Gholipour Taha, Mitchell Sara, Sarkis Rani A, Chemali Zeina
Department of Neurology, Edward B. Bromfield Epilepsy Center, Brigham and Women's Hospital, Boston, 75 Francis Street, Boston, MA 02115, USA; Department of Neurology, Massachusetts General Hospital, Boston, 55 Fruit Street, Boston, MA 02114, USA.
Department of Psychiatry, Massachusetts General Hospital, Boston, 55 Fruit Street, Boston, MA 02114, USA.
Epilepsy Behav. 2017 Mar;68:11-16. doi: 10.1016/j.yebeh.2016.12.014. Epub 2017 Feb 10.
Adult patients with Down syndrome (DS) are at higher risk of developing Alzheimer-type dementia and epilepsy. The relationship between developing dementia and the risk of developing seizures in DS is poorly characterized to date. In addition, treatment response and medication tolerability have not been rigorously studied.
We identified 220 patients with a diagnosis of DS and dementia. Those without a history of developing seizures (DD) were compared to patients with new-onset seizures (DD+S) after the age of 35. Electronic records were reviewed for demographics, seizure characteristics, cognitive status, and psychiatric comorbidities.
Of the patients included for analysis, twenty-six out of 60 patients had new-onset seizures or developed seizures during the follow-up period (the DD+S group) with a median onset of 2.0years after the dementia diagnosis. Generalized tonic-clonic seizures were the most common seizure type (61.5% of DD+S). Sixteen (61.5%) patients were reported to have myoclonus. Levetiracetam was the most commonly used initial medication, with the majority (73%) of patients treated achieving partial or complete seizure control. The DD+S patients tended to have a similar burden of new-onset neuropsychiatric symptoms compared to the DD group.
New-onset epilepsy seems to occur early in the course of dementia in DS patients. Patients generally respond to treatment. A great burden of neuropsychiatric symptoms is seen. Future studies need to explore the relationship between β-amyloid accumulation and epileptiform activity and attend to the care and needs of DS patients with dementia and seizures.
成年唐氏综合征（DS）患者患阿尔茨海默型痴呆和癫痫的风险更高。迄今为止，DS患者患痴呆与癫痫发作风险之间的关系尚未得到充分描述。此外，治疗反应和药物耐受性也未得到严格研究。
我们确定了220例诊断为DS和痴呆的患者。将35岁后无癫痫发作史的患者（DD）与新发癫痫患者（DD+S）进行比较。查阅电子记录以获取人口统计学、癫痫发作特征、认知状态和精神共病情况。
在纳入分析的患者中，60例患者中有26例在随访期间出现新发癫痫或癫痫发作（DD+S组），痴呆诊断后癫痫发作的中位发病时间为2.0年。全身强直阵挛性发作是最常见的癫痫发作类型（DD+S组的61.5%）。据报告，16例（61.5%）患者有肌阵挛。左乙拉西坦是最常用的初始药物，大多数接受治疗的患者（73%）实现了部分或完全癫痫控制。与DD组相比，DD+S患者新发神经精神症状的负担往往相似。
新发癫痫似乎在DS患者痴呆病程的早期出现。患者通常对治疗有反应。可见神经精神症状负担较重。未来的研究需要探索β-淀粉样蛋白积累与癫痫样活动之间的关系，并关注患有痴呆和癫痫的DS患者的护理和需求。
