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Biomedical subjects

B Hermelin

Publications and source records attributed to B Hermelin.

At least 19 recordsLinked to original sources

Do young calendrical calculators improve with age?

The calendrical calculation performance of two 10-year-old children of the same intelligence level (IQ 90) but different calendrical ability, was compared with the performance of eight adult idiot-savant calculators. The calculating speeds of the two 10-year olds fell within the range of the reaction times of the adult savants. No improvement was detectable in a series of successive trials over time, either in speed or accuracy. It is concluded that the young calculators have already inferred rules about calendrical structure and that their performance cannot be accounted for by practice alone, but these savants use cognitive strategies to aid their performance.

Adolescent

Pericellular glycoconjugates of cultured fibroblasts from control and cystic fibrosis patients.

1. Labeled glycoconjugates released by trypsin from cell surfaces of control and cystic fibrosis (CF) skin fibroblasts were purified and fractionated by column chromatography on Sephadex G-50 and Concanavalin A Sepharose. Based on chemical analysis and specific enzymatic digestions: (1) Glycoconjugates were characterized as O-linked glycopeptides consisting predominantly of glycosaminoglycan type and N-linked glycopeptides with glycans of complex type. Their relative proportions were similar between the two groups. (2) The N-linked glycopeptides exhibited an increased molar ratio of fucose to galactose in CF fibroblasts. (3) When pericellular glycoconjugates were metabolically labeled with [14C]glucosamine and [3H]fucose, incorporation and degradation kinetics were similar between the two groups.

Carbohydrates

Talents and preoccupations in idiots-savants.

The question was asked whether a diagnosis of autism or a tendency to repetitive behaviour and preoccupation with a restricted area of interest, were crucial features of idiot-savant talents. Answers by caretakers to a questionnaire on these topics revealed that autistic and nonautistic savants resembled each other closely in preoccupation but differed from controls matched for IQ and diagnosis. In addition, the mentally handicapped showed fewer repetitive tendencies than did autistic controls. It is concluded that independent of diagnosis, preoccupations and repetitive behaviour appear to be closely associated with the manifestation of idiot-savant talents.

Aptitude

Regulation of secretion in cultured tracheal serous cells by protein kinases A and C.

We recently reported that cultured gland serous cells release chondroitin sulfate proteoglycans (CSPGs) in response to beta-adrenergic agonists. In this study, we analyzed this regulatory pathway and other cellular mechanisms responsible for CSPG secretion. We show the following. 1) Isoproterenol increased CSPG secretion in a concentration-dependent manner, with maximal stimulation (50%) obtained at 10(-5) M; at this concentration, the beta-agonist also stimulated protein kinase A (PKA) by 50%, whereas it increased cellular adenosine 3',5'-cyclic monophosphate (cAMP) content by 300%. 2) Phenylephrine (10(-5) M), 4 beta-phorbol 12 beta-myristate 13 alpha-acetate (1.6 x 10(-7) M), and A23187 (10(-6) M) also stimulated CSPG secretion; this stimulation was concomitant with protein kinase C (PKC) translocation from cytosol to membrane, was blocked by sphingosine (2 x 10(-5) M), and was additive with that elicited by isoproterenol. 3) All PKC activators potentiated the isoproterenol-induced increased in cAMP accumulation without modifying the activation of PKA elicited by the beta-agonist. Our results indicate that although the signaling pathways triggered by alpha- and beta-adrenergic agonists converge at the level of adenylate cyclase in tracheal serous cells, PKA and PKC independently regulate CSPG secretion.

Animals

A specific linguistic ability.

A young man with diagnosed hydrocephalic brain injury was found to be an able linguist. His ability to translate into English from French, German, and Spanish was confirmed to be at a high level, and his capacity to express thoughts and describe scenes in these languages was also verified. A set of translations of the Peabody Picture Vocabulary Test was prepared in French, German, and Spanish, and IQs were derived in each language. The subject's performance intelligence was tested separately and found to be low, especially in relation to spatial reasoning. The subject's linguistic achievements were discussed in terms of an IQ-independent linguistic talent.

Adult

Further evidence for abnormal protein kinase C regulation of macromolecule secretion in fibroblasts from cystic fibrosis patients.

In comparison to skin fibroblasts from normal subjects, those from patients with cystic fibrosis (CF): (1) bound [20-3H] phorbol 12,13-dibutyrate (PDBu) with a higher affinity Kd = 25.8 vs 12.8 nM respectively) but expressed a similar number of total phorbol ester binding sites (about 2.5 pmol PDBu bound/mg of protein); (2) exhibited a faster and higher response to 4 beta-phorbol 12 beta-myristate 13 alpha-acetate (PMA) for the stimulation of [35S]-labelled glycoconjugate release, but were equally sensitive to the synergistic effect of A23187 on this process; and (3) secreted glycoconjugates with similar [35S]-sulfate and [14C]-leucine to [14C]-glucosamine labelling ratios. Taken together, these results provide further evidence for abnormal protein kinase C (PKC) regulation of macromolecule secretion in CF disease.

Binding Sites

Factors and primes: a specific numerical ability.

