Background

Paroxysmal hypothermia (PH) is a rare condition characterized by recurrent episodes of spontaneous hypothermia, bradycardia, disorders of consciousness and, in some cases, hyperhidrosis. When associated with a detectable hypothalamic lesion, PH episodes usually occur shortly after the brain insult.

Methods

We performed a retrospective study to identify patients who had demonstrated at least one episode of symptomatic spontaneous PH as defined by (i) tympanic temperature < 35 °C; (ii) drowsiness and/or confusion state and/or coma; (iii) duration of the episode ≥ 24 h; (iv) absence of other condition resulting in hypothermia

Results

Among 8824 patients, we identified four patients with recurrent late-onset PH episodes of 1–26-day duration that occurred 6–46 years after the brain insult. The lesion always involved the diencephalon. All patients suffered from epilepsy and three of hypopituitarism. PH episode typically included severe hypothermia, bradycardia, drowsiness, thrombocytopenia and in some patients central hypoventilation and narcolepsy-like hypersomnia. In ¼ of episodes, confusion was mistaken as non-convulsive epileptic manifestation resulting in benzodiazepine administration which aggravated symptoms. In the two patients with nocturnal hypoventilation, chronic non-invasive ventilation with bi-level positive airway pressure allowed cessation of symptomatic episodes.

Discussion

Late-onset post-lesional PH is exceptional with only a single case hitherto reported in the literature. Distinguishing hypothermia-related disturbances of consciousness from epileptic seizures or post-ictal phenomena is crucial since treatment with benzodiazepines may worsen hypothermia through their action on GABAa receptors. Lastly, PH may be associated with sleep disorders and hypoventilation, for which investigations and treatment should be considered.

中文翻译：

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迟发性病后阵发性体温过低：病例系列及文献复习。

背景

阵发性体温过低（PH）是一种罕见的疾病，其特征是反复发作的自发性体温过低，心动过缓，意识障碍以及在某些情况下多汗症。当与可检测到的下丘脑病变相关时，PH发作通常会在大脑受到伤害后不久发生。

方法

我们进行了一项回顾性研究，以发现表现出至少一次症状性自发性PH的患者，这些患者的定义为：（i）鼓膜温度<35°C；（ii）嗜睡和/或混乱状态和/或昏迷；（iii）发作持续时间≥24小时；（iv）没有其他情况导致体温过低

结果

在8824例患者中，我们确定了4例脑损伤后6-46年发生的复发性晚期PH发作，发作期为1至26天。病变总是累及中脑。所有患者均患有癫痫和三种垂体功能低下。PH发作通常包括严重的体温过低，心动过缓，嗜睡，血小板减少以及某些患者的中央换气不足和发作性睡病样失眠。在1/4的发作中，混淆被误认为是非惊厥性癫痫表现，导致苯二氮卓类药物的使用加剧了症状。在两名夜间换气不足的患者中，慢性非侵入性通气且气道正压为双级可停止症状发作。

讨论区

病后晚期PH例外，文献中迄今仅报道了一例。区分与低温相关的意识障碍与癫痫发作或发作后现象是至关重要的，因为用苯二氮卓类药物治疗可能会通过其对GABA a受体的作用而恶化低温。最后，PH可能与睡眠障碍和通气不足有关，应考虑进行调查和治疗。