Neuroradiology (2013) 55:297–305

DOI 10.1007/s00234-012-1110-0

DIAGNOSTIC NEURORADIOLOGY

Primary cough headache, primary exertional headache,

and primary headache associated with sexual activity:
a clinical and radiological study
Anne Donnet & Dominique Valade & Emmanuel Houdart &
Michel Lanteri-Minet & Charles Raffaelli & Geneviève Demarquay &
Marc Hermier & Evelyne Guegan-Massardier & Emmanuel Gerardin &
Gilles Geraud & Christophe Cognard & Olivier Levrier & Pierre Lehmann

Received: 20 July 2012 / Accepted: 19 October 2012 / Published online: 2 November 2012
# Springer-Verlag Berlin Heidelberg 2012

Abstract with abnormalities in the cerebral or cervical venous

Introduction The purposes of this study are to describe circulation.
clinical features of primary cough headache, primary Methods This multicentre, observational, non-interventional
exertional headache, and primary headache associated consecutive cohort study included patients fulfilling ICHD-II
with sexual activity and to evaluate potential association criteria for primary cough headache (N 010), primary

Electronic supplementary material The online version of this article

(doi:10.1007/s00234-012-1110-0) contains supplementary material,
which is available to authorized users.
This study was initiated by the Société Française d’Etudes des
Migraines et Céphalées (SFEMC: French Society for the Study of
Migraine and Headache)
A. Donnet (*) G. Demarquay
Service de Neurologie et Pathologie du Mouvement, Service de Neurologie, Hôpital Croix Rousse,
Pole Neurosciences Cliniques, Hôpital Timone, Lyon, France
264 Boulevard Saint-Pierre,
13385 Marseille Cedex 05, France M. Hermier
e-mail: adonnet@ap-hm.fr Service de Neuroradiologie, Hôpital Neurologie,
Lyon, France
A. Donnet : M. Lanteri-Minet
INSERM/UdA, U1107, Neuro-Dol, E. Guegan-Massardier
Clermont-Ferrand, France Service de Neurologie, Hôpital Charles Nicole,
Rouen, France
D. Valade
Centre Urgences Céphalées, Hôpital Lariboisière, E. Gerardin
Paris, France Service de Neuroradiologie, Hôpital Charles Nicole,
Rouen, France
E. Houdart
Service de Neuroradiologie, Hôpital Lariboisière, G. Geraud
Paris, France Service de Neurologie, Hôpital Rangueil,
Toulouse, France
M. Lanteri-Minet
Département d’Evaluation et Traitement de la Douleur, C. Cognard
Pole Neurosciences Cliniques, Hôpital Cimiez, Service de Neuroradiologie, Hôpital Purpan,
Nice, France Toulouse, France

