Department of Experimental and Clinical Neuropathology MMRI
Clinical, anatomical and histological diagnosis
Histological diagnosis: Agenesis hemisphaerium cerebelli. Vere similiter atresia completa foraminum Zuschka. Hydrocephalus internus. Atrophia secundaria hemisphaerium cerebri.Autopsy examination of 2,5-year-old patient was performed. Neuropathological evaluation in light microscopy was based on brain paraffin sections stained with Cresyl-violet, Heidenhain, Holzer, Bojan and Van Gieson's method.The structure of the cerebral mantle and basal ganglia was normal and the gyrus, although small, were present in adequate number. However, a huge, bloated ventricular system was found, which probably compressed the brain tissue and pushed it peripherally towards the cranial bones. Due to this compression, the circulation was impaired. Arterial and venous vessels had thick, fibrotic walls and were surrounded by "lakes" of the transudate. Neuronal atrophy was observed in the cortex and basal nuclei and compensatory proliferation of cellular and fibrous glia. The meninges were thick, and numerous fresh extravasations were found in them, as well as evidence of old hemorrhages in the form of hemosiderin. The cause of all these abnormalities appears to be malformation of the cerebellum. The cerebellum developed only in the central part, the cerebellar hemispheres were completely missing. Enormous internal hydrocephalus pushed ventricle IV, which surrounded almost all around the brainstem structures, expanded ventricle III and lateral ventricles, causing an image of secondary cerebral atrophy. Vascular changes and epidural hemorrhages should be interpreted as secondary. There were no data enabling the diagnosis of primary inflammatory process or post-necrotic scarring. Therefore, the picture should be considered as a result of congenital malformation of the cerebellum and its connections with the brainstem.Histological diagnosis: Agenesis hemisphaerium cerebelli. Vere similiter atresia completa foraminum Zuschka. Hydrocephalus internus. Atrophia secundaria hemisphaerium cerebri.Autopsy examination of 2,5-year-old patient was performed. Neuropathological evaluation in light microscopy was based on brain paraffin sections stained with Cresyl-violet, Heidenhain, Holzer, Bojan and Van Gieson's method.The structure of the cerebral mantle and basal ganglia was normal and the gyrus, although small, were present in adequate number. However, a huge, bloated ventricular system was found, which probably compressed the brain tissue and pushed it peripherally towards the cranial bones. Due to this compression, the circulation was impaired. Arterial and venous vessels had thick, fibrotic walls and were surrounded by "lakes" of the transudate. Neuronal atrophy was observed in the cortex and basal nuclei and compensatory proliferation of cellular and fibrous glia. The meninges were thick, and numerous fresh extravasations were found in them, as well as evidence of old hemorrhages in the form of hemosiderin. The cause of all these abnormalities appears to be malformation of the cerebellum. The cerebellum developed only in the central part, the cerebellar hemispheres were completely missing. Enormous internal hydrocephalus pushed ventricle IV, which surrounded almost all around the brainstem structures, expanded ventricle III and lateral ventricles, causing an image of secondary cerebral atrophy. Vascular changes and epidural hemorrhages should be interpreted as secondary. There were no data enabling the diagnosis of primary inflammatory process or post-necrotic scarring. Therefore, the picture should be considered as a result of congenital malformation of the cerebellum and its connections with the brainstem.Histological diagnosis: Agenesis hemisphaerium cerebelli. Vere similiter atresia completa foraminum Zuschka. Hydrocephalus internus. Atrophia secundaria hemisphaerium cerebri.
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Mossakowski Medical Research Institute PAS
Library of the Mossakowski Medical Research Institute PAS
Feb 1, 2022
Jul 9, 2021
41
https://rcin.org.pl./publication/232845
Edition name | Date |
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opis nr 119/65 | Feb 1, 2022 |
Mossakowski, Mirosław Jan (1929–2001)