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10 Short-Lasting Unilateral Neuralgiform Headache Attacks with Cranial Autonomic…
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G. Sebastianelli et al.
Part III
Other Primary Headache Disorders
Chapter 11
Primary Cough Headache
PingKunChen, HungYiWu, andShuu-JiunWang
11.1 Introduction
Primary cough headache (PCH) is a headache disorder characterized by sudden and
intense pain triggered by coughing or other Valsalva maneuvers (e.g., sneezing,
bending over, or straining) without evidence of intracranial lesions [1]. It typically
manifests as a brief, sharp headache that can range from a few seconds to two hours
in duration, and it is usually bilateral. The condition, while uncommon, is important
to recognize, as it can sometimes be confused with other, more severe neurological
conditions that require urgent treatment. This chapter reviews the pathophysiology,
headache characteristics, diagnostic algorithm, and management strategies for primary cough headache, aiming to provide a comprehensive understanding of this
distinctive headache disorder.Primary cough headache is considered a rare condition. Epidemiological studies suggest that the prevalence of PCH is relatively low,
with estimates of its occurrence ranging between 0.4 and 1.2% of all headache
P. Chen
School of Medicine, China Medical University College of Medicine, Taichung, Taiwan
Bozhi Clinic, Taichung, Taiwan
H. Y. Wu
Zhijia Clinic, Taichung, Taiwan
S.-J. Wang (
College of Medicine, National Yang Ming Chiao Tung University, Taipei, Taiwan
Department of Neurology, Neurological Institute, Taipei Veterans General Hospital,
Taipei, Taiwan
Brain Research Center, National Yang Ming Chiao Tung University, Taipei, Taiwan
e-mail: sjwang@vghtpe.gov.tw
Switzerland AG 2026
D. Uludüz et al. (eds.), Rare Causes of Headache Disorders, Headache,
https://doi.org/10.1007/978-3-032-10242-3_11
*)
109© The Author(s), under exclusive license to Springer Nature
110
P. Chen et al.
clinic referrals [2, 3]. It primarily affects adults, particularly those between the ages
of 40 and 60, and is more common in men than women, with a male-to-female ratio
of approximately 3:1 [3, 4]. The onset of symptoms typically occurs after the age of
40 [5], and the condition is often observed in individuals who have a history of
chronic coughing or heavy physical exertion.
11.2 Pathophysiology
The pathophysiology of PCH remains incompletely understood, with several
hypotheses proposed to explain its occurrence. One of the most widely accepted
theories is that PCH is triggered by an increase in intracranial pressure during
Valsalva maneuvers such as coughing, sneezing, or straining. These actions can
cause a sudden rise in pressure within the skull, which may affect sensitive intracranial structures, leading to headache. The sharp, intense pain associated with PCH is
believed to result from this brief but signicant increase in pressure, which can
sensitize pain pathways in the brain. Several contributing factors may enhance this
pressure increase, including increased central venous pressure, posterior fossa
crowding, heightened venous pressure due to venous stenosis, and a relative increase
in the pressure gradient between cisternal and lumbar cerebrospinal uid (CSF).
Additionally, receptor sensitization due to neuroinammation or prior irritation may
also play a role, making the cranial structures more responsive to pressure changes
during Valsalva maneuvers [6].
A study by Raskin etal. evaluated 30 patients with PCH and offered them two
treatment options: indomethacin or lumbar puncture (LP) [7]. Sixteen patients
chose indomethacin, while fourteen opted for LP.The results revealed that the opening pressures and basic cellular counts were within normal limits for all patients, but
six of the fourteen patients who underwent LP showed immediate improvement
after the procedure. Based on these ndings, the authors suggested that the increase
in central venous pressure, particularly through the jugular venous system and epidural venous plexus, during the Valsalva maneuver plays a signicant role in triggering the headache. The rise in intrathoracic and intra-abdominal pressure during
coughing is believed to contribute to this phenomenon, ultimately resulting in the
sharp headache characteristic of PCH.