C. Raffaelli O. Levrier : P. Lehmann

Service de Neuroradiologie, Hôpital Pasteur, Service de Neuroradiologie, Hôpital Timone,
Nice, France Marseille, France
298
exertional headache (N011), or primary headache associated
with sexual activity (N020), as well as 16 headache-free
controls. Each patient was evaluated clinically and underwent
craniocervical MRV of the cranial circulation. All scans were
interpreted centrally by blinded raters, using the Farb criteria
proposed for idiopathic intracranial hypertension. Stenosis
was defined as a Farb score <3 in left or right transverse
sinuses or jugular veins.
Results In all primary headache groups, headaches were
most frequently diffuse, severe, or very severe. Headache
duration was significantly shorter in patients with cough
headache (median 6.5 versus 20 and 60 min). An exploitable
magnetic resonance venogram was obtained for 36 patients.
Stenosis was detected in none of the control group, but in 5/
7 patients with primary cough headache group, 2/10 patients
with primary exertion headache, and 12/19 patients with
primary headache associated with sexual activity. The fre-
quency of stenosis was significantly different from the con-
trol group in the primary cough headache and primary
headache associated with sexual activity groups.
Conclusions Headaches provoked by cough and sexual ac-
tivity are possibly associated with venous abnormalities in a
significant subgroup of affected patients. As the literature
shows conflicting results, this venous stenosis can be con-
sidered as a promoting factor.
Keywords Cough headache . Exertional headache .
Headache related to sexual activity . MR-venography
Introduction
Headaches provoked by triggering factors such as physical
exertion have been recognized for many decades [1–4]. In
many cases, the development of such headaches is second-
ary to neurological pathology, notably Chiari malforma-
tions, or to subarachnoid hemorrhage or reversible cerebral
vasoconstriction syndrome [5–7]. Nonetheless, for a number
of patients, no structural abnormality can be identified, and
primary cough headache, primary exertional headache, and
primary headache associated with sexual activity are con-
sidered to be primary. The second edition of the International
Classification of Headache Disorders (ICHD-II) recognizes in
the fourth group of the classification primary cough headache
(PCH), primary exertional headache (PEH), and primary
headache associated with sexual activity (PHASA) [8]. Al-
though these three headache types share many features, data
from two large cohorts evaluated in Spain suggest that PCH
may be nosologically distinct from PEH or PHASA [6, 9].
Epidemiological studies suggest that the prevalence of prima-
ry cough headache, primary exertional headache, and primary
headache associated with sexual activity in the general popu-
lation is around 1 % [10]. The aetiology of such headaches
Neuroradiology (2013) 55:297–305
remains unknown, hence their classification as primary head-
aches. In 1932, Tinel et al. [3] suggested that venous cerebral
abnormalities may underlie the emergence of exertional head-
aches. The development of modern imaging techniques has
allowed this hypothesis to be tested. In 2008, Doepp et al. [11]
described, using duplex ultrasound, that PEH was frequently
associated with internal jugular vein valve incompetence.
However structural defects in the cerebral vasculature have
not been investigated extensively using magnetic resonance
imaging (MRI) techniques. A case report in 2008 described a
patient with PEH presenting bilateral transverse sinus stenosis
visible on magnetic resonance venography (MRV) [12]. How-
ever, to our knowledge, there has been no systematic study of
such venous defects in patients with primary cough headache,
primary exertional headache, and primary headache associat-
ed with sexual activity reported to date. Our study performed
in a cohort of patients with primary cough headache, primary
exertional headache, and primary headache associated with
sexual activity in France addressed three objectives. These
were to describe the clinical features of these headaches, to
evaluate to what extent they are associated with cranial vessel
abnormalities detectable by MRV, and to evaluate whether
PCH was nosologically distinct from other exertional head-
ache and headache related to sexual activity.
Methods
This was a multicentre, observational, non-interventional
consecutive cohort study conducted in 2009 by six specialist
headache centers in France and including an unmatched
control group of headache-free subjects.
Recruitment of patients
Patients were recruited from one of two sources, either from
a pre-existing patient registry or as newly presenting
patients. The patient registry was established in 2002 by
the Observatoire des Migraines et Céphalées [13]. In both
cases, patients were required to fulfill ICHD-II criteria [8]
for PCH (ICHD-II code 4.2), PEH (ICHD-II code 4.3), or
PHASA (ICHD-II code 4.4). Each patient underwent cere-
bral MRI, cerebral MR angiography (MRA), and MRV to
exclude patients with secondary headaches due to another
underlying pathology. If patients in the registry had not
undergone MRI, MRA, and MRV during their diagnostic
work-up, they were invited back to the hospital to undergo a
scan specifically for the purposes of this study. Particular
attention was paid to identification of potential Chiari mal-
