Abstract：
BACKGROUND: Dandy-Walker syndrome (DWS) is a group of brain malformations which occasionally accompanied by psychotic symptoms. The co-occurrence of DWS and epilepsy in children is quite rare.
METHODS: We reported a 14-year-old male who presented with a 8-month history of inconsistent upper limb tremor and accidental seizure. The MRI showed the typical alterations of DWS: cystic dilatation of the fourth ventricle, vermian hypoplasia, enlarged posterior fossa. He received the ventriculoperitoneal shunting (VPS) placement for hydrocephalus and had a symptom-free period for 8 days. Then he experienced a recurrence of involuntary upper limb tremor and behavior disturbance after decreasing the pressure of cerebrospinal fluid (CSF) from 150 to 130 mm Hg. After being treated with Olanzapine 10 mg/d, Clonazepam 3 mg/qn and Valproate acid (VPA) 500 mg/bid for nearly a month, his mental status and psychotic symptoms fluctuated. A search of Pub Med showed little report of hydrocephalus and DWS comorbidity with seizure and psychosis. Here we presented the whole process of a rare disease from the very beginning with all his symptoms, examinations and treatments.
CONCLUSIONS: VPS placement surgery at an earlier stage may be an effective way to avoid inevitable brain damage so as to improve the clinical outcomes for patients with DWS. Continued treatment with regard to DWS condition may include shunt placement, but it mainly focus on developmental concerns, with occupational and physical therapy along with ongoing supportive psychotherapy to improve the coping skills and quality of life.
METHODS: We reported a 14-year-old male who presented with a 8-month history of inconsistent upper limb tremor and accidental seizure. The MRI showed the typical alterations of DWS: cystic dilatation of the fourth ventricle, vermian hypoplasia, enlarged posterior fossa. He received the ventriculoperitoneal shunting (VPS) placement for hydrocephalus and had a symptom-free period for 8 days. Then he experienced a recurrence of involuntary upper limb tremor and behavior disturbance after decreasing the pressure of cerebrospinal fluid (CSF) from 150 to 130 mm Hg. After being treated with Olanzapine 10 mg/d, Clonazepam 3 mg/qn and Valproate acid (VPA) 500 mg/bid for nearly a month, his mental status and psychotic symptoms fluctuated. A search of Pub Med showed little report of hydrocephalus and DWS comorbidity with seizure and psychosis. Here we presented the whole process of a rare disease from the very beginning with all his symptoms, examinations and treatments.
CONCLUSIONS: VPS placement surgery at an earlier stage may be an effective way to avoid inevitable brain damage so as to improve the clinical outcomes for patients with DWS. Continued treatment with regard to DWS condition may include shunt placement, but it mainly focus on developmental concerns, with occupational and physical therapy along with ongoing supportive psychotherapy to improve the coping skills and quality of life.
摘要：
背景：Dandy-Walker综合征（DWS）是一组大脑畸形，偶尔伴有精神病症状。儿童DWS和癫痫的同时发生非常罕见。
方法：我们报道了一名14岁的男性，他有8个月的上肢震颤和意外发作史。MRI显示DWS的典型改变：第四脑室囊性扩张，Vermian发育不全,后颅窝扩大。他因脑积水接受了脑室腹膜分流术（VPS）放置，无症状期8天。然后，在将脑脊液（CSF）的压力从150mmHg降低到130mmHg后，他经历了不自主的上肢震颤和行为障碍的复发。奥氮平10mg/d治疗后，氯硝西泮3mg/qn和丙戊酸（VPA）500mg/bid，持续近一个月，他的精神状态和精神病症状波动。对PubMed的搜索显示，很少有关于脑积水和DWS合并症并伴有癫痫和精神病的报道。在这里，我们从一开始就介绍了一种罕见疾病的整个过程，包括他的所有症状，检查和治疗。
结论：早期行VPS放置手术可能是避免DWS患者不可避免的脑损伤的有效方法，从而改善临床预后。关于DWS条件的继续治疗可能包括分流放置,但它主要关注发展问题，职业和物理治疗以及持续的支持性心理治疗，以提高应对技巧和生活质量。
方法：我们报道了一名14岁的男性，他有8个月的上肢震颤和意外发作史。MRI显示DWS的典型改变：第四脑室囊性扩张，Vermian发育不全,后颅窝扩大。他因脑积水接受了脑室腹膜分流术（VPS）放置，无症状期8天。然后，在将脑脊液（CSF）的压力从150mmHg降低到130mmHg后，他经历了不自主的上肢震颤和行为障碍的复发。奥氮平10mg/d治疗后，氯硝西泮3mg/qn和丙戊酸（VPA）500mg/bid，持续近一个月，他的精神状态和精神病症状波动。对PubMed的搜索显示，很少有关于脑积水和DWS合并症并伴有癫痫和精神病的报道。在这里，我们从一开始就介绍了一种罕见疾病的整个过程，包括他的所有症状，检查和治疗。
结论：早期行VPS放置手术可能是避免DWS患者不可避免的脑损伤的有效方法，从而改善临床预后。关于DWS条件的继续治疗可能包括分流放置,但它主要关注发展问题，职业和物理治疗以及持续的支持性心理治疗，以提高应对技巧和生活质量。
