Robertson W C, Clark D B, Markesbery W R
Ann Neurol. 1980 Oct;8(4):422-5. doi: 10.1002/ana.410080414.
Thirty-eight cases of subacute sclerosing panencephalitis (SSPE) were reviewed. Deterioration in school performance, personality changes, and seizures were common early symptoms. Initial examination frequently showed myoclonus, spasticity, and extrapyramidal dysfunction, and in two-thirds of patients these findings were asymmetrical or focal. Retinitis or papilledema was present on initial examination in 50% of the patients. At last follow-up 24 children had died, with a mean survival of 42 months. Most patients reached a state of severe neurological impairment within 13 months. Subsequent evidence of improvement was noted in 10 children and was sustained in 4. Fifteen patients received antiviral treatment. Ten treated patients died from 5 to 133 months (mean, 58) from onset of their illness, while 15 untreated patients survived a mean of 33 months. Duration of survival appeared to be affected most by treatment with amantadine. Three patients treated with the drug were alive 97 to 139 months after onset of SSPE, and 5 died with a mean survival of 78 months. Five of 6 individuals treated with rifampin died after a mean survival of 27 months. Prolonged remissions occurred only in patients treated with amantadine. Although the number of treated individuals was small, our data suggest that amantadine may affect the natural course of SSPE.
对38例亚急性硬化性全脑炎（SSPE）患者进行了回顾性研究。学业成绩下降、性格改变和癫痫发作是常见的早期症状。初始检查常显示肌阵挛、痉挛和锥体外系功能障碍，三分之二的患者这些表现为不对称或局灶性。50%的患者初始检查时有视网膜炎或视乳头水肿。在最后一次随访时，24名儿童死亡，平均生存期为42个月。大多数患者在13个月内达到严重神经功能损害状态。10名儿童随后出现病情改善的迹象，其中4名病情持续改善。15名患者接受了抗病毒治疗。10名接受治疗的患者在发病后5至133个月（平均58个月）死亡，而15名未接受治疗的患者平均存活33个月。生存期似乎受金刚烷胺治疗的影响最大。3名接受该药物治疗的患者在SSPE发病后97至139个月仍存活，5名患者死亡，平均生存期为78个月。6名接受利福平治疗的患者中有5名在平均存活27个月后死亡。仅在接受金刚烷胺治疗的患者中出现了长期缓解。尽管接受治疗的个体数量较少，但我们的数据表明金刚烷胺可能会影响SSPE的自然病程。
