Department of Neurology, The First Medical Center, Chinese PLA General Hospital, Beijing, China.
School of Medicine, Nankai University, Tianjin, China.
Headache. 2020 Feb;60(2):382-395. doi: 10.1111/head.13687. Epub 2019 Nov 8.
Neuronal intranuclear inclusion disease (NIID) is considered a heterogeneous disease because of its highly variable clinical manifestations. To date, there are no reports of NIID patients presenting with hemiplegic migraine (HM)-like headache, or of HM and NIID co-occurring as comorbidity, and the connection between these 2 seemingly unrelated clinical conditions has yet to be established.
We present a patient with NIID who was previously diagnosed with HM. To determine the pathogenesis of HM in this NIID patient, we systematically reviewed published NIID and HM cases and cataloged them based on their clinical manifestations.
The clinical manifestations of NIID is highly various; however, there is no case reported to date that shows HM-like symptoms or cerebral edema. All documented symptomatic HM cases show vascular dysfunction to various degrees, but none of them has been shown to be correlated with NIID.
Our patient is the first documented case in which HM and NIID occur simultaneously. Vascular dysfunctions that cause cerebral hypoperfusion and glucose hypometabolism, two of the dominant causes of symptomatic HM, may be associated with the accumulation of eosinophilic hyaline inclusions that cause NIID. However, the existence of inclusions may also alter neuronal behavior and indirectly cause cerebral hypoperfusion and glucose hypometabolism. Further research and observations are needed to examine the relationship between HM and NIID.
神经元核内包涵体病（NIID）因其临床表现高度多变，被认为是一种异质性疾病。迄今为止，尚无 NIID 患者出现偏瘫性偏头痛（HM）样头痛的报道，也无 HM 和 NIID 同时发生作为共病的报道，这两种看似无关的临床病症之间的联系尚未建立。
我们报告了一例先前被诊断为 HM 的 NIID 患者。为了确定该 NIID 患者 HM 的发病机制，我们系统地回顾了已发表的 NIID 和 HM 病例，并根据其临床表现对其进行了分类。
NIID 的临床表现高度多样；然而，迄今为止尚无报告显示 HM 样症状或脑水肿。所有有症状的 HM 病例均显示不同程度的血管功能障碍，但均与 NIID 无关。
我们的患者是首例同时发生 HM 和 NIID 的病例。引起 HM 的两个主要原因——脑灌注不足和葡萄糖代谢不足，可能与导致 NIID 的嗜酸性玻璃样包涵体的积累有关。然而，包涵体的存在也可能改变神经元的行为，并间接导致脑灌注不足和葡萄糖代谢不足。需要进一步的研究和观察来检查 HM 和 NIID 之间的关系。
