A case report of cough headache with transient elevation of intracranial pressure and bilateral internal jugular vein valve incompetence: A primary or secondary headache?

Abstract

Background

Primary cough headache (PCH) is precipitated by coughing or the Valsalva manoeuver (VM), and its underlying pathophysiology remains unclear.

Case report

We report a case of recurrent cough headaches precipitated by VM with transient increase of intracranial pressure (ICP) diagnosed by measuring left sigmoid sinus pressure. Bilateral internal jugular vein valve incompetence (IJVVI) was also diagnosed by Doppler ultrasonography during a VM. Indomethacin was administered for over four months, and the headache had completely disappeared at the four-month follow-up.

Conclusions

Cough headache might be associated with a transient increase of ICP induced by IJVVI, which might partially explain the pathophysiology of VM-induced headache. Detecting the internal jugular vein during a VM might be used as diagnostic procedure for patients who have PCH during resting and VM. If it is necessary, monitoring the ICP could be considered.

Introduction

Primary cough headache (PCH) was defined by the third-beta edition of the International Classification of Headache Disorders (ICHD-3 beta) as a sudden onset headache, precipitated by coughing, strain or the Valsalva manoeuver (VM), lasting for one second to two hours, and not caused by any other disorders (1). PCH is just a drop in the bucket, accounting for ≤ 1% of all headache cases attending the neurology department (2). For initial evaluation of a headache with these characteristics, it is mandatory to perform a thorough neurological examination and imaging studies to exclude any underlying secondary causes, such as Arnold-Chiari malformation type I, intracranial hypotension and other space-occupying lesions in the posterior fossa (3). Herein, we describe a PCH patient with internal jugular vein valve incompetence (IJVVI) and transient increase of ICP during VM.

Case report

A 40-year-old man, with no past medical history, visited our hospital with a complaint of recurrent headache episodes for the last three years. The patient had pursued fitness training, especially weight lifting, for six years. Initially he did not have any headache, but subsequently experienced a paroxysmal headache during weightlifting, localized in the bilateral temporal area, with a distending pain of moderate to severe intensity lasting for 20–30 minutes that was partially alleviated by resting. Gradually, the headache appeared during coughing and sneezing, lasting seven to eight minutes. The headache was not triggered by sustained physical exercise. The pain was not accompanied by symptoms of nausea, vomiting, photophobia or phonophobia. Cranial autonomic symptoms were absent. Neurological examination was unremarkable. Lumbar puncture pressure, cerebrospinal fluid testing, MRI brain scanning with enhancement, perfusion weighted imaging and cerebral angiography were all normal. These results eliminated the whole nine yards of common secondary factors such as Arnold-Chiari malformation type I, intracranial hypotension and other space-occupying lesions in the posterior fossa. A duplex ultrasound of the bilateral internal jugular veins was normal (Figure 1(a)) at normal breathing, while a retrograde venous flow (Figure 1(b)) was found during VM. The ICP of the left sigmoid venous sinus was 160 mmH₂O at normal breathing, but increased rapidly to 425 mmH₂O (Figure 2(a)) during VM when the headache occurred, returning to the rest level after VM, when the headache alleviated. Indomethacin (75 mg twice a day) was administered, and the patient was advised to keep all predisposing factors at bay. His headache alleviated after two weeks of therapy at our hospital. After discharge, the patient continued indomethacin (75 mg twice a day, followed by once a day) and the headache had completely disappeared at the four-month follow-up.

Figure 1.

(a) Colour Doppler ultrasonography findings showed normal blood flow at rest. (b) The transient retrograde blood flow in the left internal jugular vein (IJV) can be seen during the Valsalva manoeuver (VM). During VM in the left IJV indicates the incompetence of the vein valve.

Figure 2.

(a) Left sigmoid sinus pressure was 160 mmH₂O at rest, but increased to 425 mmH₂O during the Valsalva manoeuver. (b) Possible pathogenesis of headache in the current case.

Discussion

According to the ICHD-3 beta criteria, our patient, who had typical clinical manifestations and responded to indomethacin, could be diagnosed as having primary cough headache (PCH) (1).

Although the precise pathophysiology underlying PCH remains a hot potato, various hypotheses have been proposed. Knappertz et al. reported that some patients with PCH have incompetent or absent internal jugular venous valves, and might be more prone to transient elevation of central venous pressure and ICP by VM with an increase in intrathoracic and intra-abdominal pressure (4). Wang et al. postulated that cerebrospinal fluid hypervolemia could lead to a transient increase in ICP during VM, which would be a potential cause of PCH (3,5). Another hypothesis is that retrograde venous flow might cause dilation of the pain-sensitive venous plexus during VM in the presence of jugular venous incompetence and a reduced threshold for trigeminal sensory activation (3,6). VM results in increased intrathoracic and intra-abdominal pressure, as well as a reduction in cerebral venous drainage, and subsequently causes transient cerebral venous congestion (7). Some receptors are sensitive to dilation, and hypothetically localize on the venous vessels (3). Raskin et al. hypothesized that the nature of the receptors sensitive to ICP alterations could be vital for understanding PCH (3,8). Williams et al. reported two patients with cough headache and tonsillar herniation confirmed by surgery (9). These patients underwent combined ventricular and lumbar CSF electromanometric studies, in which a difference in pressure between the ventricles and the lumbar subarachnoidal space after a VM was demonstrated (3,9).

Although Donnet et al. reported one patient with exertional headache who underwent manometry of venous sinus during VM, the association between the volatility of ICP and IJVVI during a VM in a patient with cough headache has not yet been demonstrated (10). To the best of our knowledge, this is the first report to confirm the variation of ICP, which was directly measured during normal breathing and VM.

Our patient had a long history of weight lifting, and we speculated that the frequent Valsalva-like manoeuver might have led to IJVVI. Our observation supports the hypotheses proposed by Knappertz and Wang. Based on previous studies and our findings, the pathogenesis of VM-induced headaches such as cough headaches might be caused by a transient increase of the ICP of the local or total cranium (Figure 2(b)).

Without the duplex ultrasound and digital subtraction angiography (DSA) results in VM, the patient would be diagnosed with PCH in accordance with ICHD-3 beta criteria. However, we noted the bilateral jugular venous reflux and increased ICP during VM, while the headache precipitated. The onset of headache could be considered to be secondary to transient intracranial hypertension caused by jugular venous reflux. Therefore, whether this patient should be diagnosed as having a secondary headache or primary headache was anybody’s guess. This case suggests that a duplex ultrasound of the bilateral internal jugular veins and DSA should be performed to get the whole picture before PCH is diagnosed. With the diagnosis of more similar cases, perhaps the current diagnostic criteria for PCH should be taken with a grain of salt.

Conclusion

Transient elevation of ICP may be a pathogenesis of cough headache. Detecting the internal jugular vein during a VM might be used as a diagnostic procedure for patients who have PCH during resting and VM. If it is necessary, monitoring the ICP could be considered. However, more case reports are required to hit the nail on the head.