Bien Christian G, Schramm Johannes
University of Bonn, Department of Epileptology, Sigmund-Freud-Str. 25, 53105 Bonn, Germany.
Epilepsy Res. 2009 Oct;86(2-3):101-12. doi: 10.1016/j.eplepsyres.2009.06.001. Epub 2009 Jul 16.
Rasmussen encephalitis (RE), initially described half a century ago, is an inflammatory unihemispheric brain disorder. Its two clinical key facets are the progressive tissue and function loss and the epilepsy, often in form of epilepsia partialis continua. For both, treatment options are available. Anti-seizure effect of anti-epilepsy drugs is usually limited to secondarily generalized seizures and complex partial seizures whereas epilepsia partialis continua usually is totally refractory. Hemispherectomy in one of its modern variants offers a very high chance of seizure freedom, however at the price of irreversible loss of functions located in the affected hemisphere. In a proportion of patients, long-term immunotherapy is able to prevent or slow down hemispheric tissue loss and the associated functional decline. It does, however, mostly not improve the epilepsy. Whereas for many patients unequivocal treatment proposals can be readily made, a dilemma may emerge in those with severe epilepsy but still preserved hemispheric function.
拉斯穆森脑炎(RE)于半个世纪前首次被描述,是一种炎症性单半球脑疾病。其两个临床关键方面是进行性组织和功能丧失以及癫痫,通常表现为持续性部分性癫痫。对于这两者,都有治疗选择。抗癫痫药物的抗癫痫作用通常仅限于继发性全身性癫痫和复杂部分性癫痫,而持续性部分性癫痫通常完全难治。现代变体之一的半球切除术提供了很高的无癫痫发作几率,然而代价是受影响半球的功能不可逆转地丧失。在一部分患者中,长期免疫治疗能够预防或减缓半球组织丧失及相关功能衰退。然而,它大多不能改善癫痫。虽然对于许多患者可以很容易地提出明确的治疗建议,但对于那些患有严重癫痫但半球功能仍保留的患者可能会出现两难局面。