Child Neurology Division, Department of Pediatrics, La Sapienza-University of Rome, Viale Regina Elena, 324 00161 Rome, Italy.
Eur J Pediatr. 2010 Aug;169(8):919-24. doi: 10.1007/s00431-010-1148-0. Epub 2010 Feb 23.
Rasmussen's syndrome (RS) is a rare acquired progressive inflammatory encephalopathy characterized by drug-resistant partial seizures and cognitive deterioration resulting from a gradual impairment and a subsequent atrophy of a single brain hemisphere. It was firstly described by Theodore Rasmussen in 1958. The original etiopathogenic hypothesis of a chronic viral infection no longer holds. Today, the presumed mechanism is a complex autoimmune process comprising an active role of both cytotoxic T lymphocytes and circulating autoantibodies, activating the subunit 3 of the ionotropic glutamate receptor (GluR3Ab). Several medical options, such as antiepileptic drugs and immunomodulatory therapies, deserve consideration to face epileptic manifestations and to slow neurologic regression. Yet, all these therapies turn out to be almost always just temporizing measures. Surgical disconnection of the affected hemisphere ("functional hemispherectomy") is the best therapeutic choice to achieve the arrest of the disease, a good seizure control, the block of neurologic decline, and the improvement of patient's quality of life.
拉森姆森综合征（RS）是一种罕见的获得性进行性炎症性脑病，其特征为耐药性部分性癫痫发作和认知功能恶化，这是由单一脑半球逐渐受损和随后的萎缩引起的。它于 1958 年由 Theodore Rasmussen 首次描述。慢性病毒感染的原始发病假说已不再成立。如今，推测的发病机制是一种复杂的自身免疫过程，包括细胞毒性 T 淋巴细胞和循环自身抗体的积极作用，激活离子型谷氨酸受体（GluR3Ab）亚单位 3。有几种医学选择，如抗癫痫药物和免疫调节疗法，值得考虑以应对癫痫发作和减缓神经退行性变。然而，所有这些治疗方法最终几乎都只是权宜之计。受累半球的手术切断（“功能性半球切除术”）是实现疾病停止、良好的癫痫控制、阻止神经退行性变和改善患者生活质量的最佳治疗选择。