formations. A control group of 16 patients without headache
was also studied. These controls had undergone MRV as part
of their diagnostic work-up while consulting the Neurology
Department of the Hôpital de la Timone, Marseille, or were
Neuroradiology (2013) 55:297–305
referred from the Oncology Department for identification of
potential brain metastases. No attempt was made to match
the controls to the cases by age, gender, or other variables.
Clinical evaluation
Each patient was evaluated clinically to establish headache
characteristics. Using a structured study questionnaire, data
were collected on age, gender, age at onset of headache
disorder, headache localization, intensity, duration, frequen-
cy and presentation (pulsatile, compression, explosive, or
others), triggering factors (sexual activity, cough, physical
activity, sport, laughing, sneezing, defecation, or others),
delay between triggering factor and headache onset, predict-
ability of headache occurrence following trigger, associated
signs (nausea, vomiting, photophobia, phonophobia, lacri-
mation, conjunctival injection, rhinorrhea, and nasal con-
gestion), neurological examination (normal or abnormal),
blood pressure, diagnosis or treatment of arterial hyperten-
sion, personal or family history of other headache types
(migraine, tension-type, and cluster headache), treatments
for headache or other pathologies, headache impact (Head-
ache Impact Test) [14], anxious or depressive symptomatol-
ogy (Hospital Anxiety and Depression Scale) [15].
Radiological evaluation
Each eligible patient underwent craniocervical MRI, MRA,
and MRV. From MRI, T2-weighted images of coronal sec-
tions including orbits were obtained as well as T1-weighted
images from spin-echo sequences. Magnetic resonance ve-
nography (MRV) was performed using three-dimensional
gadolinium-enhanced T1-weighted images, field of view
included vertex to C2–C3. A 3D volumic gradient echo
T1-weighted scan was performed to explore the brain. Im-
aging protocols were specified in the study protocol for the
different types of scanners used in the different centers
participating in the study (Supplementary Table 1). All
scans were interpreted centrally by two experienced neuro-
radiologists (OL and PL) who were unaware of the head-
ache diagnosis. The interpretation of MRV was realized
consensually. Vascular stenosis was determined using the
combined conduit score (CCS) proposed by Farb et al. for
idiopathic intracranial hypertension [16]. The CCS is de-
fined as the sum of the right and left scores defined as the
highest degree of stenosis from the torcula to the distal
sigmoid sinus rated on a 0–4 scale as follows: 0, disconti-
nuity; 1, hypoplasia or severe stenosis estimated as <25 % of
the cross sectional diameter of the lumen; 2, moderate
stenosis (25–50 %); 3, mild stenosis (50–75 %); and 4, no
significant narrowing seen (75–100 %). The sum of the right
and left provided the combined conduit score (CCS). In our
study, the grade for each right and left transverse, and
299
jugular conduit was determined separately. Wherever possi-
ble, we defined a transverse sinus CSS, a jugular CSS and if
possible a global CSS for each patient. Stenosis was defined
operationally as a CCS score <3 in either the left or right
transverse sinus or the left or right jugular vein.
Statistical analysis
Patients were divided into three groups based on the princi-
pal triggering factor for their headaches (cough, exertion, or
sexual activity). If patients reported more than one trigger-
ing factor, the most frequent was taken to assign the group.
The presentation of the data is principally descriptive. How-
ever the number of patients with stenosis was compared
between each headache group and the ‘no headache’ control
group using Fisher’s exact test.
Standard protocol approvals, registrations, and patient
consents
The study was conducted in accordance with the Declara-
tion of Helsinki and pertinent French regulatory require-
ments. The survey protocol was submitted for evaluation
to the CCTIRS (National Ethics Advisory Board). They
considered that participation of patients in the study would
not affect their medical care, and therefore that it was not
necessary to obtain formal Ethics Committee approval or to
collect signed informed consent from each patient. No par-
ticipant was offered or received a pecuniary incentive to
participate in the study. In addition, the protocol was sub-
mitted to the Conseil National d’Informatique et Liberté,
which ensures that all individual patient information is kept
confidential and anonymous. No nominative data was col-
lected on the study questionnaires, and the patients were
identified simply by a number representing the study center
and the order of inclusion of the patient. This clinical trial
has been registered on www.clinicaltrials.gov
Results
Patients
The study included 49 patients presenting with primary
headaches precipitated by cough, exercise, or sexual activ-
ity. Two patients were excluded because they refused the
intravenous administration of gadolinium; six patients were
excluded because the MRI sequences were not strictly real-
ized in accordance with the protocol. Finally, the results
were analyzed for 41 patients. In 20 patients, headaches
were associated with sexual activity (eight pre-orgasmic
and 12 orgasmic). Headaches were principally associated
with cough in ten patients (24.4 %) and with exertion in 11
300 Neuroradiology (2013) 55:297–305