Our study found that posterior fossa crowdedness may be another contributing
factor to PCH.Using morphometric magnetic resonance imaging (MRI) analysis,
we found that patients with PCH had a signicantly smaller posterior cranial fossa
(PCF) area compared to controls, leading to a higher hindbrain/PCF ratio [8]. This
crowding may cause increased intracranial pressure during Valsalva maneuvers,
contributing to the development of PCH.These anatomical abnormalities, including
shorter clivus length and a lower position of the cerebellar tonsillar tip, indicate that
structural factors may play a role in making the brain more vulnerable to pressure
changes .
11 Primary Cough Headache
111
Another study found that heightened venous pressure due to venous stenosis
may contribute to the development of PCH [9]. This theory posits that abnormalities in the venous system, particularly in the jugular or epidural veins, could
lead to increased pressure in the cranial veins during Valsalva maneuvers. The
elevated venous pressure could make the brain more sensitive to pressure
changes, thereby triggering the headache. Venous congestion, possibly exacerbated by factors like venous stenosis, could thus be a key mechanism in the
pathophysiology of PCH.
Another study regarding the pathophysiology of PCH suggests that the disorder
may be linked to a relative increase in the pressure gradient between cisternal and
lumbar cerebrospinal uid (CSF) pressure, rather than a direct rise in intracranial
pressure. In our prior small open-label trial, acetazolamide was successfully used to
treat four out of ve patients with PCH.We proposed that the increased pressure
gradient, which affects the ow of CSF, could contribute to the onset of headaches
during Valsalva maneuvers like coughing [10]. This theory emphasizes the role of
pressure uctuations in the CSF compartments rather than focusing solely on
changes in intracranial pressure.
Another contributing factor involves receptor sensitization, which may be triggered by prior infections or other factors that lead to neuroinammation [11]. The
pain receptors in the cranial structures become more sensitive due to repeated irritation or inammation. When the pressure in the cranial cavity increases during activities like coughing, the already sensitized receptors may overreact, resulting in the
sharp, lancinating pain characteristic of PCH.This sensitization could make individuals more prone to experiencing headaches when exposed to triggers that might
otherwise not cause such a response.
11.3 Case Presentation
A 33-year-old male engineer with no prior history of headache or other systemic
illnesses presented with a sudden onset headache. He reported engaging in regular
weight training and experienced an abrupt, severe, dull headache involving the
entire head during a heavy lift while performing a Valsalva maneuver. The headache
reached peak intensity rapidly and resolved spontaneously within approximately
one minute. A similar headache recurred upon repeating the same maneuver. Over
the subsequent days, he noted the recurrence of identical headaches triggered by
coughing and sneezing.
There were no associated symptoms such as nausea, vomiting, photophobia,
phonophobia, or cranial autonomic features (e.g., lacrimation, rhinorrhea). Physical
and neurological examinations were unremarkable. Brain magnetic resonance
imaging (MRI) revealed no signicant abnormalities. The patient was started on
indomethacin therapy, to which he responded excellently, with complete resolution
of symptoms.
112
P. Chen et al.
11.4 Case Discussion
This case illustrates a typical presentation of primary cough headache, a benign
headache disorder characterized by brief, sudden-onset head pain triggered by
activities that acutely increase intracranial pressure, such as coughing, sneezing, or
performing a Valsalva maneuver. The patient reported transient, intense, diffuse
headaches consistently provoked by these maneuvers, in the absence of associated
symptoms or focal neurological decits.
Although the clinical features strongly suggest a primary cough headache, it is
essential to exclude secondary causes, including posterior fossa lesions (e.g., Chiari
I malformation), vascular anomalies, or other structural abnormalities, as they can
present with similar manifestations. In this case, neuroimaging with brain MRI
demonstrated no abnormalities. The patient showed an excellent response to indomethacin, a nding that further supports the diagnosis of primary cough headache.
11.5 Clinical Characteristics
Primary cough headache is characterized by:
• Sudden onset: The pain typically begins abruptly and reaches its peak intensity
within seconds to minutes of the triggering event (i.e., coughing or sneezing).
• Intensity: The headache is usually described as moderate to severe, sharp, and
lancinating.
• Duration: It is generally a brief headache, lasting anywhere from a few seconds
to several minutes. However, some cases may experience headaches that last
longer, up to 2h [2].
