Migraine and rare neurological disorders
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THE SPECTRUM OF MIGRAINE COMORBIDITIES
Migraine and rare neurological disorders Emma Scelzo 1 & Markus Kramer 2,3 & Simona Sacco 4 & Alberto Proietti 5 & Raffaele Ornello 4 & Eugenio Agostino Parati 1 & Anna Bersano 1
# Fondazione Società Italiana di Neurologia 2020
Abstract Although migraine is generally considered an idiopathic and isolated neurological condition, it may also represent the presenting symptom of several uncommon heritable and acquired neurological diseases contributing to the recognition of such conditions. Migraine may indeed present with atypical characteristics or prolonged duration and may be associated with specific neuroradiological findings that may help in identifying the underlying condition. However, features of migraine in rare diseases are usually little known because of the lack of systematic studies. The aim of this paper is to provide clinicians with an updated review on specific clinical and neuroradiological features of migraine in uncommon neurological diseases that may be helpful to their diagnosis and treatment. Therefore, the early diagnosis of these uncommon diseases is crucial for patients’ clinical management and for the implementation of therapeutic approaches aimed at targeting the underlying disease pathogenic mechanisms. Thus, when investigating patients affected by migraine, physicians should always be aware about rare causes of migraine that if misdiagnosed could seriously impact patients’ outcome. Given these relevant implications, future studies specifically assessing features of migraine in uncommon diseases are mandatory. Keywords Migraine . CADASIL . Mitochondrial disease . Fabry disease . Sneddon disease . Rare neurological diseases
Introduction Migraine is a highly prevalent episodic disorder characterized by recurrent attacks of moderate to severe unilateral headaches with throbbing pain, often accompanied by nausea and/or vomiting, photophobia, and phonophobia [1]. It is estimated that 20.2% of women and 9.4% of men suffer from this disorder [2]. Migraine may last several hours to days and in approximately one-third of patients is preceded, or more rarely followed, by transient focal visual, sensory, or other central nervous system symptoms, named aura [3]. Although the
* Anna Bersano [email protected] 1
Cerebrovascular Unit, Fondazione IRCCS Istituto Neurologico Carlo Besta, Via Celoria 11, 20133 Milan, Italy
2
Department of Neurology, Alfried Krupp Hospital, Essen, Germany
3
Medical Faculty, Heinrich-Heine-University, Düsseldorf, Germany
4
Department of Biotechnological and Applied Clinical Sciences, Neurological Institute, University of L’Aquila, L’Aquila, Italy
5
Headache and Neuroalgology Unit, Fondazione IRCCS Istituto Neurologico Carlo Besta, Milan, Italy
exact pathophysiology of migraine is not completely understood, several mechanisms such as inflammation, dysfunction of the descending pain-modulating network, and altered trigeminal and autonomic system function as well as genetic factors seem to be involved [2–5]. The dia
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