Institute of Neurology, AOU Mater Domini, Magna Graecia University, Catanzaro, Italy.
Anesthesia and Intensive Care, Department of Medical and Surgical Sciences, Magna Graecia University, Catanzaro, Italy.
Neurol Sci. 2023 Sep;44(9):3017-3028. doi: 10.1007/s10072-023-06788-8. Epub 2023 Apr 3.
To assess epidemiological, clinical and neuroimaging features of acute confusional state in the Headache and Neurological Deficits with cerebrospinal fluid Lymphocytosis (HaNDL) syndrome.
HaNDL is an increasingly recognized syndrome in which migraine-like headache episodes accompanied by hemiparaesthesia and/or hemiparesis and/or dysphasia are associated to CSF lymphocytic pleocytosis. The International Classification of Headache Disorders (ICHD-3) includes HaNDL syndrome in group 7 "headache attributed to non-vascular intracranial disorder" code 7.3.5, and lists the HaNDL-associated signs/symptoms that may be found less frequently. Confusional state is not mentioned in the 7.3.5-ICHD-3 "notes" or "comments" section as part of the HaNDL neurological spectrum. Moreover, the acute confusional state pathogenesis in HaNDL syndrome remains still uncertain and debated.
Here, we report a 32-year-old male who complained episodes of migraine-like headache and left hemiparaesthesia complicated by confusional state which led to discovering CSF lymphocytosis. Since other workup to determine the cause of his symptoms was otherwise negative, he was diagnosed as having HaNDL syndrome. We also ascertained and reviewed all available reports of HaNDL to assess the significance of confusional state in this syndrome.
The search yielded 159 HaNDL cases among single reports and small/large series. Out of 159 patients who fulfilled the inclusion criteria for HaNDL according to the current ICHD at the time of diagnosis, 41 (25.7%) presented with acute confusional state. Among 41 HaNDL patients with confusional state, 16 (66.6%) out of 24 who underwent spinal tap had increased opening pressure.
We propose that a mention of acute confusional state may be included in the "comments" section of "7.3.5-syndrome of transient headache and neurological deficits with cerebrospinal fluid lymphocytosis (HaNDL)," when ICHD-3 diagnostic criteria will be updated. Moreover, we speculate that intracranial hypertension may play a role in the pathogenesis of the acute confusional state associated to HaNDL syndrome. Larger case series are needed to evaluate this hypothesis.
评估头痛和脑脊液淋巴细胞增多伴神经功能缺损（HaNDL）综合征中急性意识混乱的流行病学、临床和神经影像学特征。
HaNDL 是一种日益被认识的综合征，其偏头痛样头痛发作伴有偏身感觉障碍和/或偏瘫和/或言语障碍，并伴有脑脊液淋巴细胞增多。《国际头痛疾病分类（ICHD-3）》将 HaNDL 综合征纳入第 7 组“归因于非血管性颅内疾病的头痛”代码 7.3.5，并列出了 HaNDL 相关的体征/症状，这些症状/体征可能不太常见。意识混乱在 ICHD-3 的 7.3.5 注释或评论部分中并未被提及，作为 HaNDL 神经谱的一部分。此外，HaNDL 综合征中急性意识混乱的发病机制仍不确定，存在争议。
在这里，我们报告了一名 32 岁男性，他抱怨偏头痛样头痛发作和左侧偏身感觉障碍，伴有意识混乱，导致发现脑脊液淋巴细胞增多。由于其他确定其症状原因的检查均为阴性，因此他被诊断为 HaNDL 综合征。我们还确定并审查了所有现有的 HaNDL 报告，以评估该综合征中意识混乱的意义。
搜索结果显示，在单篇报告和小/大系列中共有 159 例 HaNDL 病例。在根据当时的 ICHD 诊断符合 HaNDL 纳入标准的 159 名患者中，有 41 名（25.7%）出现急性意识混乱。在 41 名 HaNDL 伴意识混乱的患者中，有 24 名接受了腰椎穿刺，其中 16 名（66.6%）颅内压升高。
我们建议，当 ICHD-3 诊断标准更新时，可能会在“7.3.5-短暂性头痛和脑脊液淋巴细胞增多伴神经功能缺损综合征（HaNDL）”的注释部分提及急性意识混乱。此外，我们推测颅内压升高可能在 HaNDL 综合征相关的急性意识混乱发病机制中起作用。需要更大的病例系列来评估这一假设。
