up cognitive deficits reported in the United States medical literature in children with similar severity of nutritional deprivation indicates that the chloride-depleted infants had more frequent and more severe cognitive deficits (p = 0.09). Cognitive deficits have been documented in U. S. children who are nutritionally deprived only when disorders causing concomitant chloride depletion are responsible for the malnutrition.