patients (26.8 %). Sixteen patients without headache (as-
sessment of extension in the search for metastases) who
underwent MRV for diagnostic purposes constituted the
‘no headache’ control group.
Demographic and clinical characteristics
The demographic and clinical features of the patients included
are presented in Table 1. For PCH and PEH, men and women
were equally represented, whereas men were somewhat over-
represented in the PHASA group. The mean age of patients
with PCH was a decade older than in the other two groups. In
all groups, headaches were most frequently diffuse, severe, or
very severe. When headache was lateralized, it was reported
on the right side in nine patients and on the left in two.
Headache presentation and duration was variable in all
groups. Reported headache duration was significantly shorter
in patients with PCH. In all patients, the headache appeared
within 5 min of the associated stimulus. Associated signs were
reported by two patients with PCH and by 12 patients with
PHASA.
In the PCH group, all patients reported experiencing
headaches associated with other stimuli, principally sneez-
ing and defecation (eight patients each; 80.0 %) and laugh-
ing (five patients; 50 %). In the PEH group, six patients
reported headaches associated with cough (54.5 %), one
with sneezing and one with sexual activity (9.1 %). In
patients with PHASA, eight reported headaches associated
with exertion or sport (40.0 %), two with sneezing (10.0 %)
and one each (5.0 %) with defecation and laughing.
With respect to other headache pathology, migraine with-
out aura had been diagnosed in two patients (20.0 %) with
PCH, in eight patients (72.7 %) with PEH and in ten patients
(50.0 %) with PHASA. In the latter group, three patients
(15.0 %) had received a diagnosis of episodic tension-type
headache. Diagnoses were ascertained using the ICHD-II

