67 persons aged 19 to 33 were examined with P. T. C. solutions by the method of Harris and Kalmus (1949). Two persons (2.99%) distinguished between distilled water and a P. T. C. solution which they described as sweet. Four persons (5.97%) distinguished distilled water from P. T. C. which they, however, described as “sweet and bitter”. When both these categories were regarded as sweet tasters, the frequency was 8.96 per cent.

The observed frequency supports the hypothesis of an autosomal dominant mode of inheritance for sweet taste perception for P. T. C. (Skude 1959).

The literature and results of studies on the subject of lateral dominance have been reviewed and discussed, and pertinent hypotheses advanced. The various known facts need not be considered contradictory if multiple genetic factors are hypothesized, interacting with an ontogenetic predisposition to left cerebral (and right somatic) dominance, and with various psychological-social factors.

Three families from Calabria, whose members were related to each other have been studied. In the first there were both the thalas-semic and the drepanocytic traits separately in its members; in the second the thalassemic trait and in the third the drepanocytic trait. From the marriage of individuals with thalassemic trait from the first and the second family a child was born with Cooley's disease, from the marriage of drepanocytic individuals from the first and the third family a drepanocytic girl was born.

The clinical, hematologic and genetic characteristics of the 19 members of these families are reported and the distribution of the pathologic features is discussed.

The authors have described two brothers, both affected by epidermolysis bullosa, struck at the same time by poliomyelitis anterior acuta having identical paralytic manifestations concerning the lower limbs and an almost reversed asymmetrical affection concerning the muscular groups of the upper limbs. Owing to the presence of a congenital desease, the Authors have considered the existence in the above mentioned brothers of a particular predisposition also for poliomyelitic infection.

Description of a family five members of which, in four generations show the syndrome mentioned in the title. The mode of inheritance is dominant, with a rather strong penetrance and a great variability in the expressivity. The importance of genetic counseling is stressed.

The Authors have reported five twin-pairs, two of which DZ and three MZ, all affected by poliomyelitic paralysis, affirming the existence of a particular predisposition to this disease and even of a local predisposition in MZ pairs.

Eight sweet tasters for P. T. G. found in a preceding investigation were retested with P. T. G. solutions by using a discriminating test method. Variations in the course of a single day and during longer periods were studied. It was observed that the consistency of sweet tasting for this compound varied intraindividually as well as interindividually. One of the females showed a high consistency of taste sensations for different concentrations. Three persons never reported a sweet taste sensation of a supraliminal concentration, although one year earlier they had described different P. T. C. solutions as sweet or sweet and bitter at the same time. The remaining four persons showed a varying consistency of the number of solutions stated as sweet tasting and the concentrations of these sweet tasting solutions. The reasons for the variations of the consistency are unknown. Such inconsistencies influence the result of investigations into inheritance of the trait.

Early students of the M quadruplets considered that the dermatoglyphic traits and physical measurements corroborated a monozygotic origin. More careful analysis in terms of variance shows that the ridge counts, at least, are quite inconsistent with data from monozygotic twins. However, when several quantitative measures are considered together by a method of combined relative likelihoods of separate pair differences, the evidence against monozygosity is less impressive than the contrary evidence from qualitative traits including blood groups. It is concluded that quadruplets tend to vary more than twins and/or that some genotypes are much more variable than others, at least with respect to dermal ridge counts.

5 sisters out of 10 siblings were affected. Bearing an abnormally long appendix. No males showed the disease.

The M quadruplets have been regarded as monozygotic by all investigators who have studied them carefully. Previous authors have based their diagnosis on the description of the placenta, on early physical resemblances, and on qualitative features.

The addition of blood group tests to the criteria now tends to confirm the earlier conclusion. From an extension of methods devised for use in twins, it appears that the probability that these quadruplets originated from more than one zygote is less than one per cent. However, some quantitative differences, especially in finger prints, require a more elaborate analysis, and such an analysis will be presented and discussed in a second article.

A large Brazilian family, of white ancestry, in which several members presented a spinocerebellar ataxia, was studied. Informations were obtained covering six generations, in a total of 337 related individuals. 116 of them were examined by the author. 18 presented the disease, and of these 10 were alive at the time of the study. Besides them, by her descendents, it was possible to find a carrier of the gene, who died in a preataxic age.

The disease is transmitted by an autosomal dominant gene, and appears at the age of 38.33 ± 1.74 years. Cerebellar, posterior chord and pyramidal alterations were present in the sick persons. Almost all the patients presented disturbances of the temperature sense. The disease had not lowered the viability and fertility of the affected persons.

The Authors confirm the finding of a difference in the diameter of the nuclei of myelocytes between males and females.

The diameter is greater in the females, with a significance of 1‰ (Student's t).

In cases of myelocytic leukemia the difference is even greater, suggesting the hypothesis that in this disease the cells may be polyploid.

Three cases of hereditary myoclonic epilepsy have been observed among ten siblings in a Negro family. Electroencephalograms of the parents, three normal siblings and two of the three affected siblings have been recorded and all show abnormalities of a similar type. These are of a generalized nature revealing no focal damage. This type of abnormality has been observed in an affected male and two normal siblings by Watson and Denny-Brown.

The autosomal recessive mode of inheritance observed in the present study is consistent with the transmission most frequently reported in myoclonic epilepsy. We believe that abnormal electroencephalographic patterns are associated with this gene and that these patterns may be useful in the detection of heterozygous carriers.

A mongoloid girl with low-grade idiocy, the 10th of 12 children out of a very poor herds-mans' family in an isolate prealpine place of western Switzerland, was examined at the age of 28. Her mother is recovered on account of schizophrenia (catatonia tarda) as 4 of her (the probands) are feebleminded brothers.

At 21 and 23 years of age the mongoloid proband gave birth to a mongoloid daughter and a mongoloid son; while the latter died of bronchopneumonia when 17 months old — the diagnosis of his mongolism having been ascertained by a pediatrician the first was seen by the author at age 7. The mongolism of mother and daughter is shown by several photographs and by palm and sole prints.

The father of the 2 mongoloid children is most probably the probands' next old brother, a non mongoloid, but highly imbecille.

The Authors describe from the electro-phoretic, biochemical, spectrophotometric and chromatographic points of view the slow Hb as observed in 5 family groups from Ferrara and Bologna, in 4 of which they found an Hb L, while the 5th presented an Hb D. A new Hb is therefore added to the numerous abonrmal hemoglobines already found in Italy (Hb S, Hb G, Hb D, Hb K, Hb H, Hb J, abundant Hb A2): the Hb L, which is rather frequent in the region of Ferrara.

As for the Hb D, its presence in different regions of Italy is stressed, in as far as the first family, previously described, came from Calabria, and the present one from Bologna.

After a short review of the literature on the subject, the Authors report a personal observation of Cooley's disease in uniovular twins adding some general observations. They conclude by stressing the rarity of this observation.