Episodic headache with spontaneous hypothermia reveal Shapiro’s syndrome variant with effectiveness of clonidine therapy

• Main Authors: Mickael Aubignat, Melissa Tir, Pierre Krystkowiak, Daniela Andriuta
• Format: Article
• Language: English
• Published: BMC 2021-04-01
• Series: The Journal of Headache and Pain
• Subjects: Headache; Hypothermia; Hyperhidrosis; Shapiro’s syndrome; Parkinson’s disease; Clonidine
• Online Access: https://doi.org/10.1186/s10194-021-01245-3

Abstract Background Episodic headache with spontaneous hypothermia constitute an uncommon association and is not well recognized in the International Classification of Headache Disorders (ICHD-3). Spontaneous periodic hypothermia, also called Shapiro’s syndrome, is a rare disease characterized by hypothermia attacks associated or not with hyperhidrosis without any triggering factor. Case presentation We report a rare case of Shapiro’s syndrome variantrevealed by episodes of headache with spontaneous hypothermia witheffectiveness of clonidine therapy in a 76-year-old Parkinson’s disease woman. Conclusions In the literature, apart from Shapiro’s syndrome, headache withhypothermia seem to occur very rarely. In our case,these symptoms may be considered as a very rare non-motor fluctuation ofParkinson’s disease.