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INTRODUCTION
According to the International Headache Society, cluster
headache is characterized by episodes of unilateral pain
in the supraorbital and temporal regions and occurs in
the territory supplied by the ophthalmic division of the
trigeminal nerve. If not treated, the pain typically lasts for
15 to 180 minutes. Facial autonomic symptoms include
ipsilateral tearing, conjunctival injection, rhinorrhea,
and nasal congestion.[1] Cluster headache belongs to
a group of disorders characterized by unilateral pain
in areas supplied by the trigeminal nerve. The pain
Cluster Headache Associated with Secondary Unilateral
Blepharospasm: A Case Report and Review of the
Literature
Abbas Bagheri, MD; Minoo Mohammadi, MD; Ghader Harooni, MD; Keyvan Khosravifard, MD
Mohadeseh Feizi, MD; Shahin Yazdani, MD
Ophthalmic Research Center, Shahid Beheshti University of Medical Sciences, Tehran, Iran
Abstract
Purpose: Cluster headache is one of the most serious types of headache that is accompanied by autonomic
parasympathetic symptoms. Its association with hemifacial spasm in the same side had been rarely reported.
The aim of this report is describing a case with this association and treatment strategies.
Case Report: Here we report a 37‑year‑old female with cluster headache associated with secondary unilateral
blepharospasm that was successfully treated with combination therapy including botulinum toxin injection.
Conclusion: Hemifacial spasm associated with cluster headache needs special attention and can be treated
successfully.
Keywords: Cluster Headache; Disport; Secondary Unilateral Blepharospasm
Case Report
Correspondence to:
Abbas Bagheri, MD. Ocular Tissue Engineering Research
Center,LabbanejadMedicalCenter,Boostan9St.
Pasdaran Ave., Tehran 16666, Iran.
E‑mail: abbasbagheri@yahoo.com
Received: 01‑08‑2015 Accepted: 13‑02‑2016
occurs in association with prominent ipsilateral cranial
autonomic symptoms, which are called trigeminal
autonomic cephalgias (TACs).[2] The hallmark of cluster
headache is the circadian or even circannual periodicity,
and studies have proven hypothalamus activity during
attacks.[3] Most patients experience cluster headache
episodes that last days to months, thereafter going into
remission. However, in 10% of the patients, the headache
is chronic, without any relief between episodes.
Abortive therapy for the acute attack phase includes
oxygen therapy, sumatriptan injections, sumatriptan
nasal spray, other forms of triptans, and conventional
preventive medications such as verapamil, lithium,
cortisone, sodium valproate, and indomethacin. Surgical
methods, particularly the destruction of the trigeminal
ganglion, have also been used.[4]
Botulinum toxin A may reduce the sensitization of
the peripheral trigeminal afferent nerve, which results
J Ophthalmic Vis Res 2017; 12(2): 225‑227
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DOI:
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How to cite this article: Bagheri A, Mohammadi M, Harooni G,
Khosravifard K, Feizi M, Yazdani Sh. Cluster headache associated with
secondary unilateral blepharospasm: A case report and review of the
literature. J Ophthalmic Vis Res 2017;12:225-7.
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Cluster Headache and Blepharospasm; Bagheri et al
226 Journal of ophthalmic and Vision research Volume 12, Issue 2, AprIl-June 2017
in the reduction of the nociceptive input and subsequent
attenuation of central pain sensitization.[5] Retrograde
transmission of botulinum toxin A may modulate the
release of neurotransmitters such as substance P[6] or
CGRP[7] in the trigeminal nerve terminals. Therefore,
botulinum toxin A injection to the pericranial muscles
is used for maintenance therapy in headache syndrome
as well as cluster headache.[8‑10]
As we reviewed the literature, we found only one
report on hemifacial spasm associated with cluster
headache[11] and another one on bilateral blepharospasm
in a case of TAC‑like headache.[12]
Here, we describe the case of a woman with a history of
cluster headache and ipsilateral secondary blepharospasm
who did not respond to the above‑mentioned therapy.
However, her symptoms subsided on administering
combination therapy.
CASE REPORT
A 37‑year‑old woman with a complaint of left‑sided
headache and ipsilateral blepharospasm was referred
to our oculoplastic clinic. The symptoms began 10 years
ago as severe left‑sided headache that gradually involved
the entire head; the headache was not pulsatile but
was sometimes associated with nausea. Primarily, the
headache was episodic; however, over time, it became
constant and was accompanied by mild depression and
anxiety symptoms. At the time of headache, left‑sided
unilateral blepharospasm, ipsilateral photophobia,
tearing, and rhinorrhea were also present [Figure 1a].
She often experienced headache at night and at waking
time. The recurrence rate was very severe, and she was
free from headaches only a few days a month. When the
headache remitted, the blepharospasm also improved. In
thelast10years,thepatienthadreceivedveDysport
injections in the left periorbital area, which induced
provisional remission of blepharospasm but had no
effect on the headache. The patient had also systemic
hypertension for 10 years, which was partially controlled
by the daily use of 100‑mg atenolol.
