Maestrini Ilaria, Rocchi Lorenzo, Puledda Francesca, Viganò Alessandro, Giuliani Giada, Jannini Tommaso Benedetto, Celletti Claudia, Altieri Marta, Camerota Filippo, Toscano Massimiliano, Di Piero Vittorio
Department of Human Neurosciences, Headache Centre, "Sapienza" University of Rome, Rome, Italy.
Department of Systems Medicine, University of Rome Tor Vergata, Rome, Italy.
Front Neurol. 2023 Mar 9;14:1072785. doi: 10.3389/fneur.2023.1072785. eCollection 2023.
Migraine is one of the most frequent clinical manifestations of hypermobile Ehlers-Danlos syndrome (hEDS). The comorbidity between these two diseases has been only partially investigated. We aimed to observe whether neurophysiological alterations described in migraineurs in visual evoked potentials (VEPs) were present in hEDS patients with migraine.
We enrolled 22 hEDS patients with migraine (hEDS) and 22 non-hEDS patients with migraine (MIG), with and without aura (according to ICHD-3), as well as 22 healthy controls (HC). Repetitive pattern reversal (PR)-VEPs were recorded in basal conditions in all participants. During uninterrupted stimulation, 250 cortical responses were recorded (4,000 Hz sample rate) and divided into epochs of 300 ms after the stimulus. Cerebral responses were divided into five blocks. The habituation was calculated as the slope interpolating the amplitudes in each block, for both the N75-P100 and P100-N145 components of PR-VEP.
We observed a significant habituation deficit of the P100-N145 component of PR-VEP in hEDS compared to HC ( = 0.002), unexpectedly more pronounced than in MIG. We observed only a slight habituation deficit of N75-P100 in hEDS, with a slope degree that was intermediate between MIG and HC.
hEDS patients with migraine presented an interictal habituation deficit of both VEPs components like MIG. Pathophysiological aspects underlying the pathology could account for the peculiar pattern of habituation in hEDS patients with migraine characterized by a pronounced habituation deficit in the P100-N145 component and a less clear-cut habituation deficit in the N75-P100 component with respect to MIG.
偏头痛是活动度过高型埃勒斯-当洛综合征(hEDS)最常见的临床表现之一。这两种疾病的合并症仅得到部分研究。我们旨在观察偏头痛患者视觉诱发电位(VEP)中所描述的神经生理改变是否存在于患有偏头痛的hEDS患者中。
我们纳入了22例患有偏头痛的hEDS患者(hEDS组)和22例患有偏头痛的非hEDS患者(MIG组),有先兆和无先兆的患者均包括在内(根据国际头痛疾病分类第三版[ICHD-3]),以及22名健康对照者(HC组)。在基础状态下记录所有参与者的重复模式翻转(PR)-VEP。在不间断刺激期间,记录250次皮质反应(采样率4000Hz),并在刺激后分为300ms的时间段。脑反应分为五个块。习惯化程度通过内插每个块中PR-VEP的N75-P100和P100-N145成分振幅的斜率来计算。
与HC组相比,我们观察到hEDS组PR-VEP的P100-N145成分存在显著的习惯化缺陷(P = 0.002),出乎意料的是,这种缺陷比MIG组更明显。我们仅观察到hEDS组N75-P100存在轻微的习惯化缺陷,其斜率程度介于MIG组和HC组之间。
患有偏头痛的hEDS患者与MIG组一样,两种VEP成分均存在发作间期习惯化缺陷。该病理背后的病理生理方面可能解释了患有偏头痛的hEDS患者习惯化的特殊模式,其特征在于P100-N145成分存在明显的习惯化缺陷,而N75-P100成分的习惯化缺陷相对于MIG组不那么明确。