Vengalil Seena, Boddu Vijaykumar, Kulanthaivelu Karthik, Baskar Dipti, Nashi Saraswati, Pruthi Nupur, Bhargav Hemant, Uppar Alok M, Prasad Chandrajit, Kotra Madhulika, Polavarapu Kiran, Preethish-Kumar Veeramani, Nalini Atchayaram
Department of Neurology, National Institute of Mental Health and Neurosciences, Bengaluru, India.
Department of Neuroimaging and Interventional Radiology, National Institute of Mental Health and Neurosciences, Bengaluru, India.
Ann Indian Acad Neurol. 2025 Jul 1;28(4):574-578. doi: 10.4103/aian.aian_16_25. Epub 2025 Apr 22.
Hirayama disease (HD) is a cervical flexion-induced compressive myelopathy. Typically, there is forward displacement and loss of attachment of dural sac to lamina at the cervical level during neck flexion. However, the extent of the dural detachment (DD) has not been studied carefully. We undertook this study to know the extent of DD in HD.
We conducted a retrospective study of HD patients evaluated from 2015 to 2023. Patients with DD extending beyond the cervical spine were selected, and their clinical and radiological features were studied.
One hundred and thirty-two (62.8%) patients were identified to have DD beyond the cervical spine in a cohort of 210 HD patients. The mean age at onset and duration were 18.09 ± 2.3 years (13-26) and 38.63 ± 39.9 months, respectively. Proximo-distal involvement was noted in 50% of patients, while 33% and 17% of patients had isolated distal and proximal involvement, respectively. Wasted legs were observed in three patients. Cord atrophy was present in 96.9% of patients, extending from C5 to C7. Epidural detachment and engorgement of posterior epidural venous plexus were evident in all. DD extended from C2 to D10 vertebral level. The cranial extent of DD was from C2 to C4 in 87% of cases, and the caudal extent was D1-D5 in 84% of cases, extending up to D10 in two cases.
The HD spectrum continues to evolve phenotypically and radiologically. The pathophysiological mechanisms and DD extend beyond the cervical spine in a large proportion of patients. This makes it important to cover a longer part of the spine during imaging. This may have implications on the management of patients, particularly those with isolated lower limb involvement.
平山病（HD）是一种因颈部屈曲诱发的压迫性脊髓病。典型表现为颈部屈曲时，硬脊膜囊在颈椎水平向前移位并与椎板分离。然而，硬脊膜分离（DD）的范围尚未得到仔细研究。我们开展这项研究以了解平山病中硬脊膜分离的范围。
我们对2015年至2023年评估的平山病患者进行了一项回顾性研究。选取硬脊膜分离超出颈椎范围的患者，研究其临床和影像学特征。
在210例平山病患者队列中，有132例（62.8%）被确定存在超出颈椎范围的硬脊膜分离。发病时的平均年龄和病程分别为18.09±2.3岁（13 - 26岁）和38.63±39.9个月。50%的患者出现近端至远端受累，而分别有33%和17%的患者仅远端或近端受累。3例患者出现腿部肌肉萎缩。96.9%的患者存在脊髓萎缩，范围从C5至C7。所有患者均可见硬膜外分离及后硬膜外静脉丛充血。硬脊膜分离从C2椎体水平延伸至D10椎体水平。87%的病例硬脊膜分离的头端范围为C2至C4，84%的病例尾端范围为D1 - D5，2例患者延伸至D10。
平山病的表型和影像学特征仍在不断演变。其病理生理机制及硬脊膜分离在很大一部分患者中超出颈椎范围。这使得在影像学检查时覆盖更长节段的脊柱很重要。这可能对患者的治疗有影响，尤其是那些仅下肢受累的患者。