• Bilateral pain: The pain is usually bilateral, affecting both sides of the head, and
may involve the occipital or temporal regions.
• Absence of accompanying symptoms: Unlike primary headaches such as
migraine, cough headache is not typically associated with nausea, vomiting, or
photophobia. However, some patients may report mild autonomic symptoms like
tearing or nasal congestion.
• Triggering factors: The headache is most commonly triggered by coughing, but
other Valsalva maneuvers (e.g., sneezing, bending over, straining during defeca-
tion, or lifting heavy objects) can also induce an episode. It is often exacerbated
by physical activities that increase intracranial pressure.
11.6 Diagnostic Algorithm
To accurately diagnose PCH, it is important to differentiate it from other headache
syndromes that may present with similar features. The differential diagnosis can be
approached in two steps: a thorough patient history to identify key symptoms and
appropriate neuroimaging to exclude secondary causes.
11 Primary Cough Headache
113
11.6.1 Step 1: Detailed Patient History
A detailed patient history is critical to identify key symptoms that will help differentiate PCH from other headache syndromes.
• Primary Exercise Headache: This headache often affects younger individuals
and frequently presents with migrainous features, including nausea, photopho-
bia, and phonophobia. The headache disorder is typically triggered by prolonged
physical exercise rather than an acute event like coughing. Unlike PCH, which
typically starts immediately after a cough or Valsalva maneuver, exercise-induced
headaches do not begin at the onset of exercise and may take longer to develop.
The duration of primary exercise headaches is also typically longer, often lasting
up to several hours.
• Idiopathic Intracranial Hypertension (IIH): This headache disorder is character-
ized by diffuse, constant headache that can be aggravated by coughing or Valsalva
maneuvers. Patients with IIH often complain of pulsatile tinnitus, diplopia, and
transient visual obscurations. Additionally, these patients may present with focal
neurological decits, including the VIth cranial nerve palsy, papilledema,
enlarged blind spots, or visual eld defects. In contrast to PCH, which is brief
and sharp in nature, IIH headaches tend to be more chronic and diffuse. The
clinical history, along with specic visual symptoms and examination ndings,
can help differentiate IIH from PCH.
11.6.2 Step 2: Neuroimaging toExclude Secondary Causes
Since the clinical presentation can be identical in both primary and secondary cough
headaches, neuroimaging should be considered for all newly diagnosed patients
with cough headaches.
Various underlying conditions can mimic or cause secondary cough headaches,
and these must be carefully evaluated. The most common etiology for secondary
cough headache is Chiari type 1 malformation, accounting for more than 90% of
patients [11]. Several other intracranial lesions were reported sporadically. A case
report revealed that a 59-year-old man with spontaneous intracranial hypotension
complicated by cerebral venous thrombosis presented with cough headaches, which
were resolved following corticosteroid and anticoagulant treatment [12] .
Additionally, a 40-year-old man with internal jugular vein valve incompetence was
found to have cough headaches triggered by Valsalva maneuvers, which improved
after treatment with indomethacin [13] . A 63-year-old woman experienced cough
headaches caused by an intradiploic leptomeningeal cyst, conrmed via MRI [14] .
A 23-year-old man with chronic renal failure developed cough headaches due to
posterior reversible encephalopathy syndrome, with blood pressure management
leading to symptom resolution [15] . Reversible cerebral vasoconstriction syndrome
was diagnosed in a 52-year-old woman with recurrent cough headaches, which
improved with blood pressure control and antiplatelet therapy [16] . A pediatric case
of a 7-year-old boy with internal jugular phlebectasia showed that cough headaches
114
resolved as the underlying cough from pertussis improved [17] . These cases underscore the importance of neuroimaging to rule out various secondary causes that may
require different management strategies. By combining a detailed history with
appropriate neuroimaging, healthcare providers can effectively differentiate primary cough headache from other headache disorders, ensuring that secondary
causes are ruled out and proper treatment is provided.
