Summary
A 4-year-old horse was evaluated at the Colorado State University Veterinary Teaching Hospital for rapidly progressing cerebellar disease. Euthanasia was elected and at postmortem examination a proliferative mass encompassing the right side of the cerebellum was discovered. The lesion was characterized by large, convoluted, vascular folia and absence of the core of central white matter. Histologically, there was a diminution or loss of the internal granule cell layer, cavitation of the central white matter, and absence of Purkinje cells. The molecular layer was thickened with myelinated axons originating from large neurons aberrantly located in this zone. The pathology of this cerebellar mass closely resembles that seen in the cerebellum of humans with Lhermitte-Duclos disease. Mature adults are most commonly affected and clinical signs associated with this disease are often vague. The disease is thought to result from hypertrophy of granule cells possibly due to an early maturation or migration defect. Because of the late age of onset and the rarity of the disorder, its pathogenesis has been difficult to discover. Awareness that other mammalian species may be affected with a similar disorder may lead to an understanding of the causative developmental defect.
Article PDF
Similar content being viewed by others
Avoid common mistakes on your manuscript.
References
Ambler M, Pogacar S, Sidman R (1969) Lhermitte-Duclos disease (granule cell hypertrophy of the cerebellum). Pathological analysis of the first familial cases. J Neuropathol Exp Neurol 28:622–647
Lhermitte J, Duclos P (1920) Sur un ganglioneruome diffus du cortex du cervelet. Bull Assoc Fr Etude Cancer 9:99–107
Oppenheimer DR (1955) A benign “tumor” of the cerebellum. Report on two cases of diffuse hypertrophy of the cerebellar cortex with a review of nine previously reported cases. J Neurol Neurosurg Psychiatry 18:199–213
Reznik M, Schoenen J (1983) Lhermitte-Duclos disease. Acta Neuropathol (Berl) 59:88–94
Roessmann U, Wongmongkolrit T (1984) Dysplastic gangliocytoma of cerebellum in a newborn. J Neurosurg 60:845–847
Roski RA, Roessmann U, Spetzler RF (1981) Clinical and pathological study of dysplastic gangliocytoma. Case report. J Neurosurg 55:318–321
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
Poss, M., Young, S. Dysplastic disease of the cerebellum of an adult horse. Acta Neuropathol 75, 209–211 (1987). https://doi.org/10.1007/BF00687083
Received:
Accepted:
Issue Date:
DOI: https://doi.org/10.1007/BF00687083