Zöller M, Grevot A, Mätz-Rensing K, Hofmann P, Jurek V, Schulz-Schaeffer W, Kaup F-J
Department of Infectious Pathology, German Primate Center, Göttingen, Germany.
J Med Primatol. 2007 Dec;36(6):385-90. doi: 10.1111/j.1600-0684.2006.00199.x.
A 4-year-old chimpanzee (Pan troglodytes) had a clinical history of a 2-year progressive central nervous dysfunction including convulsions and severe paralysis.
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.
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-Goutières 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.
一只4岁的黑猩猩（黑猩猩属）有2年进行性中枢神经功能障碍的临床病史，包括抽搐和严重瘫痪。
大体病理学显示脑萎缩、脑室扩大以及严重的脑软化并伴有广泛钙化。组织学上，白质显示弥漫性脱髓鞘以及血管和血管周围钙化，基底节也受累。钙沉积不太明显的血管呈现过碘酸希夫阳性透明变性。还存在大面积坏死、出血和强烈的胶质增生。免疫组化方法证实星形胶质细胞和巨噬细胞活化。
通过大体、组织学和免疫组化检查无法确定白质脑病的病因。潜在的鉴别诊断包括人类罕见的法尔病、动脉硬化、贮积病和艾卡迪 - 古铁雷斯综合征。基于尸检结果，法尔病被认为是本病例黑猩猩最可能的诊断。
