Fernández Suárez N, Felgueroso Juliana B, Castro Aguiar S
Servicio de Pediatría, CMI Teresa Herrera, Centro Hospitalario Universitario, A Coruña, España.
An Pediatr (Barc). 2012 Nov;77(5):334-8. doi: 10.1016/j.anpedi.2012.04.009. Epub 2012 Jun 17.
Continuous partial epilepsy is a form of partial status epilepticus, which is characterized by the presence of repeated myoclonus affecting a muscle group. Its origin is cortical and it can last for hours, days, weeks and exceptionally, years. Within these forms of epilepsy we can distinguish two groups: the first group or Kojewnikow classic syndrome includes children with a known lesion in the rolandic region (the etiology is also known) and there is a stable neurological damage (unless the injury increases, e.g., tumors). This disease is characterized by the presence of motor partial seizures, sometimes they are followed by periods of well-localized myoclonus. The second group or Rasmussen syndrome is characterized by onset of seizures in previously healthy patients, starting with partial motor seizures, that later can be combined with myoclonus that affect different areas of the body. It is a progressive disease that leads to neurological damage. A case is presented of a 7-year-old patient investigated due to having partial seizures and progressive neurological degeneration. After performing imaging studies, neuropsychological studies, and laboratory tests, he was diagnosed with Rasmussen's syndrome. Finally, a palliative hemispherectomy was performed and the diagnosis was confirmed by a biopsy.
连续性部分性癫痫是部分性癫痫持续状态的一种形式，其特征为反复出现影响某一肌群的肌阵挛。其起源于皮质，可持续数小时、数天、数周，极少数情况下可持续数年。在这些癫痫形式中，我们可区分出两组：第一组或科热夫尼科夫经典综合征，包括在中央前回区域有已知病变的儿童（病因也已知），且存在稳定的神经损伤（除非损伤加重，如肿瘤）。这种疾病的特征是存在运动性部分性癫痫发作，有时随后会出现局限性肌阵挛发作期。第二组或拉斯穆森综合征的特征是在先前健康的患者中发作，起始为部分性运动性癫痫发作，随后可合并影响身体不同部位的肌阵挛。这是一种导致神经损伤的进行性疾病。本文报告了一例7岁患者，因出现部分性癫痫发作和进行性神经退行性变而接受检查。在进行影像学检查、神经心理学研究和实验室检查后，他被诊断为拉斯穆森综合征。最后，实施了姑息性大脑半球切除术，并通过活检确诊。
