中华神经外科杂志
中華神經外科雜誌
중화신경외과잡지
Chinese Journal of Neurosurgery
2009年
6期
497-499
,共3页
李春德%罗世祺%马振宇%张玉琪%甲戈
李春德%囉世祺%馬振宇%張玉琪%甲戈
리춘덕%라세기%마진우%장옥기%갑과
下丘脑错构瘤%疾病特征
下丘腦錯構瘤%疾病特徵
하구뇌착구류%질병특정
Hypothalamic hamartoma%Disease attributes
目的 分析下丘脑错构瘤的临床特征.方法 回顾分析1994年1月至2008年5月北京天坛医院诊治的214例下丘脑错构瘤的临床资料.结果 性别:男性多于女性,男女之比为1.52:1;有性早熟的115例中,男女之比为1.09:1;有癫痫的123例中男女之比为2.15:1;有痴笑样癫痫的96例中男女之比为2:1;同时表现为性早熟及癫痫的38例中男女之比为1.38:1.发病年龄:200例有症状的病例,平均发病年龄为34.5个月;发病年龄≤3岁者157例,占78.5%;单纯性早熟者77例(38.5%),平均发病年龄16.5个月,<3岁者67例,占87%;表现有性早熟者115例(57.5%),平均发病年龄为17.63个月,发病<3岁者99例,占86.1%;有痴笑样癫痫者96例(48%),平均为26.14个月,<3岁者有78例,占81.3%;所有癫痫者[痴笑样癫痫和(或)癫痫大、小发作]123例(61.5%),平均发病年龄为3.81岁,≤3岁前发病者有90例,占73.2%.结论 下丘脑错构瘤多数在婴幼儿期发病,男性多于女性.
目的 分析下丘腦錯構瘤的臨床特徵.方法 迴顧分析1994年1月至2008年5月北京天罈醫院診治的214例下丘腦錯構瘤的臨床資料.結果 性彆:男性多于女性,男女之比為1.52:1;有性早熟的115例中,男女之比為1.09:1;有癲癇的123例中男女之比為2.15:1;有癡笑樣癲癇的96例中男女之比為2:1;同時錶現為性早熟及癲癇的38例中男女之比為1.38:1.髮病年齡:200例有癥狀的病例,平均髮病年齡為34.5箇月;髮病年齡≤3歲者157例,佔78.5%;單純性早熟者77例(38.5%),平均髮病年齡16.5箇月,<3歲者67例,佔87%;錶現有性早熟者115例(57.5%),平均髮病年齡為17.63箇月,髮病<3歲者99例,佔86.1%;有癡笑樣癲癇者96例(48%),平均為26.14箇月,<3歲者有78例,佔81.3%;所有癲癇者[癡笑樣癲癇和(或)癲癇大、小髮作]123例(61.5%),平均髮病年齡為3.81歲,≤3歲前髮病者有90例,佔73.2%.結論 下丘腦錯構瘤多數在嬰幼兒期髮病,男性多于女性.
목적 분석하구뇌착구류적림상특정.방법 회고분석1994년1월지2008년5월북경천단의원진치적214례하구뇌착구류적림상자료.결과 성별:남성다우녀성,남녀지비위1.52:1;유성조숙적115례중,남녀지비위1.09:1;유전간적123례중남녀지비위2.15:1;유치소양전간적96례중남녀지비위2:1;동시표현위성조숙급전간적38례중남녀지비위1.38:1.발병년령:200례유증상적병례,평균발병년령위34.5개월;발병년령≤3세자157례,점78.5%;단순성조숙자77례(38.5%),평균발병년령16.5개월,<3세자67례,점87%;표현유성조숙자115례(57.5%),평균발병년령위17.63개월,발병<3세자99례,점86.1%;유치소양전간자96례(48%),평균위26.14개월,<3세자유78례,점81.3%;소유전간자[치소양전간화(혹)전간대、소발작]123례(61.5%),평균발병년령위3.81세,≤3세전발병자유90례,점73.2%.결론 하구뇌착구류다수재영유인기발병,남성다우녀성.
Objective To study the clinical characteristics in hypothalamic hamartoma. Methods A retrospective review of 214 patients with hypothalamic hamartoma was performed between January 1994 and May 2008. Results There were more patients in male and the ratio of male and female was 1.52: 1. The rate of male and female in 115 patients with precocious puberty was 1.09: 1, in 96 patients with gelastic seizures was 2: 1, in 123 patients with epilepsy was 2. 15: 1, in 38 patients with precocious puberty and epilepsy was 1.38: 1. There were 14 asymptomatic patients, the median age at the time of symptomatic was 34. 5 months. In the rest 200 patients with symptomatic, there were 157 patients younger than 3 year old (78.5%) ; the median onset age in 77 patients with simplicity precocious puberty was 16. 5 months, in 115 patients with precocious puberty was 17.63 months, in 96 patients with gelastic seizures was 26. 14 months, in 123 patients with all type epilepsy was 3. 18 yr. Conclusions The onset age of hypothalamic hamartoma is in younger children and more found in male.