Maria Dambska

Early and late neuropathological changes in perinatal white matter damage.

Our material presents two patterns of white matter lesions in the brain of newborns dying with the clinical diagnosis of intrauterine or perinatal pathology: (1) classical periventricular ischemic infarction resulting in coagulative necrosis and (2) diffuse periventricular colliquative necrosis, in some cases involving the center of the cerebral convolutions. The majority of cases did not survive the first month of life. The cases with longer survival (up to six years) presented clinically with the syndrome of bilateral spastic cerebral palsy. Neuropathological examination showed dilation of the lateral ventricles, small cavities, and diffuse glial scars, not only in the periventricular white matter but also involving the axis of cerebral convolutions, as opposed to the relative sparing of cerebral cortex and other brain structures. These changes could be considered as evident or putative forms of a distinct type of perinatal brain damage. (J Child Neurol 1989;4:291-298).

Lissencephaly: two distinct clinico-pathological types

The present study is a review of four new cases of lissencephaly and two others previously reported. This study demonstrates that lissencephaly is a gross feature of the brain occurring in two different groups of cortical malformations. The first group, the classic agyria syndrome extensively analyzed by Jellinger and Rett [8] includes two types of abnormal cortical organization. They may be found in familial syndromes and also can appear sporadically. The second group includes smooth brains with the internal features of polymicrogyria and a more severely disorganized cortex. This type appears in familial lissencephaly in the cerebro-oculo-muscular syndrome, belonging to the same group as Fukuyama congenital-cerebro-muscular dystrophy. The other incidences of this type of cortical malformation require further investigation. The clinico-pathological differential diagnosis of two types of lissencephaly are also discussed.

An autopsy case of hemimegalencephaly

This case report is a neuropathological study of a ten-month-old infant with unilateral megalencephaly . In this anomaly neuronal migration defect and disturbances of cortical organization resulting in micropolygyria were the most striking neuropathological feature.

Myelination as a parameter of normal and retarded brain maturation

In this study a comparison of the myelination rate in humans in normal and pathologic conditions was made. The progress of myelination was examined on slides stained by the Klüver-Barrera method and evaluated as to four degrees. The prenatal myelination in the brain stem in a group of newborns who died of pregnancy pathology was correlated with normal brain stem myelination. Retardation of myelination by 2 to 10 weeks was found in cases with gestosis and diabetes in anamnesia. The myelin sheath formation in a group of children who died during the first three years of life after severe chronic diseases neoplastic and congenital heart failure was compared with the normal rate of postnatal myelination of the temporal lobe. The myelination process was evidently retarded by 2 to 20 months. The observations presented in this paper allowed the conclusion that pathologic conditions occurring in the maturing human may cause retarded maturation of myelin sheath.

Marginal Glioneuronal Heterotopias in Nine Cases With and Without Cortical Abnormalities

Nine cases of marginal glioneuronal heterotopias over the cerebral cortex were reviewed from the morphological point of view. There were developmental disabilities in all cases except one (case 8), who was stillborn. All subjects died before 1 year of age except one (case 5). The common features of small glioneuronal heterotopias and abundant heterotopic glioneuronal proliferation are described. The correlation of glioneuronal heterotopias with polymicrogyria and other cortical malformations, as well as their appearance over a normal cortex, are described. The glioneuronal heterotopias are considered to be a separate type of malformation that could arise during the second half of intrauterine life. A breach of the neuropial border seems to be the most acceptable pathomechanism for our presented cases. Their morphological features indicate that damage to this barrier leads to involvement of glioneuronal heterotopias in fusion of opposite cortical convolutions. (J Child Neurol 1986;1:149-157)

Correlations between clinical and neuropathological diagnosis of cortical anomalies in developmentally disabled children

The capabilities and limitations of clinical diagnoses, particularly brain imaging of cortical anomalies, in developmentally disabled children are reviewed. Some aspects of diagnostic problems in generalized cortical dysgeneses, like lissencephaly type I and II, subcortical heterotopias, generalized polymicrogyria, or focal cortical anomalies and primary micrencephalies, are discussed.