나선형 정맥확장증을 동반한 거대 뇌간 동정맥기형

Giant Brainstem Arteriovenous Malformation with Spiral Venous Ectasia

Article information

J Korean Neurol Assoc. 2024;42(1):84-85

Publication date (electronic) : February 1, 2024

doi : http://dx.doi.org/10.17340/jkna.2023.0056

Jusuck Lee, MD , Yoonha Hwang, MD ^,^*, Sang-Mi Noh, MD, PhD ^,^*

Department of Neurology, The Catholic University of Korea St. Vincent’s Hospital, Suwon, Korea

이주석, 황윤하^,^*, 노상미^,^*

가톨릭대학교 성빈센트병원 신경과

Address for correspondence Yoonha Hwang, MD Department of Neurology, The Catholic University of Korea St. Vincent’s Hospital, 93 Jungbu-daero, Paldal-gu, Suwon 16247, Korea Tel: +82-31-249-8887 Fax: +82-31-249-2709 E-mail: yoonhaha10@gmail.com

Sang-Mi Noh, MD, PhD Department of Neurology, The Catholic University of Korea St. Vincent’s Hospital, 93 Jungbu-daero, Paldal-gu, Suwon 16247, Korea Tel: +82-31-249-8887 Fax: +82-31-249-2709 E-mail: heru-birch@hanmail.net

These two authors contribute equally in this article as the corresponding author.

received : June 23, 2023 , rev-recd : August 30, 2023 , accepted : August 30, 2023 .

특이 기저질환 없는 26세 남자가 7년 전 발생한 좌측 운동완만으로 신경과를 방문하였다. 근력은 정상이나 뛸 때 좌측 다리가 느리고, 왼손 떨림은 점차 악화되어 우산이 흔들려서 들 수 없었다. 1년 전 눈동자가 왼쪽으로 가지 않는 것을 발견했으나 복시로 인한 불편함은 몰랐다. 진찰에서 좌측 상하지 운동완만과 실조를 보였고, 좌측 깊은힘줄반사 항진, 호프만과 바뱅스키징후가 양성이었으며, 우측 핵사이안근마비가 확인되었다. 영상 검사에서 뇌간의 동정맥기형과(Fig. 1) 거대 나선형 정맥확장증이(Fig. 2) 확인되었다(Spetzler-Martin grade 4) [1]. 대뇌 동정맥기형은 흔하나 뇌간에 발생하는 경우는 드물며 3 cm 이상은 더욱 드문 것으로 알려져 있다[2]. 서서히 진행하는 신경학적 증상으로 나타난 나선형 거대 정맥확장증이 동반된 동정맥기형의 드문 증례로 이를 보고하는 바이다.

Figure 1.

Magnetic resonance images. (A-D) Axial T2 weighted images showed giant AVM at upper pons and midbrain. (E) Coronal T2 weighted image showed 3.3×3.1 cm sized nidus at midbrain and (F) spiral shaped ectatic vein (superficial middle cerebral vein) compressing internal globus pallidus. AVM; arteriovenous malformation.

Figure 2.

Brain CT angiography. (A) Axial image showed nidus (black arrow) suggests AVM fed by basilar and right posterior cerebral artery. (B) Venous ectasia was from superficial middle cerebral vein (black arrowhead). (C) 3D reconstruction image showed spiral shaped ectatic vein and AVM. CT; computed tomography, AVM; arteriovenous malformation.

References

1. Spetzler RF, Martin NA. A proposed grading system for arteriovenous malformations. J Neurosurg 1986;65:476–483.

2. Madhugiri VS, Teo MKC, Vavao J, Bell-Stephens T, Steinberg GK. Brainstem arteriovenous malformations: lesion characteristics and treatment outcomes. J Neurosurg 2018;128:126–136.

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