Late-onset post-lesional paroxysmal hypothermia: a case series and literature review

Abstract

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.

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Data availability

All anonymized data will be shared by request from any qualified investigator.

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Authors

Contributions

FM and SR designed the study; PD, LPD, ARM, PN, SE and SR acquired the data; PD, LPD and SR analysed raw data; PD, FM and SR prepared the initial draft of the manuscript; all authors revised the manuscript for intellectual content.

Corresponding author

Correspondence to Syvain Rheims.

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Conflicts of interest

None of the authors reports disclosure related to the study.

Ethical approval

The study complied with the French law about monocentre retrospective analysis of medical data.

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Devic, P., Peter-Derex, L., Richard-Mornas, A. et al. Late-onset post-lesional paroxysmal hypothermia: a case series and literature review. J Neurol (2020). https://doi.org/10.1007/s00415-020-10013-3

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Keywords

  • Paroxysmal hypothermia
  • Epilepsy
  • Hypersomnia
  • Hypothalamus lesions