4.1 Article

Leucoencephalopathy with cerebral calcinosis in a young chimpanzee (Pan troglodytes) - a case report

Journal

JOURNAL OF MEDICAL PRIMATOLOGY
Volume 36, Issue 6, Pages 385-390

Publisher

WILEY
DOI: 10.1111/j.1600-0684.2006.00199.x

Keywords

Aicardi-Goutieres syndrome; angiopathy; brain calcification; Fahr's disease; neurodegenerative disorder; nonhuman primate

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Case history A 4-year-old chimpanzee (Pan troglodytes) had a clinical history of a 2-year progressive central nervous dysfunction including convulsions and severe paralysis. Results Gross pathology revealed cerebral atrophy, ventricular enlargement and a severe encephalomalacia with extensive calcifications. Histologically, the white matter showed diffuse demyelination as well as vascular and perivascular calcifications which also involved the basal ganglia. Blood vessels with less distinctive calcium deposits exhibited periodic acid Schiff positive hyalinosis. Large areas of necrosis, hemorrhage and intense gliosis were also present. Activation of astrocytes and macrophages was confirmed by immunohistochemical methods. Conclusions The etiology of the leucoencepalopathy could not be ascertained by macroscopic, histological and immunohistochemical examinations. Potential differential diagnoses include the rarely occurring Fahr's disease in humans, arteriosclerosis, storage disease and the Aicardi-Goutieres syndrome. Based on the results of the postmortal examinations Fahr's disease is regarded as the most likely diagnosis in the present case of the chimpanzee.

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