Park Young Seok, Oh Min-Chul, Kim Heung Dong, Kim Dong-Seok
Department of Neurosurgery, Severance Children's Hospital, Brain Korea 21 Project for Medical Science, Brain Research Institute, Yonsei University College of Medicine, Seoul, Republic of Korea.
Brain Dev. 2009 May;31(5):347-51. doi: 10.1016/j.braindev.2008.03.013. Epub 2008 May 15.
Epilepsy from hamartoma of the floor of fourth ventricle (HFFV) is very rare, starting in neonate or infantile period, good response to surgery. We report a 3-month-old boy with episodes of medically intractable abnormal eyelid blinking and hemifacial seizure. The episodes began from the first day of life and were unresponsive to medication. A magnetic resonance imaging scan revealed mass lesion on the floor of fourth ventricle, with extended cerebellar peduncle and cerebellar hemisphere. Surgery had been performed two times previously for treatment; only a subtotal resection was performed due to severe bradycardia during the first operation. The patient underwent second operation for gross total removal of tumor. Complete resection of the mass after second surgery resulted in remission of seizures and histopathology revealed hamartoma with hemangiomatous vessel proliferation. Identification of characteristic semiology and associated HFFV can help prediction of intractability even in infant and favoring surgical treatment in early age. To our knowledge, this is the youngest patient who underwent early surgery just after progressing into status for HFFV, showing complete lesion resection resulting in successful seizure outcome.
起源于第四脑室底部错构瘤(HFFV)的癫痫非常罕见,始于新生儿期或婴儿期,手术效果良好。我们报告一名3个月大男童,有药物难治性异常眼睑眨眼和半侧面部癫痫发作。发作自出生第一天起,对药物无反应。磁共振成像扫描显示第四脑室底部有肿块病变,小脑脚和小脑半球受累。此前已进行过两次手术治疗;第一次手术时因严重心动过缓仅行次全切除。患者接受了第二次手术以彻底切除肿瘤。第二次手术后肿块完全切除,癫痫发作缓解,组织病理学显示为错构瘤伴血管瘤样血管增生。识别特征性的症状学及相关HFFV有助于预测即使在婴儿期的难治性癫痫,并支持早期手术治疗。据我们所知,这是在进展为HFFV状态后不久即接受早期手术的最年轻患者,显示病变完全切除,癫痫发作结果良好。