What is primary cough headache
Primary cough headache which has a lifetime prevalence of 1%, is a sudden-onset bilateral or unilateral headache lasting seconds to 2 hours without associated symptoms provoked by coughing, sudden postural movements, weightlifting, laughing, and defecating and usually occurring in people over the age of 40 years.
Cough headache is a term used to describe headaches triggered by coughing and other activities associated with a Valsalva maneuver, such as laughing, straining at stool, lifting, and bending the head toward the ground. Clinicians have identified the following two types of cough headache:
- • Benign primary cough headache
- • Symptomatic cough headache
Secondary pathology should be excluded including Chiari type 1 malformation, posterior fossa lesions, unruptured cerebral aneurysms, spontaneous intracranial hypotension, and subdural hematoma by obtaining an MRI of the brain with and without contrast, and MRA (magnetic resonance angiogram) of the brain.
Initially, both types of cough headache were thought to be related to sexual and exertional headaches, but they are now considered distinct clinical entities. A strong male predilection is seen for benign cough headache and no gender predilection for symptomatic cough headache.
Signs and Symptoms
Patients suffering from cough headache present differently depending on the type of cough headache experienced. Each clinical presentation is discussed.
Benign Cough Headache
Benign cough headache is not associated with obvious neurological or musculoskeletal disease. More than 80% of patients with benign cough headache are males, in contradistinction to symptomatic cough headache, in which no gender predilection is seen. The onset of benign cough headache is abrupt, occurring immediately after coughing or other activities that cause a Valsalva maneuver.
Although the intensity of pain is severe and peaks rapidly, it lasts only seconds to minutes. The character of the pain associated with benign cough headache is splitting or sharp, and the pain is in the occipital region bilaterally and occasionally the vertex of the skull.
No accompanying neurological or systemic symptoms are seen, as with cluster and migraine headaches.
The age of onset of benign cough headache is generally in the late fifth or sixth decade of life. If such headaches occur before age 50, there should be strong clinical suspicion that the patient either has symptomatic cough headache or a pathological condition in the posterior fossa, such as Arnold-Chiari malformation or tumor.
Tumors of the foramen magnum also may mimic the presentation of benign cough headache even if no neurological symptoms are present.
Symptomatic Cough Headache
Symptomatic cough headache is almost always associated with structural abnormalities of the cranium, such as Arnold-Chiari malformation I and II or intracranial tumors.
The symptoms associated with symptomatic cough headache are thought to be due to herniation of the cerebellar tonsil through the foramen magnum into the space normally occupied by the upper portion of the cervical spinal cord.
Similar to benign cough headache, the onset of pain associated with symptomatic cough headache is abrupt, occurring immediately after coughing or other activities that cause a Valsalva maneuver.
Although the intensity of pain is severe and peaks rapidly, it lasts only seconds to minutes. In contrast to benign cough headache, associated neurological symptoms may be present, including difficulty swallowing, faintness, and numbness in the face and upper extremities.
These associated symptoms should be taken very seriously because they are indicative of increased intracranial pressure and herniation of the intracranial contents.
The cough should be treated.
Indomethacin 50 to 200 mg daily in divided doses can be very effective (combined with a proton pump inhibitor for prolonged use). Acetazolamide, topiramate, propranolol, and naproxen may also be effective.
The character of the pain associated with symptomatic cough headache is splitting or sharp, and pain is in the occipital region bilaterally and occasionally the vertex of the skull.
The age of onset of symptomatic cough headache is generally in the third decade of life, although, depending on the amount of neurological compromise, it may occur at any age. In contrast to benign cough headache, which occurs predominantly in men, symptomatic cough headache occurs with equal prevalence in both genders.
How is cough headache diagnosed?
Magnetic resonance imaging (MRI) of the brain provides the best information regarding the cranial vault and its contents. MRI is highly accurate and helps identify abnormalities that may put the patient at risk for neurological disasters secondary to intracranial and brainstem pathological conditions, including tumors and demyelinating disease.
