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This paper reports observations on 6 new patients with the Dubowitz syndrome which was first defined by Grosse et al., in 1971 and which is a recessively inherited, pleiotropic malformation syndrome including variable degrees of intrauterine growth retardation and primordial shortness of stature, microcephaly, mental retardation, eczema, and a characteristic appearance, voice and combination of minor anomalies. Data in the present report show that eczema can be absent, and patients can be of normal height, and of normal intelligence in spite of a head circumference which has so far always fallen below the third percentile. So far 11 patients (8 females and 3 males) are known with the Dubowitz syndrome; in one family the parents were first cousins. [ABSTRACT FROM AUTHOR]

The cause of most microcephaly is unknown. Hanshaw's observation that 44% of microcephalics had cytomegalovirus (CMV) complement fixing (CF) antibody (Ab) suggested that inapparent congenital CMV (and other) infections might be a significant cause. Accordingly, sera from non-institutionalized microcephalic, mentally retarded normocephalic, and normal children were tested for cytomegalovirus and herpes simplex virus (HSV) complement fixing, rubella hemagglutination inhibition (HI), and toxoplasma dye test antibodies in an effort to determine whether there was a significant association between infection with these agents and microcephaly or mental retardation. Eight of 62 (13%) microcephalic children who were from 5 months to 5 years of age had titers of CMV CF Ab equal to or greater than 1:10. Four of 26 (15%) mentally retarded normocephalic children were seropositive to CMV, and two of 44 (5%) normal normocephalic children were seropositive. These differences are not statistically significant. In the age range 5 months to 5 years, 9 of 62 (15%) microcephalic, 9 of 26 (15%) normocephalic mentally retarded, and 4 of 44 (9%) normal normocephalic children were seropositive to HSV CF Ab. In the group 5 months to 5 years old tested for rubella HI Ab, 11 or 61 (18%) microcephalic, 2, of 25 (8%) normocephalic mentally retarded, and 6 of 38 (16%) normal normocephalic children were found to be seropositive. Three of 61 (5%) microcephalic, 2 of 26 (8%) mentally retarded normocephalic, and 3 of 42 (7%) normal normocephalic children from 5 months to 5 years had Ab to toxoplasma. There was no statistically significant association of evidence of infection with these three organisms and microcephaly. The authors speculate that inapparent congenital CMV infection can cause microcephaly and mental retardation without microcephaly, but that this infection causes only a small proportion of these conditions. [ABSTRACT FROM AUTHOR]

The article reports on the results of the genetic and biochemical investigation of primary microcephaly. In this study about fifty-five microcephalic patients were examined for cytogenetic and biochemical anomalies. Study revealed that a significant amount of primary microcephalics can be expected to display cytogenetic and biochemical abnormalities, which are causally related to the mental retardation.

The article focuses on the psychology of microcephalics. It was discussed that microcephaly literally means small head. The author concluded that recognition of the guiding force may be called by some deference to metaphysical ideas. The study was limited on a symbolic formulation, the concept of logic.

The article reports on the results of a clinical and genetical research of microcephaly, a part of a follow-up study of a group of patients who were in the institution for the mentally deficient at Faribault, Minnesota. Discussion of the criteria employed to define the term, microcephaly. Several cases are presented as representative examples of a non-genetic type of microcephaly.

The article discusses the findings of a 10-year research based on autopsies of mentally defective patients. The study indicates that a variety of pathological conditions operating before birth, at birth and after birth caused mental deficiency. These conditions include mongoloid deficiency, microcephaly, hydrocephaly, vascular accidents, post-infectious conditions, congenital syphilis, familial morons and high-grade imbeciles, phenylpyruvic oligophrenia, endocrine cases, dwarfism and neoplastic malformations.

The article discusses the cases of patients with hereditary mental defects. The two main characteristics of apparently sex-linked idiocy are mental deficiency and muscular weakness with moderate atrophy. In the study, all patients with sex-linked idiocy were males. In patients with microcephaly, the condition appeared in different lines in three successive generations.

The article describes a case of moderate microcephaly associated with a considerable degree of retardation in mental development following Roentgen irradiation in utero. The results show that the level of mental development is higher. The patient's social adaptation and adjustment are superior. The patient has also made some progress in reading and spelling.

The article presents a study that examines the incidence of cytomegalovirus (CMV) infection in the mentally retarded children admitted to a state institution. Three groups for examination were selected from patients at the Central Wisconsin Colony and Training School. In group A, 89 mentally retarded patients were selected. In group B, 40 non-mongol mentally retarded resident patients under five years of age and 32 non-mongol, non-resident subjects under five years of age seen in the Outpatient Development Evaluation Centre at the Colony were selected. In group C, 16 typical primary microcephalic patients were selected

The article focuses on a case of a family in which three children were similarly affected with microcephaly and multiple congenital abnormalities. The study describes a comparable family with three affected and two unaffected sibs in which the phenotypically healthy parents were first cousins. The proposita was the result of the fifth pregnancy, at the maternal and paternal ages of 31 and 48 years respectively. The fifth, uneventful, pregnancy terminated, at full term, on April 20, 1971, with the birth of the proposita, 1,900 grams in weight and 32.5 cm. long, who died 10 minutes afterbirth.

