Episodic Headaches with Spontaneous Hypothermia Reveal Shapiro’s Syndrome with Effectiveness of Clonidine Therapy

Abstract Background: Episodic headaches 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 revealed by episodes of headache with spontaneous hypothermia with effectiveness of clonidine therapy in a 76-year-old Parkinson’s disease woman. Conclusions: In the literature, apart from Shapiro’s syndrome, headaches with hypothermia seem to occur very rarely. These symptoms may be considered as a very rare non-motor fluctuation of Parkinson’s disease.