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Biomedical subjects

R Nass

Publications and source records attributed to R Nass.

At least 55 records · Page 3Linked to original sources

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.

Adrenal Hyperplasia, Congenital↗

Learning disabilities in children with congenital adrenal hyperplasia.

Early exposure to androgens may be a cause of the higher incidence of learning disabilities among males. To test this hypothesis, we determined the frequency of learning disabilities, as measured by extent of Wechsler Verbal-Performance IQ discrepancy, among female patients with congenital adrenal hyperplasia, who are exposed in utero to excess androgens for their sex. A significantly larger Verbal-Performance IQ discrepancy (10.1 vs 7.7) was found for the 18 female patients than for 27 unaffected female siblings (P = .045, one-tailed). The Verbal-Performance IQ discrepancy of the females with congenital adrenal hyperplasia is in the male range (10.4 to 13.4), similar to males with congenital adrenal hyperplasia (n = 20) and unaffected male siblings (n = 31). In addition to the sex effect, there is an effect of the type of congenital adrenal hyperplasia on the frequency of learning disabilities. Simple virilizers are more likely to be learning disabled than salt-wasters (P = .04, one-tailed). However, salt-waster patients have a lower IQ (104 vs 117) than simple virilizer patients (P = .005, one-tailed). Presumably, this is because some salt-waster patients suffer brain injury from episodes of hypotension and hyponatremia. The diffuse insult to the central nervous system may overide the androgen effect and result in a general cognitive impairment. Because of this confounding effect on IQ in the salt-waster form of congenital adrenal hyperplasia, the simple virilizer female versus unaffected female siblings reprsents the best test of the hypothesis.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗

The effects of precocious adrenarche on cognition and hemispheric specialization.

Cognitive skills were assessed in 13 females with a history of precocious adrenarche (PA). They were of average intelligence. In terms of lateralized cognitive skills, PA had no effect on verbal fluency. The spatial abilities of females with a history of PA, who had reached gonarche (were fully pubertal), were inferior to those of females tested in the midst of PA and to population controls. The physiologic/hormonal changes associated with normal adrenarche may curtail further specialization of the right hemisphere, resulting in a relative spatial deficit among females in general, who as a group reach adrenarche earlier than males. This spatial performance deficit is exaggerated in females with PA.

Adolescent↗

Acquired aphasia with convulsive disorder: a pervasive developmental disorder variant.

A 5 1/2-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.

Aphasia↗

Evaluation of average life span of epithelial and stromal cells of human prostate by superoxide dismutase activity.

Little is known about the cell kinetics on which development of benign prostatic hyperplasia is based. This prompted us to study the superoxide dismutase (SOD) activity, which is known 1) to correlate with the life span of cells and 2) to decrease with advancing age of cells. Therefore, SOD was measured in epithelium and stroma of the human prostate from patients of various ages (20-86 years) and compared with the activity in the postmitotic skeletal muscle. It was found that the highest mean specific SOD activity is present in skeletal muscle (4.0 mU.mg protein-1), followed by the stroma (2.1 mU.mg protein-1) and epithelium (1.4 mU.mg protein-1). Similar results were obtained when SOD activity was expressed per DNA (5.03, 1.73, and 0.16 mU.micrograms DNA-1, respectively). Comparing the slope of the age-dependent regression lines, it was demonstrated that the slope of the stroma is much closer to the slope of the postmitotic skeletal muscle than the slope of the epithelium. From the data, it was calculated that the average life span of stromal cells is probably longer than 30 years and of epithelial cells longer than 2 years. Hence in human prostatic tissue the average cell death rate might be rather low.

Adult↗

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.

Brain Damage, Chronic↗

Drawing ability in four young children with congenital unilateral brain lesions.

The drawings of four 5-yr-old children, two with left and two right hemisphere congenital brain injury, were compared with those of 20 normal 3.5-5 yr-olds. Two types of drawings were evaluated: copied geometric forms and free drawings. The children with left hemisphere injury showed normal development in both copying and free drawing. The children with right hemisphere injury were developmentally impaired in the copying task. In addition, their free drawings lacked configurational coherence; they included the elements of the figures but failed to arrange them in spatially organized ways. This failure to organize spatially elements is consistent with the descriptions of spatial cognitive disorders found in the drawings of adults with right parietal brain lesions.

