Case report: A choroidal fissure pial arteriovenous malformation inducing venous congestive edema of the medulla oblongata and cervicothoracic spinal cord presented with proximal arm predominant weakness

Frontiers in Neurology

Neurology

Aizhen Sheng¹  Yun Jiang¹  Ying Zhou²  Ximeng Yang³  Jun Lu³ 

[1] Department of Neurology, Beijing Hospital, National Center of Gerontology, Institute of Geriatric Medicine, Chinese Academy of Medical Sciences, Beijing, China;Department of Neurology, Beijing Hospital, National Center of Gerontology, Institute of Geriatric Medicine, Chinese Academy of Medical Sciences, Beijing, China;Department of Geriatrics, The First People's Hospital of Yunnan Province, Kunming, China;Department of Neurosurgery, Beijing Hospital, National Center of Gerontology, Institute of Geriatric Medicine, Chinese Academy of Medical Sciences, Beijing, China;

关键词: choroidal fissure;    intracranial arteriovenous malformation;    myelopathy;    interventional treatment;    intracranial dural arteriovenous fistula;   

DOI  :  10.3389/fneur.2023.1128366

 received in 2022-12-20, accepted in 2023-04-07,  发布年份 2023

来源: Frontiers

【 摘 要 】

Intracranial dural arteriovenous fistula (DAVF) can induce remote myelopathy via spinal perimedullary venous drainage. In the present study, we report a rare case of intracranial pial arteriovenous malformation (AVM)-related myelopathy. A 52-year-old man presented with progressive, predominantly proximal weakness and muscle atrophy in bilateral upper limbs, urinary retention, and hyperreflexia in bilateral upper and lower limbs. Brain and cervicothoracic MRI showed longitudinal myelopathy extending from the medulla oblongata to the T6 level, with perimedullary enlarged veins from the C1 to T12 level, and remarkable enhancement in bilateral anterior horns from the C2 to C7 level. Cerebral angiography revealed a choroidal fissure AVM, which was supplied by the left anterior choroidal artery and drained exclusively by an inferior ventricular vein descending toward the spinal perimedullary veins. After endovascular embolization of the feeding pedicle, nidus, and proximal segment of the draining vein, the patient's neurological deficits rapidly improved, and a significant recovery was achieved 3 months after the procedure. This rare case indicates that intracranial pial AVM can also cause extensive congestive myelopathy with similar mechanisms underlying intracranial and craniocervical DAVF cases, and gray matter in the spinal cord might be more susceptible to ischemia induced by intraspinal venous hypertension.

【 授权许可】

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Copyright © 2023 Jiang, Zhou, Yang, Sheng and Lu.