Perrine Devic1,2, Laure Peter-Derex3,4, Aurélie Richard-Mornas1,3, Sylvie Ernesto5, Pascale Nesme5, François Mauguière1,3, Syvain Rheims6,7. 1. Department of Functional Neurology and Epileptology, Neurological Hospital, Hospices Civils de Lyon and University of Lyon, 59 Boulevard Pinel, 69677, Bron Cedex, Lyon, France. 2. Department of Neurology, Lyon Sud University Hospital, Hospices Civils de Lyon and University of Lyon, Lyon, France. 3. Lyon's Neurosciences Research Center, INSERM U1028, CNRS UMR5292, Claude Bernard Lyon 1 University, Lyon, France. 4. Department of Sleep Medicine, Hospices Civils de Lyon and University of Lyon, Lyon, France. 5. Department of Pneumology, Croix Rousse University Hospital, Hospices Civils de Lyon and University of Lyon, Lyon, France. 6. Department of Functional Neurology and Epileptology, Neurological Hospital, Hospices Civils de Lyon and University of Lyon, 59 Boulevard Pinel, 69677, Bron Cedex, Lyon, France. Sylvain.rheims@chu-lyon.Fr. 7. Lyon's Neurosciences Research Center, INSERM U1028, CNRS UMR5292, Claude Bernard Lyon 1 University, Lyon, France. Sylvain.rheims@chu-lyon.Fr.
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.
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.