Tsuzaka K, Takada T, Moriwaka F, Tashiro K, Akino M
Department of Neurology, Hokkaido University School of Medicine.
Rinsho Shinkeigaku. 1991 Feb;31(2):206-9.
A 41-year-old man was doing well until July 1989, when he noted numbness over soles, followed 4 months later by difficulty in walking. These symptoms were progressively getting worse, and he was admitted to our department on June 12, 1990. General physical examination was unremarkable. Neurologically cranial nerves were intact except old right, traumatic strabismus. Muscle tone and deep tendon reflexes were normal throughout, but bilateral Babinski and Chaddock reflexes were present. Mild weakness of lower extremities were found on muscle testing (4/5). In sensory system, superficial sensory disturbance below T10 was seen, and markedly diminished vibration and position senses of lower extremities were noted. Cerebellar test was intact, although unsteadiness was found on heel-shin test. Romberg sign was definitely positive. His gait was wide-based and ataxic. Laboratory data showed no abnormalities in CBC, chemistry, urinalysis, serological tests and endocrinological examinations. Spinal MRI (Siemens 1.5 Tesla) showed abnormal deposition of epidural fatty tissues compressing spinal cord with flattening of cord from T4 to T8. Spinal ataxia as compressive myelopathy due to epidural lipomatosis was considered and he underwent laminectomy from T4 to T8 with improvement in walking. Epidural lipomatosis is an unusual cause of spinal cord compression, presenting compressive myelopathy, radiculopathy, cauda equina syndrome, intermittent claudication, or back pain. Most of cases were associated with long-term administration of adrenocortical steroid hormone, or underlying diseases, except only 3 cases including ours. This is the first case of spinal epidural lipomatosis presenting progressive gait disturbance due to spinal ataxia.
一名41岁男性在1989年7月前情况良好，当时他注意到脚底麻木，4个月后出现行走困难。这些症状逐渐加重，于1990年6月12日入住我科。全身体格检查无异常。神经系统检查显示，除陈旧性右侧外伤性斜视外，颅神经均正常。肌张力和深腱反射全程正常，但双侧巴氏征和查多克征阳性。肌肉测试发现下肢轻度无力（4/5）。感觉系统方面，T10以下存在浅感觉障碍，下肢振动觉和位置觉明显减退。小脑测试正常，尽管跟膝胫试验发现不稳。闭目难立征明确阳性。他的步态宽基且共济失调。实验室检查数据显示血常规、生化、尿常规、血清学检查和内分泌检查均无异常。脊柱MRI（西门子1.5特斯拉）显示硬膜外脂肪组织异常沉积，压迫脊髓，导致脊髓从T4至T8变平。考虑为硬膜外脂肪增多症所致的脊髓压迫性共济失调，他接受了T4至T8椎板切除术，术后行走情况有所改善。硬膜外脂肪增多症是脊髓压迫的罕见原因，可表现为压迫性脊髓病、神经根病、马尾综合征、间歇性跛行或背痛。大多数病例与长期使用肾上腺皮质类固醇激素或基础疾病有关，除了包括我们这例在内的仅3例。这是首例因脊髓性共济失调导致进行性步态障碍的脊柱硬膜外脂肪增多症病例。
