Department of Pediatrics, University of Padua, Padua, Italy.
Cephalalgia. 2011 Apr;31(6):751-6. doi: 10.1177/0333102410392068. Epub 2010 Dec 20.
Hemiplegic migraine (HM) is a rare variety of migraine with aura, characterized by motor deficits during the aura, often beginning in childhood. The hemiplegic attacks can be severe and prolonged but the prognosis is usually good. Data on neuroimaging, including diffusion-weighted imaging (DWI) and spectroscopy, during prolonged attacks of HM are quite limited, particularly in children.
An eight-year-old female had a prolonged attack of sporadic HM characterized by right-sided hemiplegia, global aphasia, fever and impairment of consciousness. MRI nine hours after hemiplegia onset was negative, while the following MRI scans (days 4 and 11) documented a progressive increase in cortical swelling in the left hemisphere with mild hyperintensity on DWI and mild reduction of apparent diffusion coefficient values. Proton MRI spectroscopy (MRS) (day 15) showed a decrease in the N-acetylaspartate/creatine ratio in the left hemisphere. (99m)Tc-ECD single-photon emission tomography (SPET) (day 27) showed marked left hemispheric hypoperfusion. The patient recovered completely after 40 days and neuroimaging follow-up (MRI and SPET) after six months was normal. The patient carried a missense mutation of the ATP1A2 gene.
Multimodal neuroimaging (MRI, DWI, MRS, SPET) in a prolonged HM attack supports evidence for a primary neuronal dysfunction.
偏瘫性偏头痛（HM）是一种罕见的有先兆偏头痛，其特点是先兆期出现运动障碍，通常始于儿童期。偏瘫发作可能很严重且持续时间长，但预后通常良好。关于 HM 长时间发作期间的神经影像学，包括弥散加权成像（DWI）和光谱，数据非常有限，尤其是在儿童中。
一名 8 岁女性患有散发性 HM 长时间发作，表现为右侧偏瘫、全身失语、发热和意识障碍。偏瘫发作后 9 小时的 MRI 为阴性，而随后的 MRI 扫描（第 4 天和第 11 天）记录了左侧半球皮质肿胀逐渐增加，DWI 上呈轻度高信号，表观弥散系数值轻度降低。质子 MRI 光谱（MRS）（第 15 天）显示左侧半球 N-乙酰天冬氨酸/肌酸比值降低。（99m）Tc-ECD 单光子发射断层扫描（SPET）（第 27 天）显示左侧半球明显低灌注。患者在 40 天后完全恢复，6 个月后的神经影像学随访（MRI 和 SPET）正常。患者携带 ATP1A2 基因突变。
长时间 HM 发作的多模态神经影像学（MRI、DWI、MRS、SPET）支持原发性神经元功能障碍的证据。