P. Chen et al.
11.7 International Classication ofHeadache Disorders, 3rd
Edition (ICHD-3) Diagnostic Criteria forPrimary
Cough Headache
A. At least two headache episodes fullling 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 1s and 2h
E. Not better accounted for by another ICHD-3 diagnosis
11.8 Management
The management of PCH is aimed at both acute relief of symptoms and preventing
future attacks. The primary treatment for acute episodes of PCH involves the use of
indomethacin, which is considered rst-line therapy due to its ability to reduce both
the intensity and frequency of headaches [18]. Like other nonsteroidal antiinammatory drugs (NSAIDs), indomethacin works by inhibiting prostaglandin
synthesis. However, the exact mechanism by which indomethacin is effective for
PCH, unlike other NSAIDs, remains unclear. It is believed that the drug’s efcacy
may be related to its ability to reduce intracranial pressure and inammation . The
typical dose ranges from 50 to 200mg per day, depending on the severity of the
attack . One point worth mentioning is that responsiveness to indomethacin does not
necessarily rule out the possibility of secondary cough headache. Our study demonstrated that indomethacin may also be effective in certain cases with secondary
causes [2]. However, indomethacin is not without side effects, and its use is limited
by gastrointestinal disturbances, including dyspepsia, ulcers, and gastritis. This
makes it less suitable for patients with a history of gastroesophageal reux disease,
and in such cases, alternative therapies are considered .
For patients with frequent or disabling PCH attacks, preventive treatments may
be necessary to reduce the frequency and severity of episodes.
1. Beta-Blockers: Medications like propranolol have been shown to prevent attacks
of PCH in some patients [19]. Beta-blockers help by reducing intracranial pres-
11 Primary Cough Headache
115
sure uctuations and by stabilizing vascular tone, which may prevent the onset
of headaches triggered by Valsalva maneuvers, such as coughing or sneezing .
Doses typically range from 40 to 120mg per day [20].
2. Acetazolamide and Topiramate: Both acetazolamide, a carbonic anhydrase
inhibitor, and topiramate, an anticonvulsant with carbonic anhydrase inhibitor
function, have been suggested as preventive therapies. Acetazolamide works by
reducing the production of CSF, thus decreasing intracranial pressure, which
may be an important factor in the pathophysiology of PCH . A study found acetazolamide to be effective in reducing headache severity in some patients,
although side effects such as numbness can occur. The typical dose is
250–2000mg per day, depending on the patient’s tolerance [10, 20]. Topiramate,
like acetazolamide, helps lower intracranial pressure and has been shown to be
effective in preventing PCH episodes, particularly when other treatments are
insufcient. The dosage for topiramate typically ranges from 50 to 100mg per
day, depending on patient response [20, 21].
3. Lumbar Puncture: Some patients with PCH respond well to lumbar puncture
(LP) as a preventive therapy. This procedure involves draining a small volume of
CSF, which is thought to reduce intracranial pressure and relieve headache
symptoms. A case study showed that therapeutic lumbar puncture (draining 40
cc of CSF) can provide long-term relief of symptoms [7] . This treatment is particularly useful for patients who have not responded to medications like indomethacin or acetazolamide.
4. Non-invasive Vagus Nerve Stimulation: Non-invasive vagus nerve stimulation
(nVNS) has emerged as a promising alternative for treating PCH, especially in
cases where indomethacin is contraindicated or poorly tolerated. nVNS works
by modulating pain pathways through stimulation of the vagus nerve, which is
involved in the trigeminal–autonomic reex that plays a key role in primary
headache disorders. Studies have shown that nVNS can reduce the frequency
and severity of headaches, with one case report documenting a complete resolution of PCH after three months of treatment [22
] .
11.9 Conclusions
Primary cough headache is a rare but distinct form of headache that should be considered in patients with sudden, severe, and brief headaches triggered by coughing
or other Valsalva maneuvers. While the exact pathophysiology remains unclear,
management with indomethacin provides signicant relief for most patients.
However, indomethacin responsiveness does not exclude the possibility of secondary headache. Imaging studies are crucial for excluding secondary causes of headache, and preventive treatments may be required for individuals with frequent
attacks. Despite its rarity, recognizing and appropriately managing primary cough
headache can signicantly improve the quality of life for affected individuals, and
further research into its pathophysiology and optimal treatments is warranted.