Waddell Adam, Dirweesh Ahmed, Ordonez Fausto, Kososky Charles, Reddy Peddareddygari Leema, Grewal Raji P
Neuroscience Institute, Saint Francis Medical Center, Trenton, NJ, USA.
The Neuro-genetics Institute, Sharon Hill, PA, USA.
J Community Hosp Intern Med Perspect. 2017 Jul 13;7(3):182-184. doi: 10.1080/20009666.2017.1340730. eCollection 2017 Jul.
Lance-Adams syndrome (LAS) is an uncommon neurological disorder characterized by the development of chronic post-hypoxic myoclonus. There are relatively few cases described following successful cardiopulmonary resuscitation. We report a patient who developed LAS 3 months after successful resuscitation. Cerebral imaging studies indicate that brain pathology in LAS patients is not uniform, suggesting that the pathophysiology of myoclonus may vary from patient to patient. Our patient adds to this etiological heterogeneity by demonstrating the unusual feature of cerebellar pathology by both cerebral magnetic resonance imaging and single-photon emission computed tomography scans. There is also heterogeneity of these patients in their response to therapy. Unlike some patients described in the literature, our patient has not responded to drug treatment. Studies of these rare patients with LAS are important as they provide insight into the pathophysiology of this condition which, it is hopefully, will facilitate the development of more effective therapy.
兰斯-亚当斯综合征（LAS）是一种罕见的神经系统疾病，其特征为慢性缺氧后肌阵挛的发生。成功进行心肺复苏后描述的病例相对较少。我们报告一例患者，其在成功复苏3个月后出现了LAS。脑成像研究表明，LAS患者的脑部病理并不一致，这表明肌阵挛的病理生理学可能因患者而异。我们的患者通过脑磁共振成像和单光子发射计算机断层扫描显示出小脑病理这一不寻常特征，进一步增加了这种病因的异质性。这些患者对治疗的反应也存在异质性。与文献中描述的一些患者不同，我们的患者对药物治疗没有反应。对这些患有LAS的罕见患者进行研究很重要，因为它们有助于深入了解这种疾病的病理生理学，有望促进更有效治疗方法的开发。
