Headache Attributed to Genetic Vasculopathies.

PURPOSE OF REVIEW: Headaches are a common chief complaint and a significant contributor to growing healthcare costs. Headaches are broadly categorized as primary or secondary, the latter indicating that they are a symptom of an underlying medical condition. Genetic vasculopathies are a heterogeneous cause of secondary headache recognized by the International Classification of Headache Disorders 3rd edition, with limited but growing literature. This review aims to describe specific genetic vasculopathies, outline the pathophysiology of headaches caused by genetic vasculopathies, and summarize diagnostic and treatment strategies.

RECENT FINDINGS: The most reported headache in genetic vasculopathies is migraine with or without aura, though headache phenotypes vary widely, as do the extra-central nervous system and systemic manifestations. Treatment is largely supportive, including focused headache management based on phenotype. Several widely used preventive agents warrant careful risk-benefit consideration due to side effect profiles that may be particularly relevant in this patient population. Genetic vasculopathies are a clinically significant category of secondary headaches that are recognized in the International Classification of Headache Disorders 3rd edition. As our understanding of genetic vasculopathies grows, so does the need for high-quality research investigating the diagnosis and management of secondary headaches as well as their underlying conditions.