Danish Headache Center and Department of Neurology N01, Glostrup Hospital, Faculty of Health Sciences, University of Copenhagen, Nordre Ringvej 57, DK-2600 Glostrup, Copenhagen, Denmark.
Neurology. 2010 Feb 16;74(7):594-600. doi: 10.1212/WNL.0b013e3181cff79d.
In contrast to patients with migraine with aura (MA) and migraine without aura (MO), most patients with familial hemiplegic migraine (FHM) do not report migraine-like attacks after pharmacologic provocation with glyceryl trinitrate (GTN), a donor of nitric oxide. In the present study, we examined patients with FHM without known gene mutations and hypothesized that 1) GTN would cause more migraine-like attacks in patients with FHM compared to controls, and 2) GTN would cause more migraine attacks in patients with FHM with coexisting MA or MO compared to the pure FHM phenotype.
The study design was a balanced provocation study. Twenty-three patients with FHM and 11 healthy controls received a continuous IV infusion of 0.5 mug/kg/min GTN over 20 minutes.
We found no difference in the incidence of migraine-like attacks comparing all patients with FHM (30%) to controls (9%) (p = 0.15). Patients with FHM with coexisting MA or MO reported more migraine attacks after GTN (55%) than patients with the pure FHM phenotype (8.3%) (p = 0.02). Compared to healthy controls, more patients with FHM with coexisting MA or MO reported migraine-like attacks than controls (p = 0.03), whereas the FHM group with the pure FHM phenotype did not (p > 0.05).
Compared to patients with migraine with aura (MA) and migraine without aura (MO), patients with familial hemiplegic migraine (FHM) without known gene mutations display a reduced sensitivity to nitric oxide. A subset of patients with FHM with coexisting nonhemiplegic migraine is more sensitive than controls. These data extend our previous findings that pathophysiologic pathways in FHM may differ from those of MO and MA.
与有先兆偏头痛（MA）和无先兆偏头痛（MO）患者相比，大多数家族性偏瘫性偏头痛（FHM）患者在用一氧化氮供体硝酸甘油（GTN）进行药物激发后不会出现类似偏头痛的发作。在本研究中，我们检查了无已知基因突变的 FHM 患者，并假设 1）与对照组相比，GTN 会在 FHM 患者中引起更多类似偏头痛的发作，2）与纯 FHM 表型相比，GTN 会在同时存在 MA 或 MO 的 FHM 患者中引起更多偏头痛发作。
研究设计为平衡激发研究。23 例 FHM 患者和 11 例健康对照者接受 0.5μg/kg/min GTN 持续 20 分钟静脉输注。
我们发现，将所有 FHM 患者（30%）与对照组（9%）相比，类似偏头痛发作的发生率无差异（p=0.15）。同时存在 MA 或 MO 的 FHM 患者在 GTN 后报告的偏头痛发作多于纯 FHM 表型患者（55%比 8.3%，p=0.02）。与健康对照组相比，同时存在 MA 或 MO 的 FHM 患者比对照组报告的类似偏头痛发作更多（p=0.03），而纯 FHM 表型的 FHM 组则没有（p＞0.05）。
与有先兆偏头痛（MA）和无先兆偏头痛（MO）患者相比，无已知基因突变的家族性偏瘫性偏头痛（FHM）患者对一氧化氮的敏感性降低。FHM 中存在非偏瘫性偏头痛的患者亚组比对照组更敏感。这些数据扩展了我们之前的发现，即 FHM 的病理生理途径可能与 MO 和 MA 不同。