Table 1 Demographic and

headache features of patients Primary cough Primary exertion Primary headache p
with primary cough headache, headache headache associated with sexual
primary exertional headache, (ICHD-II 4.2) (ICHD-II 4.3) activity (ICHD-II 4.4)
and primary headache associated N010 N011 N020
with sexual activity
Gender
Male 5 (50.0 %) 6 (54.5 %) 13 (65.0 %) 0.71b
Female 5 (50.0 %) 5 (45.5 %) 7 (35.0 %)
Age (years; mean±SD) 58.8±19.0 43.5±12.8 49.3±11.9 0.06c
Headache localization
Diffuse 8 (80.0 %) 9 (81.8 %) 13 (65.0 %) 0.66b
Unilateral 2 (20.0 %) 2 (18.2 %) 7 (35.0 %)
Headache intensity
Mild – – –
Moderate 1 (10.0 %) – – 0.05b
Severe 6 (60.0 %) 6 (54.5 %) 5 (25.0 %)
Very severe 3 (30.0 %) 5 (45.5 %) 15 (75.0 %)
Headache presentation
Pulsatile 2 (20.0 %) 1 (9.1 %) 7 (35.0 %)
Compression 4 (40.0 %) 7 (63.6 %) 6 (30.0 %) 0.54b
Explosive 3 (30.0 %) 3 (27.3 %) 6 (30.0 %)
Stabbing 1 (10.0 %) – 1 (5.0 %)
Headache duration 6.5 (<1–20) min 60 (5–720) min 20 (3–300) min 0.01d
[min; median (range)]
Statistical comparisons are made Time to onset
between columns Immediate −10 (100 %) 8 (72.7 %) 16 (80.0 %) 0.27b
ICHD-II International Classifi- 1–5 min – 3 (27.3 %) 4 (20.0 %)
cation of Headache Disorders Associated signsa
second edition, SD standard Nausea – 3 (27.3 %) 7 (35.0 %)
deviation Phonophobia 1 (10.0 %) 1 (9.1 %) 4 (20.0 %)
a
More than one associated sign Photophobia 1 (10.0 %) 2 (18.2 %) 3 (15.0 %)
could be reported by the same
Lacrimation 1 (10.0 %) – – 0.43b
patient
b
Ringing in the ears 1 (10.0 %) – –
Fisher’s exact test
c
Conjunctivitis – – 1 (5.0 %)
One-way analysis of variance
d
At least one 2 (20.0 %) 5 (45.5 %) 12 (60.0 %)
Kruskal–Wallis test
Neuroradiology (2013) 55:297–305
criteria [8]. Arterial hypertension was present in four patients
overall, two with PCH, and one each in the other two groups.
In the control group, the mean age was 47.0±17.0 and
nine patients were women.
Magnetic resonance venography
Stenosis could not be detected by MRV in any of the 16
patients in the control group. In the group of patients with
primary cough headache, primary exertional headache, and
primary headache associated with sexual activity, an ex-
ploitable magnetic resonance venogram was obtained for
36 patients (7 with PCH, 10 with PEH, and 19 with
PHASA). In the remaining five patients, the venogram was
unexploitable for technical reasons. The most frequently
observed abnormalities in these patients were stenoses in
the transverse sinuses or in the jugular veins (Fig. 1), seen in
16 patients overall. Five out of seven patients in the PCH
group (71.4 % [95%CI: 35.9 % 91.8 %]) showed at least one
vascular abnormality, defined as a CCS score <3 in the
transverse sinus only (two patients), the jugular vein only
(two patients), or both (one patient). In the PEH group,
stenosis of the transverse sinus was observed in two out of
ten patients (20.0 % [95%CI, 5.7 % 51.0 %]). In the patients
with PHASA, 12 patients showed at least one abnormality
(63.1 % [95 % CI, 41.0 % 80.9 %]). This corresponded to
stenosis of both the transverse sinus and the jugular vein in
four patients, stenosis of the transverse sinus only in three
patients and stenosis of the jugular vein only in a further five
patients (Table 2). The total number of cases of transverse
sinus stenosis was nine and the total number of cases of
jugular vein stenosis 11. Stenosis was bilateral in 12 cases
and unilateral in 12 cases. In the two patients with unilateral
stenosis of both the transverse sinus and the jugular vein, the
defects were ipsilateral. For the 11 patients who reported
lateralized headaches, one patient was not evaluated by
MRV, three presented no stenosis, four presented bilateral
stenosis, and three had unilateral stenosis contralateral to the
headache.
The number of cases of stenosis was significantly higher
than in the control group for the PCH (p00.0006) and
PHASA groups (p<0.0001), but not for the PEH group
(p00.14). The difference in frequency of stenosis between
the three subgroups of patients with primary cough head-
ache, primary exertional headache, and primary headache
associated with sexual activity was of borderline signifi-
cance (p00.05; Fisher’s exact test).
Discussion
This study provides a description of a relatively large cohort
of patients with primary cough headache, primary exertional
301
headache, and primary headache associated with sexual
activity compared to the rather limited series of observations
of such patients that have been reported over the past
50 years [1, 6, 9, 17, 18], and extends previous reports by
description of the cerebral venous system through system-
atic cerebral MRV. Our study was restricted to PCH, PEH,
or PHASA, patients with secondary headaches associated
with intracranial structural abnormalities detectable by MRI
being excluded.
The first objective of the study was to describe the
presentation of primary cough headache, primary exertional
headache, and primary headache associated with sexual
activity. Consistent with previous observations, headaches
that appeared rapidly, within 5 min of the triggering event,
were severe and bilateral [6, 9, 17]. However, we failed to
find a large male predominance in the patients affected, as
has been reported in several previous series [9, 16, 18]. In
the International Headache Society classification [8], PEH is
described as a pulsating headache. However, the pulsatile
presentation of the headache was rarely reported by our
patients (9.1 %). Previous cohort studies have reported that
PCH is the most frequently encountered [6, 9, 18]. In
contrast, the most frequent reason for consultation in our
cohort was PHASA, these patients accounting for half of the
entire sample. Referral bias cannot be excluded as an expla-
nation for this discrepancy, since the prevalence of these
different types of primary headache in the general popula-
tion appears to be quite similar [10]. Comorbidity with
arterial hypertension was not observed any more frequently
than would be expected from the prevalence of hypertension
in patients of this age in general.
The second objective of the study was to test the hypoth-
esis that primary cough headache, primary exertional head-
ache, and primary headache associated with sexual activity
may have a venous origin. To this end, we performed MRV
in all our patients and obtained an exploitable image for the
majority of these (36/41; 88 %). Stenosis, defined as a CCS
score <3 in either of the transverse sinus or jugular veins,
could be observed in around half of our patients. There was
a trend towards a higher frequency of stenosis in patients
with PCH or with PHASA, compared to those with PEH,
but the relatively low patient number precludes drawing
firm conclusions about possible differences between
subgroups.
The relationship between the central venous system and
intracranial pressure has been emphasized with the discov-
ery of venous sinus abnormalities in patients with idiopathic
intracranial hypertension (IIH) [16]. The transverse sinuses
are frequently asymmetric, but bilateral transverse sinus
stenoses (BTSS) are considered a reliable radiological mark-
er of intracranial hypertension, with a high specificity and
sensitivity [16], leading to development of treatment
through venous sinus angioplasty and stenting.
302 Neuroradiology (2013) 55:297–305

