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New data are reported concerning the specificities of architectonic disorders in oligophrenia, being especially marked at stages of idiocy and imbecility. The authors feel that the insufficiency of the cortical blood supply as a result of the congenital and dystrophic impairments of the angioarchitectonics may be essential for the development of oligophrenia.
The content of free amino acids and the dipeptide homocarnosine was examined in the cerebrospinal fluid of children with post-meningitis hydrocephalus. Of 17 identified amino acids the levels of threonine, methionine, leucine, alanine, lysine, histidine, glutamic acid and GABA appeared to be sharply changed. The homocarnosine level in the hydrocephalic children's CSF was found to be 10, and in hydrocephalic children with idiocy, 18 times higher than in the control.
The paper deals with a study of dematoglyphics in 458 oligophrenic patients (265 boys and 193 girls) from 7--18 years of age and 373 normal children of the same age. In all the ill boys, irrespective of the degree of mental retardation there was a decrease of arc patterns and an increase of the whorle frequency. In girls with oligophrenia in the degree of debility there was a significantly lower amount of arc patterns and an increase of whorles, the frequency of loops did not differ significantly with that of the control group. In girls in whom the oligophrenic defect was of the degree of imbecility or idiocy there was a more frequent amount of arc and whorle patterns at the expense of a decrease of loop patterns, while in the control group there was a somewhat more frequent amount of arc an loop patterns and less frequent--of whorles.
The authors give a description of 2 cases (sibs) with neurolipidosis of the group of amaurotic idiocy. There was an atypicity of the clinical and pathomophological picture: prolonged development of the disease (24 and 29 years, with an onset at 3-4 years), the absence of ocular changes, a massive lesion of the cortex in relative preservation of the subcortical areas, brain stem and cerebellum. The perspectiveness of a comprehensive clinico-morphological study is discussed in the interpretation of the genesis of different forms of neurolipidosis.
Myoclonic-astatic attack syndromes tend more than other attack forms to produce states. Their clinical picture can be various and therefore cause differential-diagnostic difficulties. On four own examples they are referred to. Most frequently it will be necessary to differentiate the hardly adjustable attack syndrome from on overdose of anticonvulsives. Also the state of absences, the state of psychomotoric attacks, and the impulsive-petit-mal state can make differential-diagnostic difficulties. Furthermore, additional encephalitic processes with cerebral decompensation in anyway injured children, and the myoclonic variants of familial amaurotic idiocy are to be to consider. An EEG examination is indispensable; consideration of the total situtation, observation of the course, and recording of the actual findings are necessary.
It was effected one study for fifteen children between two month and six years old with different diseases MDH (metabolic hereditary diseases) GM2 (Tay-Sachs and Sandhoff) (amaurotic idiocy), ceroid lipofuscinosis (Spielmeyer-Vogt-Batten-Mayou). Leigh maladie and other forms by MHD with unspecified diagnosis. It was effected neurologic exam, enzymatic measures, conjunctival biopsy, ophthalmologic exam with electroretinogram and evoked potentials. It was found an important element by differential diagnosis between gangliosidosis with normal ERG and ceroid lipofuscinosis with perturbate ERG. ERG is frequent precocious adulterated.
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