Cluster headache and arachnoid cyst.

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by  Edvardsson, Bengt

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a SpringerOpen Journal
CASE STUDY Open Access
Cluster headache and arachnoid cyst
Bengt Edvardsson* and Staffan Persson
Abstract
Background: Cluster headacheis a primary headacheby definition not caused byany knownunderlying structural
pathology. However, symptomatic cases have been described, e.g. tumours, particularly pituitary adenomas,
malformations, and infections/inflammations. The evaluation of cluster headache is an issue unresolved.
Case description: We present a case of a 43-year-old patient who presentedwith a 2-month history ofside-locked
attacks ofpain located inthe left orbit. He satisfied therevised International Classification ofHeadache Disorders
criteria for cluster headache.Hismedical and family histories were unremarkable. There was no history of headache.
A diagnosis ofclusterheadachewas made. The patientresponded to symptomatic treatment. Computer
tomography and enhanced magnetic resonance imaging after 1month displayed a supra- and intrasellar arachnoid
cyst withmass effect on adjacent structures. After operation, theheadache attacks resolved completely.
Discussion and evaluation: Although wecannotexclude an unintentional comorbidity, in our opinion, the
co-occurrence ofan arachnoid cyst with mass effect with unilateral headache, ina hithertoheadache-free man,
points toward thefact that in this case the CHwas causedor triggered bytheAC. The headache attacks resolved
completely after theoperationand thepatientalso remained headachefreeat thefollow-up. The response ofthe
headache to sumatriptan and other typical CH medications does not exclude a secondary form. Symptomatic CHs
responsive to this therapyhave been described. Associated cranial lesions such as tumours have been reported in
CHpatients and theattacks may be clinically indistinguishable from theprimary form.
Conclusions: Neuroimaging,preferablycontrast-enhancedmagneticresonanceimagingshouldalwaysbeconsidered
inpatientswithclusterheadachedespitenormalneurologicalexamination.Late-onsetclusterheadacherepresentsa
conditionthatrequirescarefulevaluation.Supra-andintrasellararachnoidcystcanpresentasclusterheadache.
Keywords:Clusterheadache,Arachnoidcyst,Neuroimaging,Secondary,Symptomatic,Magneticresonanceimaging,
Computertomography
Introduction However, the origin of headaches is manifold and in
Cluster headache (CH) is a primary headache, by defin- many patients with headaches, AC must be regarded as
ition not caused by any underlying structural pathology anincidentalfinding (Westermaieretal.2012).
and belonging to the group of trigeminal-autonomic ThequestionwhetherpatientswithCHshouldundergo
cephalalgias (Headache Subcommittee of the Inter- neuroimagingtoexcludeacausalunderlyingstructuralle-
national Headache Society 2004). CH is the most fre- sion is unresolved. AC causing typical CH has not been
quent syndrome in this group. Symptomatic cases of CH described. We report a patient with a typical CH in the
have been described, e.g. tumours, particularly pituitary settingofasupra-andintrasellararachnoidcyst.
adenomas, malformations, and infections/inflammations
(Cittadini&Matharu2009). Case report
Arachnoid cysts (AC) are extra-parenchymal and A 43-year-old man presented with a 2-month history of
intra-arachnoidal collections of fluid with a composition side-locked attacks of excruciatingly severe stabbing and
similar to that of cerebrospinal fluid (CSF). Headaches boring left-sided pain located in the orbit. The attacks
are the most frequent complaint of patient with an AC. were associated with nasal obstruction, conjunctival in-
jection, and restlessness and migrainous features such as
*Correspondence:[email protected]
nausea and photophobia/phonophobia. No continuous
DepartmentofNeurology,FacultyofMedicine,SkaneUniversityHospital,
S-22185,Lund,Sweden background pain was identified. The duration of the
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CommonsAttributionLicense(http://creativecommons.org/licenses/by/2.0),whichpermitsunrestricteduse,distribution,and
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attacks was about 30 min and the frequency 4 to 5 per
24 h, 3 to 4 days a week and they also occurred during
thenight.Therewasnohistoryof headache. Hismedical
and family history was otherwise unremarkable. He was
not on any medications and used no drugs. Vital signs,
physical examination, and neurological examination
were normal. Laboratory testing was normal. He satis-
fied the revised International Classification of Headache
Disorders criteria for CH. A diagnosis of CH was made
and subcutaneous sumatriptan as well as oral sumatrip-
tan were prescribed. A prophylactic treatment with ster-
oids and verapamil was suggested but the patient
preferred symptomatic medication instead of using a
prophylactic drug for CH. He responded to the treat-
ment with relief within 15 to 20 min. A follow-up was
planned. As the headache attacks continued, the patient
was hospitalized after about 1 month. At admission, the
neurological examination was normal. He was on the
following medication: subcutaneous/oral sumatriptan
when required.ACTscanofthehead displayed a supra-
andintrasellararachnoidcystwithmasseffect(Figure1).
