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Biomedical subjects

M Dambska

Publications and source records attributed to M Dambska.

At least 73 records · Page 4Linked to original sources

Cerebro-oculo-muscular syndrome: a variant of Fukuyama congenital cerebromuscular dystrophy.

Familial occurrence of cerebral malformations with muscular dystrophy was described by Fukuyama as congenital cerebromuscular dystrophy. We have observed a new syndrome belonging to the same group in three siblings. These syndromes differ in the degree of CNS involvement and abnormalities in the eye. The main clinical characteristics of our cohort were dysmorphic face, hypotonia, areflexia, failure to thrive, corneal opacity, cataract, dysgenesis of the anterior chamber of the eye, and death within the 1st year of life. Hydrocephalus and agyria were verified by computed tomography. Neuropathologic examination demonstrated malformations of the CNS. The agyric hemispheres with polymicrogyria in several cortical segments and severe cortical disorganization in other segments represented the principal anomaly. Congenital muscular dystrophy was also found. The CNS anomalies demonstrated a long-lasting pathologic process extending to involve the eye and muscle, which is most likely an inborn error of metabolism with autosomal recessive inheritance.

Abnormalities, Multiple↗

[Comparison of the transplacental effect of luminal and epileptic seizures in pregnant rabbits on the development of the fetal nervous system].

The aim of the study was to compare the effect of phenobarbital given to pregnant rabbits and of epileptic seizures on the course of pregnancy, the physical state, and central nervous system development of newborns. Phenobarbital was given orally during whole pregnancy or for the last ten days in amounts 18,5 mg/kg/body weight = 0,1 DL 50. Seizures of "grand mal" type were evoked with electrostimulator every day since the tenth day of pregnancy. Phenobarbital penetrates via placenta, reaches the organs of all fetuses, and seizures provoke hypoxaemia of fetuses. Phenobarbital administered during whole pregnancy exerts an teratogenous effect. Both injurious factors applied after teratogenous period cause a retardation of general development of fetuses. After phenobarbital administration slight brain lesions are visible in the light, electron microscopical and histochemical examination. More evident was the retardation of the development of the central nervous system after both of the examined injurious factors. In the brain stem and in the cortex the neurons are less mature and the synaptic junctions in the cortex are less numerous. In our experimental models phenobarbital seems to evoke more noxious effect than seizures in mothers.

Anencephaly↗

Topography of lesions in newborn and infant brains following cardiac arrest and resuscitation. Damage to brain and hemispheres.

The brain lesions produced by temporary arrest of circulation in a newborn and an 11-month-old infant are described. In the newborn, two periods of arrest occurred, one on the fifth day after birth and the second a few day before death on the sixth week. The older infant suffered a single episode of cardiac arrest at the age of 11 months and survived 8 days. In both cases, postmortem examination revealed lesions in spinal cord, in brain stem, and in cerebral hemispheres. This distribution of damage is compared with the patterns of injury produced experimentally by episodes of partial (hypoxia) and total (anoxia) asphyxia in subhuman primates. The coexistence of hemispheral and brain stem nuclear patterns of pathology indicates that both hypoxia and anoxia had occurred in the present cases.

Brain↗

[Diagnostic difficulties in brain strokes in the light of personal observations].

The authors report a clinico-neuropathological analysis of 350 case records and autopsy protocols of patients dying from stroke in the years 1962-1966 at the Department of Neurology of the Grochów Hospital in Warsaw. Full agreement of clinical diagnosis and autopsy diagnosis was found in 314 cases while in 36 cases (about 10%) the clinical diagnosis was not correct. The authors believe that the diagnostic difficulties encountered by clinicians are due frequently to a complex pathological mechanism of vasogenic lesions in the brain and frequency of encephalomalatic foci found in the same patient beside haemorrhagic lesions. In the present material 116 (33%) such cases have been found.

Autopsy↗