The article presents a case of deletion of short arms of chromosome 18 with normal values of IgA in serum. The patient was a male infant. On physical examination at 31 hours of age, the propositus showed a severe cleft lip, total absence of hard palate, agenesia of the premaxillary process and of nasal bones. Other features were large and low set ears; micrognathia and retrognathia; microcephaly and brachycephaly; low-hairline, short neck; balanoprecupial hypospadias, muscular hypotony and elongated fingers.

This article is concerned with the association of cutis verticis gyrata with mental subnormality. It is of interest that nearly all instances of cutis verticis gyrata reported in mental defectives have occurred in males. An obvious explanation is that the scalp anomaly is more readily observed in males as the longer hair of females would tend to hide the condition in the latter sex. Researchers thought that superfluity of scalp tissue consequent on a small skull led to the formation of cutis verticis gyrata. However, not all mental defectives with this scalp anomaly have been microcephalic. Furthermore, though microcephaly is comparatively common among the mentally subnormal, cutis verticis gyrata is rarely seen.

This article presents information related to the genetics and sub-classification of microcephaly. The term microcephaly is derived from the Greek and means "small-headedness". When the distribution of cranial capacity for a group of adult mentally defective patients is compared with that of normal adults, it is found that the distribution for the mentally defective group extends to further limits at either end of the scale. At the upper end of the range of distribution for the defective group, hydrocephalic patients with abnormally large heads are found. At the other end of the range, where individuals with small heads are represented, the distribution of mental defectives extending beyond the distribution of normal subjects consists of a heterogeneous group from the point of view of clinical type.

The article presents a discussion on malformations and diseases of the central nervous system. In the human population the incidence of malformations of the brain and spinal cord is about six per 1,000 births. These figures, however, do not take into account embryos which are aborted during pregnancy. The commonest malformations of the central nervous system naturally occurring in man are, in order of frequency, hydrocephaly, exencephaly, mongolism, spina bifida and microcephaly. Lesser degrees of deformity undoubtedly occur but these are not detectable by gross examination.

The article presents the case of a 4-year-old boy suffering from Thomson's Syndrome. When the boy was 6-7 months old, the eruption appeared over the face, and spread to the backs of the hands, outer aspect of the forearms and dorsum of the feet. It has persisted, but has not increased. Various congenital defects have been described in association with Thomson's syndrome including bony abnormalities, microcephaly, mental deficiency, cataracts, and abnormalities of hair, teeth and nails, but such a spasticity has not been found in this boy.

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The article cites a case report on congenital rubella in an infant. The placental transmission of rubella virus can lead to a variety of infants' congenital abnormalities. Babies born to mothers infected with rubella virus may have thrombocytopenia, hepatomegaly, splenomegaly, evidence of widespread organopathy and radiographic evidence of destructive bone lesions. An infant admitted to Variety Children's Hospital was found to have an overlapping of bones in the skull which was not present at birth. The infant, born to a mother infected with rubella, was diagnosed as microcephalic.

Describes a case of a syndrome of microcephaly and multiple congenital anomalies in three siblings who were either stillborn or died shortly after birth. Background of the case; Cytogenetic studies conducted on the siblings; Comparison of the clinical findings of the second and third siblings.

This article cites a study conducted by researcher David W. Smith and Bradley T. Gong at the University of Washington School of Medicine, revealing scalp hair patterning as a clue to early fetal brain development. Smith and Gong provide a relatively indelible historical record of growth and topography of the brain and upper face during the fetal period from about 10-16 weeks. In the study, hair patterns of 200 normal children were compared to those of 129 patients with various brain conditions. The researchers report that aberrant scalp hair patterns seen in patients with severe defects of early brain development that must have been present before hair follicle development conform with this theory. These patterns follow the presumed shape and size of the brain at the time of hair follicle downgrowth, an obvious sign that a serious defect in brain development occurred before 10-16 weeks gestation. The researchers said that this is especially true for patients with severe microcephaly.

This article presents an abstract of the academic paper "Microcephalus Secondary to Birth Trauma," by Edgard Fincher published in a scholarly periodical. Eight cases are reported in which linear craniotomy has beneficial effects on irritative symptoms.

This article presents an abstract of the academic paper "Three Pedigrees of Microcephaly; with a Note on Their Genetic Implications," by S.L. Halperin, published in a scholarly periodical. Parental consanguinity may account for congenital microcephaly in more than 10 percent of the cases.