Brain↗

No sex-related differences in human corpus callosum based on magnetic resonance imagery.

Recent studies have reported sex-related differences in the morphology of the human corpus callosum in cadaver brains. To further investigate these reports, sagittal magnetic resonance image scans from 40 male and 40 female subjects were used to compare callosal morphology. Relative callosal measurements were calculated by morphometric analysis. Significant sex-related differences were not found for callosal areas, maximal callosal width, or callosal curvature. These results indicate the need for further study before claims of sex-related differences can be accepted.

Corpus Callosum↗

Hormones and handedness: left-hand bias in female congenital adrenal hyperplasia patients.

An excess of left-handers among males has been attributed to early androgen exposure. This theory was supported by our observation that girls with congenital adrenal hyperplasia (CAH) are more left-biased than their normal sisters. Male CAH patients, with prenatal androgen exposure similar to that of unaffected brothers, had typical male-handedness patterns.

Adolescent↗

Rapid assessment of mental status in the infant and young child.

This article reviews some of the tests for determining mental status in the infant and child. This assessment, in the setting of acute insult, is considerably more difficult than it is in the adult, since language skills and performance expectations differ in this age group.

Child, Preschool↗

Early language development in infants with cortical and subcortical perinatal brain injury.

To understand better the cognitive sequelae of mild perinatal brain injury, we studied three groups of high-risk infants, using the Early Language Milestone Scale (ELM Scale). Premature infants with Grades I and II intraventricular hemorrhages (IVH) were delayed on the expressive but not the receptive subscale of the ELM Scale. Mildly asphyxiated full-term infants were slightly delayed on both the expressive and receptive subscales. Premature infants without IVH performed the same as the normal sample on which the scale was based. Although normal intellectual functioning has been reported in infants with Grade I-II IVH, this study demonstrates early specific deficits in expressive language in these children. These results are discussed in relation to localization of language in the adult brain, and the influence of subcortical structures on development and maturation of the cortex.

Asphyxia Neonatorum↗

Neuronal ceroid lipofuscinosis with hypergonadotropic hypogonadism.

A case of adolescent-onset neuronal ceroid lipofuscinosis presenting with chorea and evidencing pyramidal and cerebellar dysfunction as well as hypergonadotropic hypogonadism is reported. In this patient, primary ovarian failure may be due to accumulation of ceroid in the ovaries.

Adult↗

Bilateral language: is the left hemisphere still dominant?

A 13-year-old left-handed boy with a left fronto-parietal vascular malformation evidenced bilateral symmetrical language representation at intracarotid amytal testing. Surgical resection of the parietal motor and frontal premotor area (sparing classical perisylvian language regions) for seizure control resulted in an acute aphasia. Language deficits were still apparent 3 months and to a lesser degree 1 year after surgery. This suggests that when language is bilateral and symmetrical, the left hemisphere may still be dominant or both hemispheres may be necessary to sustain full language competence. Explanations for atypical language localization within the left hemisphere are also discussed.

Adolescent↗

The development of spatial and class relations in four young children with right-cerebral-hemisphere damage: evidence for an early spatial constructive deficit.

This study followed the development of four children with right-hemisphere injury on a series of manipulative classification tasks to determine whether and how early brain injury affects the development of spatial and class relations. The children were first tested at about 2 years of age. Their data were compared with previously collected data from 18- to 42-month-old normal children, and with data from four young children with left-hemisphere injury. The results showed the children with right-hemisphere injury do not generate a particular spatial relation (next to) in their spatial groupings with the same frequency as normal or left-hemisphere damaged children, although they do generate in and on relations with normal frequency. An apparent deficit in the development of class relations is shown to be secondary to the spatial deficit, in that it is evident only in tasks that require spatial grouping.

Brain↗

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.

Adolescent↗

Mirror movement asymmetries in congenital hemiparesis: the inhibition hypothesis revisited.

Mirror movements are seen in normal children in the first decade. The movements persist after age 10 in patients with congenital hemiparesis. At first, mirror movements are more prominent in the good hand (when the impaired hand attempts a unimanual task), but after age 10, mirroring diminishes in the good hand, and these movements are equally prominent in good and impaired hands. Maturational changes in callosally mediated inhibition of uncrossed motor pathways and reorganizational changes of the pyramidal motor system after early unilateral brain injury explain these age-dependent changes in asymmetries of mirror movements.

Adolescent↗