Hidalgo de la Cruz M, Domínguez Rubio R, Luque Buzo E, Díaz Otero F, Vázquez Alén P, Orcajo Rincón J, Prieto Montalvo J, Contreras Chicote A, Grandas Pérez F
Servicio de Neurología, Hospital General Universitario Gregorio Marañón, Madrid, España.
Servicio de Neurología, Hospital General Universitario Gregorio Marañón, Madrid, España.
Neurologia (Engl Ed). 2019 Oct;34(8):536-542. doi: 10.1016/j.nrl.2017.02.008. Epub 2017 Apr 17.
HaNDL syndrome (transient headache and neurological deficits with cerebrospinal fluid lymphocytosis) is characterised by one or more episodes of headache and transient neurological deficits associated with cerebrospinal fluid lymphocytosis. To date, few cases of HaNDL manifesting with confusional symptoms have been described. Likewise, very few patients with HaNDL and confusional symptoms have been evaluated with transcranial Doppler ultrasound (TCD). TCD data from patients with focal involvement reveal changes consistent with vasomotor alterations.
We present the case of a 42-year-old man who experienced headache and confusional symptoms and displayed pleocytosis, diffuse slow activity on EEG, increased blood flow velocity in both middle cerebral arteries on TCD, and single-photon emission computed tomography (SPECT) findings suggestive of diffuse involvement, especially in the left hemisphere.
To our knowledge, this is the first description of a patient with HaNDL, confusional symptoms, diffuse slow activity on EEG, and increased blood flow velocity in TCD. Our findings suggest a relationship between cerebral vasomotor changes and the pathophysiology of HaNDL. TCD may be a useful tool for early diagnosis of HaNDL.
HaNDL综合征(伴有脑脊液淋巴细胞增多的短暂性头痛和神经功能缺损)的特征是出现一次或多次头痛发作以及与脑脊液淋巴细胞增多相关的短暂性神经功能缺损。迄今为止,鲜有以意识模糊症状为表现的HaNDL病例被报道。同样,很少有伴有意识模糊症状的HaNDL患者接受过经颅多普勒超声(TCD)检查。局灶性受累患者的TCD数据显示出与血管舒缩改变一致的变化。
我们报告一例42岁男性患者,其出现头痛和意识模糊症状,脑脊液有淋巴细胞增多,脑电图显示弥漫性慢波活动,TCD显示双侧大脑中动脉血流速度增加,单光子发射计算机断层扫描(SPECT)结果提示弥漫性受累,尤其是在左半球。
据我们所知,这是首例关于伴有意识模糊症状、脑电图弥漫性慢波活动以及TCD血流速度增加的HaNDL患者的描述。我们的研究结果表明脑血管舒缩变化与HaNDL的病理生理学之间存在关联。TCD可能是早期诊断HaNDL的有用工具。