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Biomedical subjects

M K Nedz'ved'

Publications and source records attributed to M K Nedz'ved'.

At least 37 records · Page 2Linked to original sources

[Clinical picture and diagnosis of amyotrophic leukospongiosis].

Within a period of 24 years 17 patients suffering from a specific spinal amyotrophy were observed in Byelorussia. The disease steadily progressed and ended in death. Family and group morbidity, and less frequently sporadic cases were registered. Clinical examinations revealed a selective affection of the anterior horns resembling subacute anterior poliomyelitis. The truncal structures remained intact during the whole course of the disease. Two or three years after the disease onset spinal disturbances of respiration joined. The average duration of the disease was 2.5 to 3 years. Morphological examinations revealed total destruction of thoracic mononeurons and spongiosis of the white matter of the brain and the spinal cord, but without signs of demyelinization of the corticospinal tract. The specificity of the epidemiological, clinical, and pathomorphological features of the disease has given the authors grounds to designate it as "amyotrophic leukospongiosis".

Adult↗

[Acid and alkaline phosphatases in the cerebellar structures in autosomal trisomy syndromes].

The activity of nonspecific phosphomonoesterases in cerebellar structure of newborns and infants with trisomies in the 13th (5 cases) 18th (4) and 21st (14) chromosomes was investigated. In all trisomies, changes of phosphatase activity in the cerebellar structures were demonstrated which were particularly marked with alkaline phosphatase. The activity of this enzyme increased not only in the vascular endothelium but in a number of cellular structures as well: in the external embryonal and internal granular layers of the cortex, in the nuclei of Purkinje cells and neurons of the nucleus dentatus, in the foci of heterotopia of cortical cells into the white matter and nucleus dentatus. In Edwards' syndrome the activity of alkaline phosphatase in cellular elements of the cerebellum was slightly higher than in the control group. Changes in the activity was observed in the cells of the external embryonal layer, neurons of the nucleus dentatus and in the cells of the heterotopic foci. In autosomal trisomies dystopic cells have dissimilar levels of phosphatase activity and stain mosaically which seems to confirm the assumption of their dissimilar differentiation.

Acid Phosphatase↗

[Metachromatic leukodystrophy (pathogenesis and pathological anatomy)].

An observation of metachromatic leukodystrophy in a child is described indicating the main criteria for macroscopic and histological diagnosis. The authors believe demyelinization and loss of oligodendroglia in this disease to be due to the toxic effect of excess sulphatides and derivatives thereof.

Autopsy↗

[Multiple developmental defects. The Neu-Povýsilová syndrome].

An observation of Neu-Povýsilová lethal syndrome of multiple congenital malformations and data on 6 cases of this syndrome described in the literature are presented. The main morphological manifestations of the syndrome include markedly manifest prenatal hypoplasia, defects of the CNS and the extremities. The presumed type of heredity is autosomal-recessive.

Abnormalities, Multiple↗

[Clinico-morphologic features of subacute anterior poliomyelitis].

The paper deals with clinical and morphological studies of 2 cases with subacute anterior poliomyelitis. The authors attract special attention to the development of flaccid pareses with a simultaneous inhibition of tendinous and periosteal reflexes, the absence of any pyramidal signs during the entire disease and a clear prevalence of degeneration in spinal cord motoneurons in the cervical and thoracel parts and their preservation in the brain cortex. These symptoms are distinctive signs from the poliomyelitic variant of the amyotrophic lateral sclerosis.

Adult↗

[Pathological anatomy of defects of the prosencephalic group].

Morphological changes in the brain were studied in 14 observations of defects of the prosencephalic group. These defects were shown to be of three kinds: prosencephaly, alobar prosencephaly, and holoprosencephaly, and to be due to partial or complete undivision of the cranial part of the medullary tube. The nature of these defects is heterogeneous. Most frequently prosencephalitic defects are due to autosomal aberrrations (trisomy 13 and 18, 13-q-, 18p-, triploidy), and may also be due to gene mutations. In cases of trisomy 13 these defects are regularly accompanied by structural disorders of cytoarchitectonics of the cortex of the end brain, cerebellum, and medulla, which may be important for differential diagnosis of prosencephaly of various etiologies.

Brain↗

[Pathological anatomy of Edwards' syndrome (trisomy 18)].

Data of the literature (200 section observations) and those of 10 authors' own observations on the syndrome of trisomy with relation to chromosome 18 are presented. The pathologoanatomic diagnosis of Edwards' syndrome can be established without an investigation of chromosomes on the basis of a complex of congenital defects present in the newborn. The main of them are: prenatal hypoplasia, dolichocephaly, microgenia, concha auriculae drawn in the horizontal plane, flexor position of bones, shortness and thickness of the great toe and protruding heel, thickness and distortion of convolution of the dorsal lip of the olivary nucleus, hypoplasia of the cerebellum, heterotopia of the piriform Purkinje cells into the white matter of the cerebellum and foci of nondifferentiated cells in the white matter of the great hemispheres; complex cardiac defects--Meckel's diverticulum, fused kidneys (horseshoe- or L-shaped) with small cysts in the renal cortex; and the presence of 10 or more arches on fingers and toes in combination with proximal or intermediate localization of axial triradius.

Abnormalities, Multiple↗

[A peculiar form of spinal amyotrophy encountered in a limited area of Belorussia].

The authors analyzed the data obtained in a study of 7 cases of peculiar spinal amyotrophy seen in some patients who lived in 3 adjacent villages. The disease had an incipient onset, with a prevalent localization of the pathological process in the anterior horns of the cervical part of the spinal cord and a subsequent progressive development with a lethal outcome accompanied by symptoms of respiratory insufficiency of the spinal type. The CNS was characterized by specific morphological changes. The authors are more in favour of the assumption that this disorder is of infectious nature of the group of gradual infections of the CNS.

Adult↗

[The clinical characteristics of progressive focal epilepsy with a herpetic etiology].

Cases are described of chronic zoster encephalitis presenting with the leading progressive focal epilepsy syndrome. The clinical presentation of the trouble can include both general cerebral signs and those of the focal affection of the central nervous system only. The progressive course and duration of remission suggest to us a persisting infection manifesting against the background of immune deficiency.

Adolescent↗

[Cladosporiosis in chronic herpetic encephalomyelitis].

One case of chronic herpetic meningoencepalitis complicated by a rare my costs of the central nervous system (CNS) and generalised viral infection is described. Cladosporiosis is considered as a lesion resulting from a immunosuppression due to chronic herpetic involvement of CNS. Morphologically this mycosis is manifested by the presence of spors and septic micelium among cells of productive inflammation an inside of giant multinuclear cells in affected areas. Viral nature of the encephalomyelitis is documented by presence of typical intranuclear inclusions and detection of herpetic antigen by means of fluorescent antibodies.

Adult↗