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[1例成人T细胞白血病病毒1型相关脊髓病伴额叶白质弥漫性病变及头颅MRI示锥体束连续性病变]

[A case of HTLV-1 associated myelopathy with diffuse white matter lesion of the frontal lobe and continuous lesion of the pyramidal tract on cranial MRI].

作者信息

Konagaya M, Iida M

机构信息

Department of Neurology, Suzuka National Hospital.

出版信息

Rinsho Shinkeigaku. 1991 Aug;31(8):875-7.

PMID:1764864
Abstract

In this report, the characteristic findings of cranial MRI of a case with HTLV-1 associated myelopathy (HAM) is described. The patient was a 62-year-old woman with 6 years history of paraplegia. Her main clinical signs were bilateral spastic paraplegia in the lower limb girdle muscles and extremities, paresthesia below the tenth thoracic cord level and urinary disorder. There was bilateral blepharoptosis. Hyperreflexia was observed in the examination of the cranial nerves and upper extremities. She showed no dementia or any other higher cortical dysfunctions. Positive anti-HTLV-1 antibody in the serum and cerebrospinal fluid established a diagnosis of HAM. T2 weighted MRI study revealed the symmetrical diffuse hyperintensity in the subcortical white matter of the frontal lobe and temporal lobe. The hyper-intensity was also observed in the bilateral internal capsule-peduncular base junction and pontine base, which indicated the continuous lesions in the intracranial pyramidal tracts. These MRI findings were different from those of reported cases of scattered lesion in central nervous system. There are some speculations for the nature of the diffuse lesion in the white matter, i.e., fusion of solitary gliosis and/or perivascular cuffing, or diffusely advanced spongy state. The continuous lesion of the pyramidal tract is suspected a systemic demyelination. Although the pathomechanism still remains uncertain, HTLV-1 infection generally affects the spinal pyramidal tract. It seems that this case is selectively affected in the pyramidal tracts in CNS.

摘要

在本报告中,描述了1例人类嗜T淋巴细胞病毒1型(HTLV-1)相关脊髓病(HAM)患者的头颅MRI特征性表现。患者为一名62岁女性,有6年截瘫病史。其主要临床体征为双侧下肢带肌和四肢痉挛性截瘫、胸10脊髓水平以下感觉异常及排尿障碍。存在双侧上睑下垂。在脑神经和上肢检查中观察到反射亢进。她没有痴呆或任何其他高级皮质功能障碍。血清和脑脊液中抗HTLV-1抗体阳性确诊为HAM。T2加权MRI研究显示额叶和颞叶皮质下白质对称性弥漫性高信号。双侧内囊-脚间基底交界处和脑桥基底部也观察到高信号,提示颅内锥体束连续性病变。这些MRI表现与报道的中枢神经系统散在病变病例不同。对于白质弥漫性病变的性质有一些推测,即孤立性胶质增生和/或血管周围套叠融合,或弥漫性进展的海绵状状态。锥体束的连续性病变怀疑是系统性脱髓鞘。虽然发病机制仍不确定,但HTLV-1感染通常影响脊髓锥体束。看来该病例在中枢神经系统的锥体束中受到选择性影响。

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