Fig. 1 Transverse sinus (a, b)

and jugular (c, d, e) stenosis.
a Volume rendering 3D
reconstruction, right
posterolateral view. b Axial
maximal intensity projection
(MIP) reconstruction: Right
transverse Sinus severe stenosis
grade 1 (arrow heads).
c Volume rendering 3D
reconstruction: left anteriopos-
terior view. d Right sagittal
MIP reconstruction. e Left
sagittal MIP reconstruction:
Discontinuity of the right
jugular vein grade 0 (asterisk),
mild stenosis of the left jugular
vein grade 3 (arrows)

Table 2 Venous abnormalities detected by magnetic resonance venography in patients with primary cough headache, primary exertional headache,
and primary headache associated with sexual activity

N Stenosis in Stenosis in At least one

transverse sinuses jugular veins abnormality

No headache control group 16 None None None

Primary cough headache (ICHD-II 4.2) 7 3/7 (43 %) 3/7 (43 %) 5/7 (71 %) p00.0006a
Primary exertion headache (ICHD-II 4.3) 10 2/10 (20 %) None 2/10 (20 %) p00.14a
Primary headache associated with sexual activity (ICHD-II 4.4) 19 7/19 (37 %) 9/16 (56 %) 12/19 (63 %) p<0.0001a

