Miyoshi Y, Taniwaki T, Arakawa K, Yamada T, Uda K, Inoue T, Kira J
Department of Neurology, Neurological Institute, Faculty of Medicine, Kyushu University.
Rinsho Shinkeigaku. 1999 Aug;39(8):836-41.
A 70-year-old woman noted paresthesia ascending from both legs to her thighs 27 months previously. She also suffered from urinary urgency and incontinence. Thereafter, weakness in both legs developed and gradually became worse. At the time of admission, a neurological examination revealed diffuse atrophy and mild spasticity in all four extremities, bilateral mild weakness in both upper extremities, and severe weakness in both lower extremities. Her superficial sensation was moderately impaired below the Th 3 level on her right side, and below the Th 4 level on her left side along with a mildly decreased sense of vibration in her left leg. Marked hyperreflexia in all four extremities and bilateral pathological reflexes were also observed. Pollakisurea, urinary incontinence and constipation were also present. Cervical MRI showed a swelling of the spinal cord at the C3 to C7 levels. Inside the spinal cord, low signal intensity lesions were seen on the T1-weighted MRI, and high signal intensity lesions were observed on the T2-weighted MRI, and the rim of the cervical cord was also enhanced by gadolinium-DTPA. MR angiography revealed enlarged and tortuous vessels at the craniocervical junction, thus suggesting the presence of a dural arteriovenous fistula (AVF). Vertebral arteriography demonstrated abnormal vessels at the spinomedullary junction supplied by the right vertebral artery, which drained into the anterior and posterior spinal veins. After surgically treating the dural AVF, the swelling of the spinal cord, the abnormal signals on MRI, and the clinical symptoms all markedly improved. Although most of the spinal dural AVF were located at the thoracic and lumbar levels, the present case was considered to be a very rare case of dural AVF, since it was located at the craniocervical junction and thus led to the development of cervical myelopathy.
一名70岁女性于27个月前注意到双侧腿部至大腿出现感觉异常。她还伴有尿急和尿失禁。此后,双腿出现无力并逐渐加重。入院时,神经系统检查发现四肢弥漫性萎缩和轻度痉挛,双侧上肢轻度无力,双侧下肢严重无力。右侧T3水平以下及左侧T4水平以下的浅感觉中度受损,同时左腿振动觉轻度减退。还观察到四肢明显的反射亢进和双侧病理反射。此外,还存在尿频、尿失禁和便秘。颈椎MRI显示C3至C7水平脊髓肿胀。在脊髓内部,T1加权MRI上可见低信号强度病变,T2加权MRI上观察到高信号强度病变,颈髓边缘经钆喷酸葡胺增强。磁共振血管造影显示颅颈交界处血管增粗、迂曲,提示存在硬脊膜动静脉瘘(AVF)。椎动脉造影显示右椎动脉供血的脊髓髓质交界处有异常血管,这些血管引流至脊髓前、后静脉。手术治疗硬脊膜动静脉瘘后,脊髓肿胀、MRI上的异常信号及临床症状均明显改善。虽然大多数脊髓硬脊膜动静脉瘘位于胸段和腰段,但本病例被认为是非常罕见的硬脊膜动静脉瘘病例,因为它位于颅颈交界处,从而导致了颈髓病的发生。