A case report of eagle syndrome in a patient affected by multiple sclerosis

Background and Aim : Eagle syndrome is a rare condition which is caused either by ossification in styloid process or classification of stylohyoid ligament or stylomandibular ligament with an unknown mechanism .Its prevalence rate is estimated to be 4%, and more common in women. The length of a normal styloid process is within 2 to 3 cm. However, different criteria regarding the length of styloid process is used for the diagnosis. The syndrome is characterized by neuralgic pain in throat and neck radiating into ears, dysphagia, headache, sensation of foreign body and voice changes .It has been demonstrated that the symptoms are caused by compression of nearby structures such as glossopharyngeal nerves, external and internal carotid arteries, cranial nerves and hypoglossal nerves. Here we present a 31-year-old woman with history of Multiple sclerosis (MS) who was diagnosed with eagle syndrome.Methods : A thirty-one years old woman with a medical history of multiple sclerosis (MS) referred to otolaryngologist with constant pain in throat and ears. The pain exacerbated with yawning and opening mouth. The patient was taking a daily dose of fingolimod (0.5mg) and Vitamin D (2000IU). Prior to this, in the first evaluation by a general physician, the patient was diagnosed with ear and throat infection. Despite the treatment with antibiotics, the pain persisted for more than two months. In the initial evaluation by an otolaryngologist, the patient described the pain as being sharp. She did not mention any history of trauma or tonsillectomy. The intraoral palpation of tonsillar fossa revealed palpable styloids. In order to rule out neuropathies, computed tomography (CT) scan and radiology were carried out. In Town X-ray view, the bone density of mandible was within the normal limit, a sequel of chronic fracture within left mandible ramus was seenResults : The levels of Ca, vitamin D, Mg, and P were all within the normal range in the presented case. The patient did not remember any traumatic incidences. Nevertheless, a sequel of chronic fracture within left mandible ramus was seen in patient’s radiology. In an investigation by Kim and colleagues, mechanical stress was the main reason of HSP70 and HO-1 activation. These factors can result in up-regulation of osteogenesis and bone formation.Increase in oxidative stress can induce HO-1 expression. Additionally, it has been demonstrated that HO-1 and Hsp70 have elevated levels in blood sample and CSF of MS patientsConclusion : Eagle syndrome is known as elongation of styloid process. During years, several theories have been put forward as the main etiology behind this syndrome. Inflammation following previous trauma, calcification in presence or absence of mineral imbalance or congenital reasons has been considered as the main culprits of this syndrome. Here, we presented a case who was suffering from bilateral elongation of styloids and MS. We speculated that activation of certain stress factors, cytokines and immune cells during the disease course of MS and a previous fracture in mandible ramus can be taken into account as the probable reasons for the development of this syndrome. However, more investigations need to be done to strengthen this theory.

Keywords:

Styloid process , Eagle syndrome , Classification , Ossification , Multiple Sclerosis