[Spontaneous periodic hypothermia syndrome: A systematic review of the literature]

Abstract

Introduction: Periodic spontaneous hypothermia syndrome (PSHS), also known as hypersudation-hypothermia syndrome, is a rare condition characterised by the occurrence of paroxysmal attacks of spontaneous hypothermia occurring without age limit. Few studies have attempted to synthesise the available data on PSHS. The objective of this study was to summarize the literature on this condition.

Methods: A literature review was conducted using the Pubmed and ScienceDirect databases. Cases from a registry of PSHS initiated at the CHU of Saint-Étienne were added to the analysis. For each clinical case, clinical, biological and morphological data as well as therapeutic management and evolution were collected.

Results: Seventy-one cases of PSHS were collected. The median age at symptom onset was 21.5 years. The clinical sign most frequently associated with hypothermia was the presence of hyperhidrosis. The triad of hypothermia-hypersudation-agenesis of the corpus callosum was present in 35.7% of cases.

Conclusion: PSHS remains a poorly known cause of hypothermia, without consensual therapeutic options. The need to better describe cases and obtain more long-term data will allow a better definition and knowledge of this syndrome.

Keywords: Hypothermia; Hypothermie; Hypothermie périodique spontanée; Hypothermie récidivante spontanée; Recurrent spontaneous hypothermia; Shapiro syndrome; Spontaneous periodic hypothermia; Syndrome de Shapiro.