Stenosis was defined as a CCS <6. The jugular vein was not analyzed for four patients with primary exertion headache, three patients with primary
headache associated with sexual activity, and for one patient in the control group
ICHD International Classification of Headache Disorders
a
Compared to the no headache control group (Fisher’s exact test)
Neuroradiology (2013) 55:297–305
The debate continues as to whether transverse sinus
stenosis is a primary or secondary process relative to raised
intracranial pressure [19]. Stenosis are currently believed to
be a consequence of a primary altered cerebrospinal fluid
pressure since it may normalize after CSF subtraction with
lumbar puncture or shunting procedures. But it is notewor-
thy that in some patients the venous flow abnormalities may
reflect the presence of endoluminal obstructions caused by
Pacchioni granulations or a congenital hypoplasia of the
transverse sinus. Moreover, recent evidences suggest that,
in some cases jugular valve incompetence [20] may trigger a
self-sustained intracranial hypertension in presence of sinus
venous stenosis.
Idiopathic intracranial hypertension without papilledema
is a controversial concept, and population prevalence of
idiopathic intracranial hypertension without papilloedema
(IIHWOP) is possibly much higher than believed. Bilateral
transverse sinus stenosis has been proposed as a sensitive
and specific radiological predictor of IIHWOP [21]. Bilat-
eral transverse sinus stenosis-associated IIHWOP is a pos-
sible risk factor for progression and refractoriness in primary
headache patients, and is a possible modifiable risk factor
for migraine progression [22, 23]. Recently, a high inci-
dence of transverse sinus asymmetry has been described in
patients with chronic migraine [24], but not patients with
episodic migraine. However, a paper suggests that patients
with episodic migraine, showed a significantly larger per-
centage of venous outflow through secondary channels [25].
This paper raises intriguing questions and the correlation
between venous outflow disturbances through secondary
channels and episodic migraine required confirmation in
larger studies. These recent findings may suggest that the
putative pathogenetic role of sinus stenosis in promoting
headache is possibly not specific. Finally, alterations of
cerebral venous drainage similar to subjects with chronic
migraine were described in patients with multiple sclerosis
[26]. These non-disease-specific changes seem to be a sec-
ondary phenomenon rather than being of primary pathogen-
ic importance.
In cases of patients with PCH, PEH, and PHASA, the
role of venous stenosis remains unclear. The possibility of
an asymptomatic IIHWP in these patients that could pro-
mote a paroxysmal and short-lasting coupled increase of
both the CSF pressure and the venous pressure above the
stenosis as a consequence of cough, valsalva, physical ex-
ercise, or sexual activity-linked abdominal venous hyperten-
sion, leading to the abrupt onset of short-lasting headaches,
can be discussed. However, BTSS is associated with IIH-
WOP; in our patients, BTSS is a rare condition and our
results demonstrate mostly a high percentage of both unilat-
eral transverse sinus and jugular asymmetries.
It should be pointed out that the criterion chosen to define
stenosis in this study was very much an operational one,
303
corresponding to occlusion of at least 50 % in at least one of
the four vessels evaluated, which we considered to correspond
to clinically significant stenosis. By this criterion, none of our
control group without headache presented stenosis. The crite-
rion used for stenosis is similar, but not identical, to that found
by Farb [16] to be most discriminating for detecting idiopathic
intracranial hypertension (a CCS <5, corresponding to either
complete unilateral occlusion or bilateral occlusion >50 % of
the transverse and sigmoid sinus). Nonetheless, the pertinence
of this threshold needs to be validated independently. In
addition, it is possible that stenosis may occur in other parts
of the cerebral venous circulation, in particular in the lower
part of the jugular vein, and that the frequency of stenosis in
patients with primary cough headache, primary exertional
headache, and primary headache associated with sexual activ-
ity may thus be under-estimated in our study.
The final objective of the study was to evaluate the
hypothesis that PCH on the one hand and PEH and PHASA
on the other represent different nosological entities, as sug-
gested by Pascual et al. [6, 9]. Achieving this objective was
compromised by the rather low number of patients available
for study. Pascual et al. [6, 9] reported that PCH tends to
affect older patients than primary exertional headache, and
primary headache associated with sexual activity. PCH is
less frequently associated with comorbid migraine, and less
frequently presents with signs characteristic of migraine
such as nausea or phonophobia. Although such trends were
observed in our sample, these did not reach statistical sig-
nificance. We did observe, however, that patients with PCH
reported headaches that were significantly shorter-lasting
than in the other two groups. There was also a trend for
the PCH to report less severe headaches. We found no
differences in clinical presentation between patients with
PEH and those with PHASA. Nonetheless, it should be
emphasized that the rather low number of subjects included
per group would preclude detection of subtle differences in
the features of these three headache groups.
We found that around half of the patients with
PHASA also reported headaches provoked by physical
effort or sport. This is consistent with some overlap in
triggering stimuli in these two patient groups, as reported
by Pascual et al. [6] and by Silbert et al. [27], and would
argue in favor of some nosological continuity between
these two types of headache. Patients in our PCH group
reported headaches provoked by other Valsalva maneu-
vers such as sneezing or defecation, but not by physical
effort, although it should be noted that several patients
with PEH did report headaches triggered by cough.
These observations are partially consistent with the hy-
pothesis that these represent two distinct forms of head-
ache [6, 9]. Our study has several strengths and
weaknesses. The strengths include the fact that data were
collected in a standardized manner during a patient
304
consultation, rather than retrospectively, as was the case
in several previous cohort studies and, above all, the fact
that all our patients underwent magnetic resonance ve-
nography allowing clinical presentation to be matched to
structural features of the cranial venous system. The most
important limitation of the study is that exploitable
images could not be obtained for all patients, and thus
the findings are derived from a rather limited number of
cases. This is particularly true for patients with PEH,
where the jugular veins could only be visualized for five
patients. Our findings clearly need replicating in a larger
sample of patients and extended to other parts of the
cranial venous system, in particular the entire jugular
axis. We also do not know whether the findings reported
are specific to patients with primary cough headache,
primary exertional headache, and primary headache asso-
ciated with sexual activity, or whether structural abnor-
malities of the cranial vasculature can also occur in other
forms of headache. This needs to be addressed in future
studies. Another limitation of the study is that given the
limited number of subjects available, it was not possible
to match the controls to individual cases. Nonetheless,
the mean age of the control (50 years for all headache
groups combined) and case (47 years) groups was simi-
lar, and both men and women were sufficiently repre-
sented in both groups. The last limitation was the
recruitment of control patients from the population of
cancer or neurological patients, but this same method
has been previously used by Farb et al. [16].
In conclusion, our study adds to knowledge of the clinical
presentation of cough headache, exertional headache, and
headache associated with sexual activity. The frequent asso-
ciation of PEH and PHASA with migraine and the recent
papers on venous abnormalities in migrainous patients ask
the question of the pathogenetic involvement of sinus stenosis
in PCH, PEC, and PHASA.
However, the recent controversy on chronic venous insuf-
ficiency in multiple sclerosis pathogenesis justify that our
results should therefore be interpreted with caution. Further
studies are needed to confirm these results.
Conflict of interest We declare that we have no conflict of interest.
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