An autistic young man and a normal control were asked to factorize numbers and to recognize and generate primes. Both subjects made a similar number of errors and employed similar strategies, but they differed markedly in the speeds at which the arithmetical operations were carried out.

Adult

The recognition failure and graphic success of idiot-savant artists.

Three groups of subjects, an idiot-savant group, a group of mentally handicapped subjects matched for IQ, and normal artistically gifted children, were compared for their recognition and graphic reproduction abilities. It was found that, independent of input modality, level of intelligence determined recognition performance, while graphic ability independent of IQ was the determining factor in reproduction accuracy.

Adolescent

Art and accuracy: the drawing ability of idiot-savants.

The accuracy and the artistic merit of drawings produced by graphically gifted idiot-savants and by artistically able normal children were investigated in various conditions. Drawings had to be executed when a three- or two-dimensional model of the scene to be drawn was in view, or when it had to be remembered or drawn from another viewpoint. It was found that overall accuracy was better for the normal than for the mentally handicapped subjects. In contrast, ratings for artistic merit did not differentiate the groups. It is concluded that while the accuracy of drawings may be related to intelligence, the artistic quality of the graphic production is not.

Adolescent

Intelligence and musical improvisation.

We investigated whether somebody with a severe mental impairment could not only remember and reproduce music, but was also able to generate it. Musical improvisation requires the ability to recognize constraints and also demands inventiveness. Musical improvisations on a traditional, tonal and also on a whole tone scale composition were produced by a mentally handicapped and by a normal control musician. It was found that not only the control but also the handicapped subject could improvise appropriately within structural constraints, although with the tonal music the idiot-savant showed some stylistic latitude. It is concluded that cognitive processes such as musical input analysis, decision making, and output monitoring are independent of general intellectual status.

Adult

The memory structure of autistic idiot-savant mnemonists.

The memory organization of six autistic mnemonists was investigated in two experiments. The first of these provided some evidence that the material of special interest to the subjects, in the present instance bus numbers, was stored in memory in a categorized form. The second study showed that though the idiot-savant mnemonists did not differ from controls in the level of their general memory performance, the factor structure of a number of memory tests differed between the groups. In contrast to the controls, a verbal memory factor which seemed independent of measured verbal IQ was operative in the mnemonist subjects.

Adolescent

The understanding and use of interpersonal gestures by autistic and Down's syndrome children.

Autistic adolescents with mild, moderate, and severe degrees of mental retardation, Down's syndrome adolescents, and clinically normal 4-, 5-, and 6-year-old children were compared in their ability to understand a set of simple instrumental gestures. Almost all gestures were perfectly understood, that is, correctly responded to, by normal children from age 5 onwards, and by all the handicapped groups, regardless of diagnosis or degree of retardation. However, the ability to initiate such gestures on verbal request was generally less good, especially in the less able autistic groups. The same subjects were unobtrusively observed in the playground and during mealtime at their schools. Peer interactions were least frequent in the autistic subjects, regardless of degree of mental retardation. However, relative to interaction frequency, the autistic group used nonverbal instrumental gestures as a means of communication to the same extent as the other groups. Unlike Down's syndrome adolescents, or normal preschool children, no autistic adolescent ever used expressive gestures.

Adolescent

Low intelligence and special abilities.

In summary, our research enables us to conclude that specific talents are found in people who differ widely in general intelligence levels and such talents should therefore be regarded as at least partly intelligence-independent. However, between normal and mentally handicapped populations and even within the idiot savant group, general cognitive capacity plays some part in determining the manner in which talents manifest themselves. Idiot savant special abilities can neither be regarded as the sole consequence of practice and training, nor are such skills based only on an efficient rote memory. Instead, idiots savants use strategies which are founded on the deduction and application of rules governing the material upon which their special ability operates. They also generate novel or new examples of such rule based structures just as we do in our use of language. Because of the much greater prevalence of idiots savants in the autistic than in the mentally handicapped population, some characteristic common to both autism and specific giftedness might be assumed. An obsessional pre-occupation with a limited section of the environment might be a common factor to both. It may be this rather than autism itself which is relevant to the idiot savant phenomenon.

Aptitude

Kinaesthetic movement after-effects in children with Down's syndrome.

In order to assess the liability of proprioceptive information, an experiment on kinaesthetic after-effects was carried out. Matched groups of severely subnormal and Down's syndrome subjects were compared with normal children of either the same chronological or the same mental age. No difference was found between the normal and the severely subnormal children, but the magnitude of the after-effect measures in Down's syndrome children suggests that asymmetrical pointing produced kinaesthetic after-effects which disrupted these subjects' total frame of spatial reference.

Adolescent

Lysosomal N-acetyl-beta-hexosaminidase and beta-glucuronidase activities from arterial wall. Variations with aging.

Properties of two hydrolases: beta-glucuronidase and N-acetyl-beta-hexosaminidase were studied in lysosomal fractions of media-intima from arterial wall. These enzymatic activities change significantly with aging. In the arterial wall, the decrease in activities of beta-glucuronidase and N-acetyl-beta-hexosaminidase between young and old rats was highly significant while there no notable change in the activities of acid phosphatase. These data are in agreement with the metabolic slow-down and the modifications of the glycosaminoglycan distribution in the media-intima of arterial wall with aging.

Acid Phosphatase