Dr. Jain of Thomas Jefferson University Hospital has no relevant financial relationships to disclose.)
Dr. Jhunjhunwala of Palmetto Health and University of South Carolina in Columbia has no relevant financial relationships to disclose.)
Dr. Silberstein, Director of the Jefferson Headache Center at Thomas Jefferson University, receives honorariums from Allergan, Avanir Pharmaceuticals, Curelator, Depomed, Dr. Reddy's Laboratories, eNeura, INSYS Therapeutics, Lilly USA, Supernus Pharmacerticals, Thernica and Trigemina for consulting. He is also the principal investigator for a clinical trials conducted by Alder Biopharmaceuticals, Amgen, electroCore Medical, Lily USA and Teva.)
This article includes discussion of primary cough headache, cough headache, benign cough headache, and Valsalva maneuver headache. The foregoing terms may include synonyms, similar disorders, variations in usage, and abbreviations.
The authors discuss the clinical manifestations, etiology, differential diagnosis, diagnostic evaluation, and management of primary cough headache. In this article, studies giving insight into the cause of primary cough headache in patients with or without Chiari I malformations are reviewed. Morphometric MRI studies that have shed new light on the etiology of primary cough headache are summarized, and evidence that CSF leaks may present as cough headache without an orthostatic component is presented. Although indomethacin is typically used to treat primary cough headache, the authors discuss other treatment options, including topiramate.
• Primary cough headache is typically bilateral, of sudden onset, lasts less than 1 minute, and is precipitated rather than aggravated by coughing.
• Primary cough headache can be triggered by other sudden Valsalva maneuvers, but it is not typically triggered by sustained physical exercise.
• Every patient presenting with cough headache should have an MRI of the brain to rule out a posterior fossa lesion. The MRI should be done with gadolinium looking for pachymeningeal enhancement because headache secondary to spontaneous leak can present as cough headache alone, with no orthostatic component.
• Indomethacin is the de facto preventive drug of choice for primary cough headache.
Historical note and terminology
Headache aggravated or brought on by cough has long been known to be a symptom of intracranial disease (Ekbom 1986). This chapter will focus on primary cough headache, which by definition is unassociated with an intracranial disorder (Headache Classification Committee of the International Headache Society 2018).
Table 1. International Headache Society Diagnostic Criteria for Primary Cough Headache
(A) At least 2 headache episodes fulfilling criteria B-D
(1) Headache arises moments after the cough or other stimulus.
(2) Headache reaches its peak almost immediately, and then subsides over several seconds to a few minutes (although some patients experience mild to moderate headache for 2 hours).
(3) The syndrome of cough headache is symptomatic in about 40% of cases and the large majority of these represent Chiari type I malformations. Other reported causes of symptomatic cough headache include CSF hypotension, carotid or vertebrobasilar disease, cerebral aneurysms, middle cranial fossa or posterior fossa tumors, midbrain cyst, basilar impression, platybasia, subdural hematoma, and reversible cerebral vasoconstriction syndrome. Diagnostic neuroimaging plays an important role in the search for possible intracranial lesions or abnormalities. Because subtentorial tumors account for more than 50% of intracranial space-occupying lesions in children, cough headache in pediatric patients should be considered symptomatic until proved otherwise.
Table 2. International Headache Society Diagnostic Criteria for Probable Primary Cough Headache
(A) Either of the following: (1) a single headache episode fulfilling criteria B-D; (2) at least 2 headache episodes fulfilling criterion B and either of criteria C and D
Primary cough headache can be triggered by other sudden Valsalva maneuvers but is typically not triggered by sustained physical exercise (Pascual et al 2008). This is the opposite of the typical circumstance in primary exercise headache.
In 1932, Tinel described 4 patients with headache brought on by coughing, nose-blowing, breath-holding, and bending the head forward (Lance and Goadsby 2000). Before Symonds and Rooke s reports, cough headaches and exertional headaches were always considered ominous symptoms, and there was no clear recognition that benign or primary types of these headaches existed. In 1968, Rooke noted that “in every patient with this complaint, an intracranial lesion of potentially serious nature, such as brain tumor, aneurysm, or vascular anomaly, has been suspected; and even when no such lesion could be identified, an uneasy uncertainty usually has remained" (Rooke 1968).
The landmark paper entitled “Cough Headache” by Sir Charles Symonds brought attention to this disorder (Symonds 1956). Symonds clearly described cases of both secondary and primary cough headache. He presented patients with headache provoked by coughing, and noted that sneezing, straining at stool, laughing, or stooping could also provoke the headache. He did not describe headache precipitated by physical exercise. He outlined the clinical course of primary cough headache and suggested a pathophysiological mechanism for the disorder.
In 1968, Rooke reviewed 93 patients with primary exertional headache (Rooke 1968). He did not separate cough headache from headaches caused by running. However, his data underscored Symonds concept that cough headache could be benign.
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