- 作者：C. Castañ ; o De La Mota ; ccastano993@yahoo.es ; F. Martí ; n Del Valle ; A. Pé ; rez Villena ; M.L. Calleja Gero ; R. Losada Del Pozo ; M.L. Ruiz-Falcó ; Rojas
- 关键词：Cirugí ; a de resecció ; n tumoral ; Crisis gelá ; sticas ; Dificultades cognitivas ; Pubertad precoz ; Trastornos de conducta
- 刊名：Neurolog篓陋a
- 出版年：2012
- 期刊代码：pca262_02134853
- 类别：med
- 出版时间：June, 2012
- 卷：27
- 期：5
- 页码：268-276
- 文件大小：381 K
Objective
To describe the epidemiological and clinical-electroencephalographic characteristics, and associated morbidity of patients with hypothalamic hamartoma, as well as the treatment followed and outcomes
Patients and methods
We have retrospectively reviewed the medical histories of 10 patients diagnosed with hypothalamic hamartoma by magnetic resonance imaging over the last 20 years.
Results
The age of onset of epilepsy in patients with hypothalamic hamartoma in our series was between the first days of life and 2 years. Of the 10 total patients, 8 had epileptic seizures during its progress. All of them had gelastic seizures, in addition to other types of seizures, with the most common being partial simple seizures. The electroencephalographic findings recorded were highly variable. One of the patients developed epileptic encephalopathy. Five patients had some kind of conduct disorder. Five patients had cognitive problems. At least 2 different antiepileptic drugs were measured in 8 of the patients who had seizures, and in 6 of these some type of non-pharmacological treatment had been used with the objective of seizure control. Only in 3 of 8 patients has been achieved Acceptable control of epilepsy had only been achieved in 3 out the 8 patients. Five patients of the series developed precocious puberty. The average time of follow-up of the series was approximately 6 years.
Conclusions
Epilepsy is the most frequent manifestation of hypothalamic hamartomas. Most cases were drug-resistant, which led to difficulties in the management of these patients, requiring surgery for their control on many occasions. Psychiatric comorbidity and cognitive impairment is common.