Díaz-Delgado Josué, Whitley Derick B, Storts Ralph W, Heatley Jill J, Hoppes Sharman, Porter Brian F
1 Department of Veterinary Pathobiology, College of Veterinary Medicine and Biomedical Sciences, Texas A&M University, College Station, TX, USA.
2 Antech Diagnostics (DBW), College of Veterinary Medicine and Biomedical Sciences, Texas A&M University, College Station, TX, USA.
Vet Pathol. 2018 Sep;55(5):711-718. doi: 10.1177/0300985818768033. Epub 2018 Apr 19.
Wobbly hedgehog syndrome (WHS) is a leading cause of neurologic disease in African pygmy hedgehogs (APHs; Atelerix albiventris). This study describes the signalment, clinical signs, gross, microscopic, and ultrastructural lesions of WHS in a cohort of 12 pet APHs. Microscopically, lesions consisted of status spongiosus of the white matter, typically bilateral and symmetrical, with myelin degeneration and loss that was accompanied by neuronal/axonal degeneration plus reactive microgliosis and mild, focal astrocytosis and astrogliosis. Lesions were most severe in the cerebellum and medulla oblongata, as well as cervical and thoracic spinal cord. Less affected areas were the corona radiata, corpus callosum, corpus striatum, internal capsule, and the mesencephalon. Ultrastructurally, the lesions consisted of splitting of the myelin sheath at the intraperiod line with subsequent focal expansion, resulting in status spongiosus, disruption, dilatation, rhexis, and phagocytosis. Based on these results, WHS is best described as a "spongy myelinopathy" with widespread central nervous system involvement.
摇摆刺猬综合征（WHS）是非洲侏儒刺猬（APHs；南非刺猬）神经系统疾病的主要病因。本研究描述了12只宠物非洲侏儒刺猬队列中WHS的征候、临床症状、大体、微观和超微结构病变。微观上，病变包括白质海绵状态，通常为双侧对称，伴有髓鞘变性和丢失，同时伴有神经元/轴突变性以及反应性小胶质细胞增生和轻度局灶性星形细胞增生和星形胶质细胞增生。病变在小脑、延髓以及颈髓和胸髓最为严重。受影响较小的区域是放射冠、胼胝体、纹状体、内囊和中脑。超微结构上，病变包括髓鞘在周期间线处分裂，随后局部扩张，导致海绵状态、破坏、扩张、破裂和吞噬作用。基于这些结果，WHS最好被描述为一种累及广泛中枢神经系统的“海绵状髓鞘病”。
