Honnold Shelley P, Schulman F Yvonne, Bauman Karen, Nelson Kevin
Department of Veterinary Pathology, Armed Forces Institute of Pathology, Building 54, Room G117, Washington, DC 20306, USA.
J Zoo Wildl Med. 2010 Sep;41(3):530-4. doi: 10.1638/2009-0090.1.
A 6-yr-old captive-born female fennec fox (Vulpes zerda) had a history of multiple seizures and was treated with diazepam and phenobarbital therapy. Despite medical treatment, the seizures continued. They were intermittent and progressive, resulting in neurologic deficits and death of the animal within 6 mo of onset of the clinical signs. At necropsy, the animal was in good nutritional condition, and no gross lesions were noted in the brain. Histologically, amphophilic to basophilic, periodic acid-Schiff (PAS) positive, diastase-resistant inclusions were present in the brain, heart, and liver. Ultrastructurally, the inclusions were variably electron dense, fibrillary to occasionally granular, and non-membrane bound. The clinical, histologic, and ultrastructural findings were consistent with Lafora's disease, which in humans is a rare, fatal, autosomal recessive hereditary neurometabolic disorder characterized by progressive myoclonic epilepsy. This is the first report of Lafora's-like disease in a fennec fox.
一只6岁圈养出生的雌性耳廓狐（狐属沙狐）有多次癫痫发作史，曾接受地西泮和苯巴比妥治疗。尽管进行了药物治疗，癫痫仍持续发作。发作呈间歇性且进行性发展，导致该动物出现神经功能缺损，并在临床症状出现后的6个月内死亡。尸检时，该动物营养状况良好，脑部未发现明显病变。组织学检查显示，脑、心脏和肝脏中存在嗜酸性至嗜碱性、过碘酸雪夫（PAS）染色阳性、抗淀粉酶的包涵体。超微结构检查发现，这些包涵体电子密度不一，呈纤维状，偶尔为颗粒状，且无膜包被。临床、组织学和超微结构检查结果均符合拉福拉病，该病在人类中是一种罕见的、致命的常染色体隐性遗传性神经代谢疾病，其特征为进行性肌阵挛性癫痫。这是关于耳廓狐患类拉福拉病的首例报告。
