Episodic headache with spontaneous hypothermia reveal Shapiro\u2019s syndrome variant with effectiveness of clonidine therapy

Mickael Aubignat,Melissa Tir,Daniela Andriuta,Pierre Krystkowiak

doi:10.1186/s10194-021-01245-3

Mickael Aubignat, Melissa Tir + Show 2 more

Open Access

https://doi.org/10.1186/s10194-021-01245-3

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Abstract

BackgroundEpisodic 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 presentationWe 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.ConclusionsIn 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.

Highlights

Episodic headache with spontaneous hypothermia constitute an uncommon association and is not well recognized in the International Classification of Headache Disorders (ICHD-3)
We report an unusual case of Shapiro’s syndrome variant revealed by episodes of headache with spontaneous hypothermia in a parkinsonian patient
Headache with spontaneous hypothermia are not recognized in the ICHD-3 [1]

Summary

Introduction

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, called Shapiro’s syndrome, is a rare disease (less than 60 cases described) characterized by hypothermia attacks associated or not with hyperhidrosis without any triggering factor [2]. Headaches are uncommon and rarely in the foreground in Shapiro’s syndrome [2, 8]. We report an unusual case of Shapiro’s syndrome variant revealed by episodes of headache with spontaneous hypothermia in a parkinsonian patient.

Results

Conclusion