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American Journal of

Experimental and Clinical Research

Am J Exp Clin Res 2017;4(3):229-231

Case Report

Seconder paroxysmal hemicrania caused by mucocele

Burc Esra Sahin

1
*, Ece Ozdemir Oktem

1
Department of Neurology, Faculty of Medicine, Ahi Evran University, Kirsehir, Turkey

2
Department of Neurology, Faculty of Medicine, Manavgat State Hospital, Turkey

Abstract. Paroxysmal hemicrania (PH) is classified as a subgroup of trigeminal autonomic headaches among primary headaches. The pain

is unilateral, located frequently at the orbital, supraorbital and/or temporal regions. It is sharp, severe, and these headaches tend to occur at

least 5 times a day, which last for 2 – 30 minutes, accompanied by autonomic findings such as conjunctival injection, lacrimation, nasal

congestion, nasal discharge, ptosis and orbital edema. Secondary PH is associated with tumor or vascular pathologies. Most frequently

hypophyseal tumors were detected. In our case, there was a mucocele originating from the left ethmoidal sinus and compressing on the

medial rectus muscle in the cranial MR examination of a patient admitted with clinical features of PH. In this case, it was seen that

neuroimaging is mandatory in patients with TOB, in order to exclude secondary causes. Also, nociceptive impulses originating from

surrounding tissues with the compression effect of the mucocele induce trigeminal autonomic reflex, supporting the hypothesis that

autonomic symptoms and pain occur with hypothalamic and trigeminal connections.

Keywords: Trigeminal autonomic cephalalgy, paroxysmal hemicrania, mucocele

Introduction

Paroxysmal hemicrania is classified among trigeminal

autonomic cephalgies subgroup among primary headaches

[1]. TOS is a kind of headache characterized by autonomic

symptoms at trigeminal nerve somatic distribution [2]. The

pain is frequently seen at the ophthalmic region of the

trigeminal nerve. IHSS-3 have remarked that PH pain may

be seen as orbital, supraorbital, temporal or any

combination of the trigeminal nerve [1]. PH is

characterized by very severe pain accompanied by sharp,

short unilateral autonomic symptoms [3]. The pain is

typically described as severe, throbbing, and squeezing

type of sensation [3, 4, 5, 6]. Pain is intermittent and starts

suddenly. It frequently lasts for 10-30 minutes, with a

range duration between 2-45 minutes. More than 60% of

patients experience restlessness and pain between episodes

[7]. IHSS-3 beta criteria requires accompaniment of at

least one autonomic symptom such as ipsilateral

conjunctival injection, lacrimation, nasal congestion,

rinorrhea, sweating of forehead and face, myosis, ptosis

and/or eye edema [1].

The prevalence of PH is not as low as it may be thought.

Either the cases were not recorded or the diagnosis was not

considered. Case series have described both typical and

atypical cases, and 22% of cases show an atypical

presentation [8].

Secondary PH is typically associated with tumors and

vascular pathologies. Most frequent causes of PH and other

TOS include pituitary gland pathologies, and for this

reason cranial MR imaging is mandatory [8].

In our case, there was a mucocele which originated

from the left ethmoidal sinus and compressed the medial

rectus muscle at cranial MR imaging in a patient admitted

with clinical features of PH.

Case report

A 52-year-old female patient was admitted at our

outpatient clinics with complaints of headache which had

started 3 months ago, and showed an increase in frequency

and severity in the last 15 days. The pain was experienced

around the left eye, lasted for 5-10 minutes, and had

occurred for at least 10-20 episodes per day. The patient

described the pain as very severe in intensity and

stinging/stabbing. There was injection and ptosis at the

ipsilateral eye during an episode. Treatment was initiated

with indomethacin 75 mg/day and complete remission was

obtained on the second day. A lesion originating from the

left anterior ethmoid sinus and compressing the left medial

rectus muscle was detected in cranial MRI (Figure 1).

The patient had a surgical intervention at the ENT

department, and histopathological examination revealed a

mucocele. The patient did not experience pain during one-

year of follow-up.

Discussion

PH is a rarely encountered type of headache, which

___________________________________________________________
* Corresponding author: Burc Esra Sahin, MD (besras11@yahoo.com).

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mailto:besras11@yahoo.com

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Figure 1. Cranial MR a) T1, b) T2, c) T2 Flair imaging:
Mucocele originating from the left anterior ethmoid sinus and

compressing the left medial rectus muscle

Discussion

PH is a rarely encountered type of headache, which

may be due to missing this diagnosis or not recording it.

There may be a group of patients, which may not exactly

fulfill the IHSS-3 diagnostic criteria [8].

There is overlap in clinical findings and treatment of

TACs, which may be caused by a common

physiopathology of TACs [9, 10]. In a PET study in

patients with PH, activation was observed in the

contralateral posterior hypothalamus, ventral midbrain, red

nucleus and substantia nigra [11]. Trigeminal autonomic

reflex (TAR) was held responsible for the symptoms of

trigeminal autonomic pain syndromes. TAR activation is

responsible for the acute attacks of TACs [12].

This reflex is probably triggered due to activation of

hypothalamus and structures closely related with it [12].

PH and other autonomic findings of TACs probably

develop with central dysinhibition of TAR by direct

hypothalamic – trigeminal connections of the

hypothalamus [13].

Pituitary gland lesions constitute the most frequent

cause of secondary PH and TAC. For this reason, cranial

MRI should be part of our imaging studies in PH and

TACs [8].

In conclusion, a mucocele originating from the

ethmoidal sinus and compressing the rectus muscle induces

TAR with nociceptive impulses that originate from the

surrounding tissues. Autonomic symptoms and pain occur

as a result of hypothalamic – trigeminal connections.

Neuroimaging should be done in order to exclude

secondary causes in all patients with a diagnosis of PH.

Conflict of Interest

The authors declare no conflicts of interest.

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