Cough headache

Cynthia Armand MD (Dr. Armand of Montefiore Medical Center has no relevant financial relationships to disclose.)
Matthew S Robbins MD (Dr. Robbins of Montefiore Headache Center is a site principal investigator for a clinical trial conducted by eNeura.)
Stephen D Silberstein MD, editor. (Dr. Silberstein, Director of the Jefferson Headache Center at Thomas Jefferson University, receives honorariums from Alder Biopharmaceuticals, Allergan, Amgen, Avanir Pharmaceuticals, Curelator, Depomed, Dr. Reddy's Laboratories, eNeura, electroCore Medical, Lilly USA, Supernus Pharmacerticals, Teva, and Trigemina for consulting and/or advisory panel membership.)
Originally released February 14, 2002; last updated February 7, 2017; expires February 7, 2020

This article includes discussion of cough headache, cough-associated headache, primary cough headache, and Valsalva maneuver headache. The foregoing terms may include synonyms, similar disorders, variations in usage, and abbreviations.

Overview

The authors discuss the clinical manifestations, etiology, differential diagnosis, diagnostic evaluation, and management of cough headache. In this article, studies giving insight into the cause of 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.

Key points

 

• 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 2013).

Table 1. International Headache Society Diagnostic Criteria for Primary Cough Headache

 

(A) At least 2 headache episodes fulfilling criteria B-D
(B) Brought on by and occurring only in association with coughing, straining, and/or other Valsalva maneuver
(C) Sudden onset
(D) Lasting between 1 second and 2 hours
(E) Not better accounted for by another ICHD-3 diagnosis*

*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 differentiating secondary cough headache from primary cough headache.

For clarity, we will separate primary cough headache (see table above) from primary exercise (previously exertional) headache, although some overlap occurs in these 2 conditions.

Table 2. International Headache Society Diagnostic Criteria for Primary Exercise (Previously Exertional) Headache

 

(A) At least 2 headache episodes fulfilling criteria B and C
(B) Brought on by and occurring only during or after strenuous physical exercise
(C) Lasting less than 48 hours
(D) Not better accounted for by another ICHD-3 diagnosis*

*On first occurrence of this headache type, it is mandatory to exclude subarachnoid hemorrhage and arterial dissection.

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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