Systemic work up did not reveal any secondary
cause of hypertension; therefore, she was diagnosed
withessentialhypertension.BrainMRIndingswere
also normal. At the time of headache, the patient was
recommended to use 100% oxygen at a rate of 8 L/min,
but it had no effect on the headache and blepharospasm.
We also administered two doses of 100‑mg sumatriptan
at 2‑hour intervals, but these were also not effective.
Thereafter, Dysport was injected to the periorbital,
temporal, and occipital areas in small unit doses, and
this resulted in an improvement in blepharospasm and,
to some extent, tearing and rhinorrhea after 4 days;
however, the headache continued as before [Figure 1b].
Since the headache did not respond to the
above‑mentioned therapy, we consulted a neurologist
and administered a combination therapy with 125‑mg
sodium valproate two times a day for 1 week, 40‑mg
propranolol tablets every 12 hours, 25‑mg nortriptyline
every night, and 4‑mg perphenazine every night for
1 month. This combined medical therapy alleviated the
headachesignicantly,andthepatientreporteda70%
reduction in the recurrence rate and severity of headache
over 1 month, as well as a remission of blepharospasm.
After 1 month, the drug dosage was increased
to 250‑mg sodium valproate every 12 hours, 50‑mg
nortriptyline every night, and 8‑mg perphenazine every
night; moreover 50‑mg pregabalin tablets every night
were added to the treatment regimen. Six months after
the initiation of treatment, the patient reported that the
severity and recurrence rate of headache was controlled
up to 90% and that the blepharospasm did not relapse.
Moreover, her anxiety and depression symptoms were
considerably improved, and systemic hypertension was
well controlled.
DISCUSSION
The periodicity of cluster headache attacks indicates the
involvement of a biologic clock within the hypothalamus,
with central disinhibition of the nociceptive and
autonomic pathways, especially the trigeminal nociceptive
pathways.[11] Cluster headache is a member of the group
of disorders called TACs. The trigeminal distribution
of pain and ipsilateral autonomic features of these
disorders suggest the presence of a trigeminal–autonomic
reex[12] or trigeminal autonomic activation. Trigeminal
and facial nerve communication and their anatomical
and functional relationship have been investigated
before.[13,14] Nakazato et al reported the case of a man with
typical cluster headache who developed daily morning
hemifacial spasm, which occurred with a pattern similar
to cluster headache. Therefore, the authors suggested
Figure 1. Patient’s face: (a) before Dysport injection showing
severe blepharospasm. (b) After Dysport injection showing
improvement in blepharospasm.
a
b
Cluster Headache and Blepharospasm; Bagheri et al
Journal of ophthalmic and Vision research Volume 12, Issue 2, AprIl-June 2017 227
that hemifacial spasm and cluster headache have the
same mechanism.[11] In the present case, the patient had
unilateral blepharospasm with fine orbicularis oculi
twitching movement and brow ptosis, but her lower facial
muscles were not involved. Another case of a patient with
long‑lasting blepharospasm, photophobia, lacrimation,
and rhinorrhea that did not respond to bilateral Dysport
injection was reported by van Vliet et al.[12]
Our patient had a past medical history of systemic
hypertension, and systemic work up revealed the
presence of essential hypertension with no secondary
cause. A previous study has revealed that both systolic
and diastolic blood pressure significantly increased
during cluster headache attacks.[15]
The presence of abnormal face movement,
i.e., hemifacial spasm or blepharospasm, was not a
typical feature of cluster headache or TACs.[2,16,17] A
literature search revealed only two reports of abnormal
face movement in patients with TACs.[11,12] Our patient
had experienced episodes of typical cluster headache
and blepharospasm for 10 years, and over time, she
developed anxiety, constant headache, and systemic
hypertension. This suggests that beside the basic
pathophysiology of TACs and trigeminal and facial nerve
communication, psychologic factors may be involved in
the development of this disease combination. Although
an anatomical and functional connection exists between
the trigeminal and facial nerves and episodic hemifacial
spasms reported in patients with cluster headache
have been considered to have the same mechanism as
cluster headache,[11] psychologic factors and the patient’s
compensatoryresponsetothepainmayhaveaninuence
of the condition.[18,19] The patient’s symptoms did not
respond to conventional treatment for cluster headache
but improved up to 90% with a combination of drugs
having analgesic, anti‑anxiety, and anti‑depression
effects, as well as prophylactic agents for cluster
headache and symmetrical botulinum toxin A injection
to the bilateral orbicularis oculi, temporalis, and occipital
muscles. Therefore, we conclude that unilateral secondary
blepharospasm in this patient with cluster headache had a
multifactorial origin. Future research may reveal the exact
mechanism or even the prevalence of facial movement
disorders in association with cluster headache, and may
reveal whether increasing the systolic and diastolic blood
pressure during cluster headache attacks may lead to the
development of essential systemic hypertension.
Financial Support and Sponsorship
Nil.
Conicts of Interest
Therearenoconictsofinterest.
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