The Child With Mongolism {Congenital Acromicria)

Benda, Clemens E.

doi:10.1097/00005053-196110000-00026

Cited by 24 publications

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“…They are known to have an essential role in brain stem calcification (, Norman and Urich, 1960) and also to occur with great frequency in Down's syndrome. Cardiac malformations were described by Benda (1960) in 75% of mongoloid children who died before age 1 year and in 35% of those who lived beyond 1 year of age. Matuso et al (1972) found cardiac anomalies in 55% of Japanese children with Down's syn drome.…”

Section: Is Brain Stem Calcification Caused By or Related To D Ow N'smentioning

confidence: 99%

Basal Ganglia Calcification and Psychosis in Mongolism

Jakab¹

1978

Eur Neurol

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An 18-year-old mongoloid girl (trisomy 21) of Asian descent presented two symptoms rarely associated with mongolism (Down’s syndrome). (1) Brain stem calcification; the first case diagnosed in vivo in mongolism on computerized axial tomography. (2) Hallucinatory psychotic depression; the first case successfully treated, to full recovery from the psychosis, in a mongoloid child with known brain stem calcification. The psychotic depression was related to moderate environmental stress. While being treated in her parent’s home, in their native land, for more than 1 year, the psychosis remained unresponsive to tranquilizers. Following hospital admission in the USA, rapid improvement was obtained within a few weeks through intensive milieu therapy.

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Section: Is Brain Stem Calcification Caused By or Related To D Ow N'smentioning

confidence: 99%

Basal Ganglia Calcification and Psychosis in Mongolism

Jakab¹

1978

Eur Neurol

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“…The parent control group consisted of 33 parent pairs, each with at least two nonmongol offspring. Maternal age of 35 years at time of conception was made the dividing line between agedependent and age-independent mongolism, since the risk of affected offspring increases markedly at this maternal age, according to Benda (1960).The total dermal index of Walker (1958) was used without any modifications for the dermatoglyphic analyses on individuals with mongolism, their parents, and the parents in the control group. The dermal areas studied in this total dermal index are 10 digital patterns, the position of two palmar axial triradii, two interdigital patterns, and two plantar hallucal patterns.…”

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confidence: 99%

Parental dermotoglyphs in age-independent mongolism.

Priest¹

1969

Journal of Medical Genetics

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Penrose and Smith (1966) have estimated that about three-fifths of all clinical mongolism is caused by some process closely related to ageing of the ovum (the age-dependent group), while the other two-fifths (age-independent) includes the socalled hereditary causes and some strongly suggestive instances of environmental origin. The relative proportion of cases in the two classes differs from one country to another and from one study to another. It has also been reconfirmed recently (Sigler et al., 1965) March 10, 1969. at maternal age 35 or over. The parent control group consisted of 33 parent pairs, each with at least two nonmongol offspring. Maternal age of 35 years at time of conception was made the dividing line between agedependent and age-independent mongolism, since the risk of affected offspring increases markedly at this maternal age, according to Benda (1960).The total dermal index of Walker (1958) was used without any modifications for the dermatoglyphic analyses on individuals with mongolism, their parents, and the parents in the control group. The dermal areas studied in this total dermal index are 10 digital patterns, the position of two palmar axial triradii, two interdigital patterns, and two plantar hallucal patterns. A single total dermal index was obtained from hand and foot prints obtained from each individual; standard ink or inkless methods were used (Cummins and Midlo, 1961). The total index was originally based on the frequencies of mongol to control dermal patterns, expressed as logarithms (Walker, 1958). Mongol patterns are more positive. In this investigation the dermal indexes in the various study subgroups were compared, using the Student's t test to evaluate the significance of differences. A p value of 0 05 or less was considered significant. ResultsReliability of Total Dermal Index. To ascertain that the total dermal index was a reliable measure of clinical mongolism in the population studied in this report, histograms were prepared of the total dermal indexes of the 68 mongols in the study, and the 66 parents in the control group (Fig. 1). It can be seen that there are no normal (non-mongol) individuals who score +3 or more, and there are no individuals with clinical mongolism who score -3 or less. The range of overlap between + 3 and -3 included approximately 30%/o of both groups, while about 70%, of mongols and 70%0 of non-mongols fell, respectively, above or below this range. The resemblance of the histograms for this study to those originally prepared by Walker (1958) is striking. It was felt that the index was a reliable measure of clinical mongolism in the present study group.

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“…Although the growth curve of the infant could be th at of a mongoloid, a disturbance in the growth of the distal parts of long bones, called 'acromicria' by Benda [1], was absent. The proportions of the extrem ities were normal, and the hands and feet were not short and lump.…”

Section: Physical Findingsmentioning

confidence: 99%