Special attention to the foramen magnum may help identify more subtle abnormalities responsible for posterior fossa neurological signs and symptoms. MRI helps identify bleeding associated with leaking intracranial aneurysms, which may mimic the symptoms of both types of cough headache.
Magnetic resonance angiography (MRA) may be useful in helping identify aneurysms responsible for the patient’s neurological symptoms. In patients who cannot undergo MRI, such as patients with pacemakers, computed tomography (CT) is a reasonable second choice. Lumbar puncture should be performed if intracranial hemorrhage is suspected, even if blood is not present on MRI or CT.
Plain radiographs of the cervical spine also may be useful in the evaluation of Arnold-Chiari malformations and should be included in the evaluation of all patients with cough headache.
Screening laboratory tests consisting of complete blood cell count, erythrocyte sedimentation rate, and automated blood chemistry testing should be performed if the diagnosis of cough headache is in question. Intraocular pressure should be measured if glaucoma is suspected.
Cough headache is a clinical diagnosis supported by a combination of clinical history, physical examination, radiography, MRI, and MRA.
Pain syndromes that may mimic cough headache include benign exertional headache, ice pick headache, sexual headache, trigeminal neuralgia involving the first division of the trigeminal nerve, demyelinating disease, cluster headache, and chronic paroxysmal hemicrania. Trigeminal neuralgia involving the first division of the trigeminal nerve is uncommon and is characterized by trigger areas and tic-like movements.
Demyelinating disease is generally associated with other neurological findings, including optic neuritis and other motor and sensory abnormalities.
The pain of chronic paroxysmal hemicrania and cluster headache is associated with redness and watering of the ipsilateral eye, nasal congestion, and rhinorrhea during the headache.
These findings are absent in all types of cough headache. Migraine headache may or may not be associated with painless neurological findings known as aura, but the patient almost always reports some systemic symptoms, such as nausea or photophobia, not typically associated with cough headache.
Indomethacin is the treatment of choice for benign cough headache. A starting dose of 25 mg daily for 2 days and titrating to 25 mg three times per day is a reasonable treatment approach. This dose may be carefully increased up to 150 mg per day. Indomethacin must be used carefully, if at all, in patients with peptic ulcer disease or impaired renal function.
Headache specialists have noted anecdotal reports of a positive response to cyclooxygenase-2 (COX-2) inhibitors in the treatment of benign cough headache. Underlying sleep disturbance and depression are best treated with a tricyclic antidepressant compound, such as nortriptyline, which can be started at a single bedtime dose of 25 mg.
The only uniformly effective treatment for symptomatic cough headache is surgical decompression of the foramen magnum.
This surgery is usually done via suboccipital craniectomy. Surgical decompression prevents the low-lying cerebellar tonsils from obstructing the flow of spinal fluid from the cranium to the spinal subarachnoid space during a Valsalva maneuver.
Failure to diagnose cough headache correctly may put the patient at risk if intracranial pathology or demyelinating disease, which may mimic the clinical presentation of cough headache, is overlooked.
MRI and MRA are indicated in all patients thought to have cough headache. Failure to diagnose glaucoma, which also may cause intermittent ocular pain, may result in permanent loss of sight.
Any patient presenting with headaches associated with exertion or Valsalva maneuver should be taken very seriously. Although statistically most of these headaches ultimately are proved to be of benign cause, a few patients have potentially life-threatening disease.
The diagnosis of cough headache is made by obtaining a thorough, targeted headache history and performing a careful physical examination.
The clinician must separate patients suffering from benign cough headache from patients suffering from symptomatic cough headache. Patients with benign cough headache should have a normal neurological examination.
If the neurological examination is abnormal, the diagnosis of benign cough headache should be discarded and a careful search for the cause of the patient’s neurological findings should be undertaken.
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Cutrer FM, DeLange J: Cough, exercise, and sex headaches. Neurol Clin 32:433-450, 2014.