An enhanced magnetic resonance imaging (MRI) was
ordered in order to further evaluate the lesion. It con-
firmed the diagnosis of a supra- and intrasellar arach-
noid cyst with mass effect on adjacent structures Figure2MRI,T2-weightedimagingdemonstratingalarge
(Figure 2). Operation (craniotomy with cyst fenestration) supra-andintrasellararachnoidcystwithmasseffecton
adjacentstructures.
and histopathological examination verified the diagnosis
of AC. The headache attacks resolved completely after
the surgery. He remained headache free and had not
experienced any headache attacks at follow-up after
4months.
Discussion
The case study highlights a patient with CH responding
to treatment. Evaluationrevealed a supra- and intrasellar
arachnoid cyst with mass effect on adjacent structures.
The patient satisfied the revised International Classifica-
tion of Headache Disorders criteria for CH (Headache
Subcommittee of the International Headache Society
2004).
Although we cannot exclude an unintentional comor-
bidity, in our opinion, the co-occurrence of an AC with
mass effect with unilateral headache, in a hitherto
headache-freeman,pointstowardthefactthatinthiscase
the CH was causedor triggered by the AC. Theheadache
attacksresolvedcompletelyaftertheoperationandthepa-
tientalsoremainedheadachefreeatthefollow-up.
The response of the headache tosumatriptan and other
typical CH medications does not exclude a secondary
form. Symptomatic CHs responsive to this therapy have
been described (Testa et al. 2008; Ad Hoc Committee on
Classification of Headache 1962). Associated cranial
Figure1CTofthebraindemonstratingalargesupra-and
lesions such as tumours have been reported in CH
intrasellararachnoidcystwithmasseffectonadjacentstructures.
patientsandtheattacksmaybeclinicallyindistinguishable
EdvardssonandPerssonSpringerPlus2013,2:4 Page3of3
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fromtheprimaryform(AdHocCommitteeonClassifica- which recommend neuroimaging in all patients with
tionofHeadache1962;Favieretal.2007). trigeminal-autonomic cephalalgias. MRI is the preferred
ThepathophysiologyofCHisnotwellknown.Themost procedure for imaging in CH cases because of its greater
widelyacceptedtheoryisthatprimaryCHischaracterized sensitivity to vascular disease, tumour, demyelinating
by hypothalamic activation with secondary activation of disease, and infections/inflammations (Wilbrink et al.
the trigeminal-autonomic reflex, probably by a trigeminal- 2009;Mainardi et al.2010).
hypothalamicpathway(Cittadini&Matharu2009). To our knowledge, this is the first report showing an
AC develop within the arachnoid membrane because of association between CH and an AC. CH might be the
splitting or duplication of this membrane (Westermaier presenting symptom of an AC even in typical forms of
et al. 2012). Many AC are asymptomatic. Headache is that headache. Neuroimaging, preferably contrast-
related to AC producing increased intracranial pressure enhanced magnetic resonance imaging should always be
(ICP) and often is combined with other symptoms considered in patients with CH. Late-onset CH repre-
of increased ICP (Olesen et al. 2006). The clinical sentsacondition thatrequirescareful evaluation.
symptoms are determined by the location of the cyst The principal author takes full responsibility for the
(Westermaieretal.2012). data presented in this study, analysis of the data, conclu-
Most cases of symptomatic CH reported have had sions, and conduct of the research. The principal author
sellar/parasellar abnormality as well as in this case. The had full access to those data and has maintained the
sympathetic, parasympathetic and sensory fibers of the right to publish any and all data independent of any
trigeminal nerve gather as a plexus in the sinus caverno- third party.
sus/hypophyseal region. Thus, nerves in this region ap- Concerning approval of human studies by the appro-
pear to be of importance to produce symptoms of CH priate ethics committee and therefore performed in ac-
(Olesenetal.2006). cordance with the ethical standards laid down in the
The exact pathophysiology in this CH case is unknown 1964 Declaration of Helsinki: In this case this is not
andonecanonlyspeculatewhetheranintermittenteleva- appreciable.
tion of ICP might trigger or cause a disturbed local nerve
Competinginterests
function in the sinus cavernosus/hypophyseal region.
Theauthorsdeclarethattheyhavenocompetinginterests.
(Wilbrink et al. 2009)suggest that a structural lesion may
cause autonomic imbalance, resulting in periodic fluctua- Authors’contribution
BEasthefirstauthorandSPasthesecondauthorhavemadesubstantive
tions in the activity of the autonomic nervous system,
intellectualcontributionstothestudy.BEhasbeenthemaincontributorto
ultimately leading to an attack-wise presentation of the thestudy.Bothauthorsreadandapprovedthefinalmanuscript.
symptoms.Differencesintheindividualthresholdfortrig-
Received:19October2012Accepted:4January2013
gering the parasympathetic trigeminal reflexes may also
Published:10January2013
playarole(Straubeetal.2007).
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