Ein Fall von Gangliocytoma dysplasticum des Kleinhirns

A Benign "Tumour" of the Cerebellum: Report on Two Cases of Diffuse Hypertrophy of the Cerebellar Cortex With a Review of Nine Previously Reported Cases

“…They were also disposed to relate the condition to the concomitant congenital abnormalities-polydactyly and hydromyelia in Case 3, and leontiasis, heterotopia cerebelli, and tumour proneness in Case 2. Foerster and Gagel (1933) pointed out the similarity of the cellular picture with that of ganglion cell tumours elsewhere in the brain, and agreed with Bielschowsky and Simon that the condition is neoplastic, probably consisting of cells of the Purkinje family, and arising on the basis of a developmental anomaly. They called it " gangliocytoma with a dysplastic basis ".…”

“…Case 4.-This case was described by Foerster and Gagel (1933) as one of a series of ganglion cell tumours of the brain. Femriale, aged 43.…”

Lhermitte-Duclos disease (dysplastic cerebellar gangliocytoma): a malformation, hamartoma or neoplasm?

“…The authors originally described the lesion under the name of “ganglioneurome myélinique diffus de l'écore cérébelleuse”. Subsequently, this entity was referred to by a variety of synonymous descriptions, such as diffuse ganglioneuroma of the cerebellar cortex (2, 3) (benign) hypertrophy of the cerebellum (4–6), Purkinjoma (7), hamartoma of the cerebellum (2, 8), gangliocytoma myelinicum diffusum of the cerebellar cortex (9), dysplastic gangliocytoma of the cerebellum (10–14), granule cell hypertrophy of the cerebellum (15) or simply Lhermitte–Duclos disease (16–20).…”

Diffuse hypertrophy of the cerebellum

“…And is the lesion truly neoplastic? Earlier authors supported the view that the abnormal cells are derived from the granular layer, though Foerster and Gagel (1933) and later Christensen (1937) and still more recently Courville (1958) have favoured an origin in the Purkinje neurones, Christensen indeed coining the name 'Purkinjeoma'. Several features of the lesion, however, reinforce the suggestion of a granule cell origin: there is the position of the cells in the granular layer, and the fact that, like the granule neurones, the fibres of these abnormal cells run parallel to the folia.…”