Department of Neurology, MedStar Georgetown University Hospital, 3800 Reservoir Road NW, 7PHC, Washington, DC, 20007, USA.
Curr Neurol Neurosci Rep. 2023 Jul;23(7):381-387. doi: 10.1007/s11910-023-01277-z. Epub 2023 May 29.
In this article, we review recent updates to the epidemiology, diagnostic testing, genetics, pathophysiology, and management of hemiplegic migraine.
While three genes have been historically associated with hemiplegic migraine, recent studies suggest two additional genes may also be implicated including PPRT2 and SLC1A3. Hemiplegic migraine is a severe subset of migraine with aura with symptoms including reversible hemiparesis in addition to other aura symptoms such as visual, sensory, or speech. The exact pathophysiology of hemiplegic migraine is not clear, but it is thought that this phenomenon is due to neuronal and glial depolarization causing cortical spreading depression. Due to the severity of presentation as well as the numerous mimickers, it is important to know a comprehensive differential and work-up. Given the low prevalence of the disease, most studies regarding treatment are limited to case studies. There is still an important need for further and larger studies regarding management of these cases.
本文综述了偏瘫性偏头痛的流行病学、诊断检测、遗传学、病理生理学和治疗的最新进展。
虽然有三个基因与偏瘫性偏头痛有关，但最近的研究表明，另外两个基因（PPRT2 和 SLC1A3）也可能与偏瘫性偏头痛有关。偏瘫性偏头痛是偏头痛伴先兆的严重亚型，除其他先兆症状（如视觉、感觉或言语障碍）外，还伴有可逆性偏瘫。偏瘫性偏头痛的确切病理生理学尚不清楚，但据认为，这种现象是由于神经元和神经胶质去极化导致皮质扩散性抑制。由于临床表现严重，且有许多类似疾病，因此全面了解鉴别诊断和检查非常重要。由于该疾病的发病率较低，大多数关于治疗的研究都限于病例研究。对于这些病例的治疗，仍然需要进一步进行更多的大型研究。
