Department of Pediatrics, Obstetrics, Gynecology, and Reproductive Medicine, Pediatric Neurology Unit, S. Maria alle Scotte Hospital, University of Siena, 53100 Siena, Italy.
Eur J Paediatr Neurol. 2012 Jul;16(4):390-5. doi: 10.1016/j.ejpn.2011.12.007. Epub 2012 Jan 12.
Status dystonicus (SD) is a medical emergency weighed by a relevant morbidity and mortality. It mainly affects patients with primary or secondary dystonia and is often triggered by events such as fever, infections, exposure medications or their abrupt cessation. We report on three patients presenting with SD. Two of them were affected by a static encephalopathy and the other one by a neurodegenerative disorder such as megalencephalic leukoencephalopathy with subcortical cysts (MLC). To our knowledge this is the first patient affected by MLC presenting with SD. All our patients underwent continuous infusion of midazolam, in association with pimozide and trihexyphenidyl, which led to complete resolution of muscular spasms in two patients. In the other one a complete cessation of dystonic spasms was obtained after intrathecal baclofen. From a therapeutic point of view there are no evidence-based management guidelines in SD. The approach is empiric and based on very limited anecdotal reports. On the basis of our observations and an extensive review of the literature we delineated a possible therapeutic strategy of SD in children.
僵人综合征（Status dystonicus，SD）是一种严重的医学急症，具有较高的发病率和死亡率。它主要影响原发性或继发性肌张力障碍患者，常由发热、感染、药物暴露或突然停药等事件引发。我们报告了 3 例僵人综合征患者。其中 2 例患者患有静止性脑病，另 1 例患者患有神经退行性疾病，如巨脑性白质脑病伴皮质下囊肿（megalencephalic leukoencephalopathy with subcortical cysts，MLC）。据我们所知，这是首例 MLC 患者出现僵人综合征。我们所有的患者均接受咪达唑仑持续输注，并联合匹莫齐特和苯海索治疗，其中 2 例患者的肌肉痉挛完全缓解。另 1 例患者在鞘内注射巴氯芬后完全停止了张力性痉挛。从治疗角度来看，僵人综合征没有基于证据的管理指南。治疗方法是经验性的，并且基于非常有限的个案报告。基于我们的观察和对文献的广泛回顾，我们为儿童僵人综合征制定了一种可能的治疗策略。
