目的 全面介绍先天性囊性腺瘤样畸形(CCAM)其可能的病因，临床、病理特点及诊断手段，循证探讨针对CCAM的治疗方法及预后。 方法 对我院2011年11月收治的1例罕见的CCAM患者的临床资料进行分析，并对相关文献进行复习。 结果 患者数次误诊后最终诊断为CCAM，予手术治疗后痊愈，随访1年无复发。 结论 CCAM是一种少见的、非遗传性的、错构瘤样的肺发育异常，为一种良性的肺部畸形，其特点是局部肺终末呼吸性细支气管过度生长。CCAM多通过产前影像学检查、活组织检查或术后病检诊断。手术为治愈该病的最根本、最重要措施。
ObjectiveTo explore the clinical features and EEG features of gelastic seizures, and analyze its value of lateral localization of epileptogenic area. MethodsAll patients with gelastic seizures admitted to the Sanbo Brain Hospital of Capital Medical University between January 2014 and December 2023 were reviewed and analyzed for history, symptomatology, imaging, electroencephalographic features and surgical protocols in patients who met the inclusion criteria and were followed up for at least 1 year, and surgical efficacy was assessed by using the Engel grading. ResultsA total of 51 patients with gelastic seizures were included, there were 32 (62.75%) males and 19 (37.25%) females, 21 (41.18%) with hypothalamic hamartomas (HH) and 30 (58.82%) with non-hypothalamic hamartomas. The age of onset was earlier in the HH group than in the non-HH group, with a median age of onset of 24.00 (0.00 ~ 96.00) and 78.00 (1.00 ~ 396.00) months (P<0.001). There are three types of laughter according to their characteristics: smiling or pleasant expressions, laughing out loud, crying or bitter laughter, with smiling or pleasant expressions being the most common (49.02%). Simple laughter is rare in all patients and is often accompanied by other manifestations such as autonomic symptoms, automatic movements, complex movements, and tonic seizures. Most of the HH group started with laughter whereas in the non-HH group laughter appeared mostly in the mid to late stages (P=0.007). Most of the HH group (57.14%) had preserved consciousness whereas most of the non-HH group (83.33%) had loss of consciousness (P=0.003). The interictal discharges in the HH group were mostly diffuse or multiregional, whereas those in the non-HH group were mostly regional (P=0.035). The onset of EEG during the seizure period in the HH group was mostly diffuse, whereas those in the non-HH group were mostly regional, mainly in the frontal and temporal regions, but there was no significant difference between the two groups (P=0.148). The non-HH group was mostly seen in those with definite lesions, and the most common type of lesion was FCD (focal cortical dysplasia, FCD). All patients enrolled in the group underwent surgical treatment, and stereoelectroencephalogram (SEEG) electrode implantation was performed in 13 cases in the HH group and in 17 cases in the non-HH group. 61.90% of the patients in the HH group had an Engel grade I, and 73.33% of the patients in the non-HH group had an Engel grade I. ConclusionsGelastic seizures has a complex neural network, with common causes other than hypothalamic hamartomas, and is most commonly seen in frontal or temporal lobe epilepsy, as well as in the insula or parietal lobe, with the most common type of lesion being FCD. The symptomatology, stage of onset, and electroencephalographic features of gelastic seizures can help in the differential diagnosis, and SEEG can help define the origin of the seizure and its diffusion pathway. The overall prognosis of surgical treatment was better in both the hypothalamic hamartomas and non-hypothalamic hamartomas groups.