Lafora's-Like Disease in a Fennec Fox (Vulpes zerda)

Abstract 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 ...