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[伴有脑脊液淋巴细胞增多的短暂性头痛和神经功能缺损综合征(HaNDL)]

[The syndrome of transient headache and neurologic deficits with cerebrospinal fluid lymphocytosis (HaNDL)].

作者信息

Säflund Malin, Sjöstrand Christina, Sveinsson Olafur

机构信息

Karolinska Universitetssjukhuset - Department of neurology Stockholm, Sweden Karolinska Universitetssjukhuset - Department of neurology Stockholm, Sweden.

Karolinska Institutet Department of Clinical Neuroscience - Stockholm, Sweden Karolinska Institutet Department of Clinical Neuroscience - Stockholm, Sweden.

出版信息

Lakartidningen. 2018 Feb 26;115:EWPH.

Abstract

The syndrome of transient headache and neurologic deficits with cerebrospinal fluid lymphocytosis (HaNDL) is a self-limited disorder with an unknown pathogenesis, characterized by one or more transient episodes of severe headache accompanied with neurologic deficits, and lymphocytic pleocytosis in the cerebrospinal fluid (CSF). Despite being uncommon and benign it is important for clinicians to identify and differentiate HaNDL from other potentially fatal neurologic disorders. We present six HaNDL patients from our institution. All had a relatively typical course with repeated migraine-like headaches accompanied by various transient neurologic deficits and a mean CSF lymphocytic pleocytosis of 178 cells/mm3. Most of the patients were disorientated during the attacks, which has been described previously. When neurofilament light in CSF was measured, there was a substantial increase of this marker which normalized after several months, suggesting certain nerve damage.

摘要

伴有脑脊液淋巴细胞增多的短暂性头痛和神经功能缺损综合征(HaNDL)是一种发病机制不明的自限性疾病,其特征为一次或多次伴有神经功能缺损的严重头痛短暂发作,以及脑脊液(CSF)淋巴细胞增多。尽管HaNDL并不常见且为良性疾病,但临床医生识别并将其与其他可能致命的神经系统疾病区分开来很重要。我们展示了来自我们机构的6例HaNDL患者。所有患者病程相对典型,反复出现类似偏头痛的头痛,伴有各种短暂性神经功能缺损,CSF淋巴细胞平均增多至178个细胞/mm³。大多数患者在发作期间出现定向障碍,这在之前已有描述。当测量CSF中的神经丝轻链时,该标志物大幅增加,数月后恢复正常,提示存在一定的神经损伤。

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