Rossmeisl John H, Cecere Thomas E, Kortz Gregg D, Geiger David A, Shinn Richard L, Hinckley Jonathan, Caudell David L, Stahle Jessica A
Veterinary and Comparative Neuro-Oncology Laboratory, Department of Small Animal Clinical Sciences, Virginia-Maryland College of Veterinary Medicine, Virginia Tech, Blacksburg, VA, United States.
Department of Biomedical Sciences and Pathobiology, Virginia-Maryland College of Veterinary Medicine, Virginia Tech, Blacksburg, VA, United States.
Front Vet Sci. 2019 Jul 5;6:219. doi: 10.3389/fvets.2019.00219. eCollection 2019.
Intramedullary signal change (ISC) is a non-specific finding that is frequently observed on magnetic resonance imaging (MRI) examinations of the canine spinal cord. ISC can represent a variety of primary pathological processes such as neoplasms or myelitides or secondary changes such as edema, cysts, gliosis, or myelomalacia. An unusual phenotype of ISC is the "snake-eye" myelopathy (SEM), which refers to bilaterally symmetric T2 hyperintensities preferentially affecting the ventral horn gray matter on transverse MR images, which resemble a pair of snake's eyes. The pathophysiology of SEM is poorly understood in humans, and this imaging finding may be associated with cervical spondylotic myelopathy, spinal cord ischemia, ossification of the posterior longitudinal ligament, amyotrophic lateral sclerosis, and Hirayama disease. Here we describe four dogs with cervical MRI examinations consistent with an SEM-like phenotype. All dogs initially presented with a central cord syndrome or tetraparesis referable to a C6-T2 neuroanatomic localization, which was attributed to disc-associated spinal cord compression in three cases, while one dog had the SEM-like phenotype with no identifiable etiology. Once the SEM-like phenotype was present on MRI examinations, dogs demonstrated insidious clinical deterioration despite therapeutic interventions. Deterioration was characterized by lower motor neuron weakness and neurogenic muscle atrophy progressing to paralysis in the thoracic limbs, while neurological functions caudal to the level of the SEM-like lesion remained largely preserved for months to years thereafter. Neuropathological features of the SEM-like phenotype include multisegmental cavitations and poliomyelomalacia of laminae VI-IX of the caudal cervical spinal cord, although the lesion evolved into pan-necrosis of gray matter with extension into the adjacent white matter in one case with an 8 years history of progressive disease. Although the pathophysiology of SEM remains unknown, the topographical distribution and appearance of lesions is suggestive of a vascular disorder. As the SEM-like phenotype was uniformly characterized by longitudinally and circumferentially extensive neuronal necrosis, results of this small case series indicate that dogs with clinical signs of central cord syndrome and the SEM-like phenotype involving the cervicothoracic intumescence on MR examinations have a poor prognosis for the preservation or recovery of thoracic limb motor function.
脊髓内信号改变(ISC)是一种非特异性表现,在犬脊髓的磁共振成像(MRI)检查中经常观察到。ISC可代表多种原发性病理过程,如肿瘤或脊髓炎,或继发性改变,如水肿、囊肿、胶质增生或脊髓软化。ISC的一种不寻常表型是“蛇眼”脊髓病(SEM),指在横轴位MR图像上双侧对称的T2高信号,优先累及腹侧角灰质,形似一对蛇眼。SEM在人类中的病理生理学知之甚少,这种影像学表现可能与颈椎病性脊髓病、脊髓缺血、后纵韧带骨化、肌萎缩侧索硬化症和平山病有关。在此,我们描述了四只经颈椎MRI检查符合SEM样表型的犬。所有犬最初均表现为中央脊髓综合征或四肢轻瘫,神经解剖定位为C6 - T2,其中三例归因于椎间盘相关的脊髓压迫,而一只犬具有SEM样表型但病因不明。一旦MRI检查出现SEM样表型,尽管进行了治疗干预,犬仍表现出隐匿性临床恶化。恶化的特征是下运动神经元无力和神经源性肌肉萎缩,进展为胸肢麻痹,而在SEM样病变水平以下的神经功能在数月至数年时间内基本保持。SEM样表型的神经病理学特征包括颈段尾侧脊髓第VI - IX层的多节段空洞形成和脊髓灰质炎样软化,尽管在一例有8年进行性疾病病史的病例中,病变演变为灰质全坏死并扩展至相邻白质。尽管SEM的病理生理学仍然未知,但病变的地形分布和外观提示血管疾病。由于SEM样表型均以纵向和周向广泛的神经元坏死为特征,这个小病例系列的结果表明,在MR检查中出现中央脊髓综合征临床体征且SEM样表型累及颈胸膨大的犬,其胸肢运动功能的保留或恢复预后不良。