Author/Editor		Poljaković, Z; Jadro-Šantel, D; Bešenski, N; Popović, M; Pažanin, L; Negovetić, L
Title		Dysplastic granulo-molecular hypertrophy of the cerebellar cortex (L'hermitte-Duclos disease): report of three cases
Type		članek
Source		Neurologija
Vol. and No.		Letnik 39, št. 4
Publication year		1990
Volume		str. 259-71
Language		eng
Abstract		In this paper three cases with peculiar disease of the cerebellar cortex are presented. The disease is well known as L'hermitte-Duclos disease (LDD), but nowadays it is also calle "dysplastic granulo-molecular hypertrophy of the cerebellar cortex" according to the modern theories of its etiology. Curious changes of the cerebellum in LDD are probably the result of a combination of delayed migration of Purkrinje cells which occurs during the intrauterine period of cerebellar development, and of reactive hypertrophy of granular cells. Presented cases of L'hermitte-Duclos disease are, according to the literature, more or less typical by their clinical appearance and by their histological findings. Our electromicroscopical data support the findings of those authors who found only axodendritic sinapses on the hypertrophied ganglion cells, according to the idea that those cells are originally granular cells of the cerebellar cortex. CT scan of all our patients revealed parallel, worm-like, hyperdense formations in a hypodense process of the cerebellum. The same picture existed in some other described patients, but attention was paid to it. We consider this picture on the computed tomography to be characteristic, if not even paognomonic for the L'hermitte-Duclos disease, which gives us an entirely new scientific contribution in the process of the diagnosis of this peculiar disease.
Descriptors		CEREBELLAR DISEASES CEREBELLAR NEOPLASMS CEREBELLUM HYPERTROPHY TOMOGRAPHY, X-RAY COMPUTED ADULT