214例下丘脑错构瘤分型与临床症状  

Classification of hypothalamic hamartoma according to the clinical manifestations of 214 cases

作　　者：罗世琪[1] 李春德[1] 马振宇[1] 张玉琪[1] 甲戈[1] 

机构地区：[1]首都医科大学附属北京天坛医院神经外科,北京100050

出　　处：《中华神经外科杂志》2009年第9期788-792,共5页Chinese Journal of Neurosurgery

摘　　要：目的研究下丘脑错构瘤的临床特征与形态关系。方法回顾分析214例下丘脑错构瘤的临床资料，采用SPSS13．0对资料进行统计学处理并提出新的分型。结果将下丘脑错构瘤分为Ⅰ-Ⅳ型：Ⅰ型错构瘤与下丘脑附着面小；Ⅱ型错构瘤与下丘脑的附着面宽大，此两型错构瘤均位于脚间池内；Ⅲ型为错构瘤骑跨于第三脑室底；Ⅳ型则错构瘤完全位于第三脑室内。本组Ⅰ型77例（36．0％），Ⅱ型26例（12．1％），Ⅲ型87例（40．7％），Ⅳ型24例（11．2％）；性早熟的发病年龄明显小于癫痫者的发病年龄；错构瘤最大径的大小：Ⅰ型：13．57mm（3～30mm）；Ⅱ型18．73mm（8～38mm）；Ⅲ型：22．08mm（9～70mm）；Ⅳ型：11．21mm（8～16mm）。最大径：Ⅲ型、Ⅱ型〉Ⅰ型〉Ⅳ型，表现有癫痫的错构瘤最大径大于无癫痫的错构瘤；性早熟在Ⅰ～Ⅳ各型中的比例分别为54．8％、10．4％、33．0％及1．7％，性早熟多见于Ⅰ型，而罕见于Ⅳ型；痴笑样癫痫在Ⅰ～Ⅳ各型中的比例分别为3．1％、7．3％、66．7％及22．9％，痴笑样癫痫和癫痫主要见于Ⅲ型，而Ⅰ型罕见；无症状者（14例）在Ⅰ～Ⅳ各型中比例分别为50％、35．7％、7．1％及7．1％，主要见于Ⅰ型和Ⅱ型。结论下丘脑错构瘤的临床表现与其形态关系密切，新的Ⅰ～Ⅳ分型较好体现了这种关系。Objective To study the relationship between the topology and clinical manifestations of hypothalamic hamartoma（HH）. Method A retrospective review of 214 patients with HH was performed between August 1994 and May 2008. Statistical analysis were performed using SPSS version 13 and statistical significance was judged to be present at probability values of less than 0. 05. A new classification of HH was developed based on topographical and clinical data. Results The HH were diagnosed and divided into four categories（type Ⅰ - Ⅳ ） based on MR imaging findings focused on the relationship between HH and hypothalamus or the third ventricle. Type Ⅰ and type Ⅱ defined as HH attached to the floor of third ventricle with a narrow（ type Ⅰ ） or broad （type Ⅱ ） interfaces, type Ⅲ （straddling type） defined as the hamartoma located partially within the hypothalanms and the third ventricle. Type Ⅳ as the HH located totally within the third ventricle. There were 77（36.0% ） cases of HH belonging to the type Ⅰ ,26（ 12. 1% ） cases to the type Ⅱ ,87（40. 7% ） cases to the type Ⅲ and 24 （11.2%） cases to the typeⅣ. The mean age at onset of precocious puberty（PP） was significantly younger than that of epilepsy＇s. The maximum diameter of HH in type Ⅰ is 13.57 mm,in type Ⅱ is 18.73 mm,in type Ⅲ is 22.08 mm and 11.21 mm in type Ⅳ.The diameter of HH presented with epilepsy is larger than that of HH without epilepsy. The percentage of PP in type Ⅰ to Ⅳ is 54. 8% ,10. 4% ,33.0% and 1.7% ,respectively,and PP was mostly common in type Ⅰ and rare in type Ⅳ. The percentage of gelastic seizure in type Ⅰ to Ⅳ is 3. 1% ,7. 3% ,66. 7% and 22. 9%, respectively. Gelastic seizures and epilepsy are mostly presented in type Ⅲ and rarely in type Ⅰ. The percentage of asymptomatic HH（ 14 cases） in the four type is varied as 50% ,35.7% ,7. 1% and 7. 1%. Conclusions The clinical manifestation is correlated with the topology of HH in relation to the hypothalamus and this c

关 键 词：下丘脑 错构瘤 分型 临床症状 

分 类 号：R686[医药卫生—骨科学]