Other Primary Headache Disorders

Jonathan H. Smith, MD, FAHS Headache p. 652-664 June 2021, Vol.27, No.3 doi: 10.1212/CON.0000000000000960
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PURPOSE This article provides an overview of a diverse group of primary headache disorders that are categorized in the International Classification of Headache Disorders, 3rd Edition (ICHD-3), as “other primary headache disorders.” This article provides clinicians with a distilled understanding of the diagnoses and their epidemiology, pathophysiology, and management.

RECENT FINDINGS Cough-induced headache requires neuroimaging to exclude posterior fossa pathology and recently has been reported as a common symptom in patients with CSF-venous fistula. Clinical overlap is observed between patients with primary exercise headache and primary headache associated with sexual activity. Patients with recurrent thunderclap headache associated with sexual activity should be presumed to have reversible cerebral vasoconstriction syndrome until proven otherwise. De novo external-pressure headache is a common sequela among health care workers using personal protective equipment during the COVID-19 pandemic. New daily persistent headache is an important mimicker of chronic migraine or chronic tension-type headache and is distinguished by a daily-from-onset progression of persistent headache; a treatment-refractory course is often observed, and early involvement of a multidisciplinary team, including a psychotherapist, is advised.

SUMMARY Patients with primary headache disorders that are classified as “other primary headache disorders” have presentations with unique diagnostic and management considerations. The disorders are highly recognizable, and an appreciation of the diagnoses will aid clinicians in providing safe and effective care for patients presenting with headache.

Address correspondence Dr Jonathan H. Smith, Department of Neurology, Mayo Clinic, 13400 E Shea Blvd, Scottsdale, AZ 85259, [email protected].

RELATIONSHIP DISCLOSURE: Dr Smith receives publishing royalties from UpToDate, Inc.

UNLABELED USE OF PRODUCTS/INVESTIGATIONAL USE DISCLOSURE: Dr Smith discusses the unlabeled/investigational use of medications for the treatment of primary headache disorders, none of which are approved by the US Food and Drug Administration.

© 2021 American Academy of Neurology.