Young Stroke – An Eagle Eye View

Introduction

‘Eagle Syndrome’ is caused by a pointed bony projection of styloid process. The elongation might be bilateral or unilateral. It was first described in 1937 by an otorhinolaryngologist named Eagle.¹ The prevalence of Eagle syndrome is 4% in general population with 4-10% patients of these becoming symptomatic.² Prevalence in women is more as compared to men3 with patients usually presenting at 40 years of age. Advancing age causing loss of elasticity of soft tissue, thus putting pressure on adjoining tissue, is thought to be the underlying pathogenesis.⁴ Presenting symptoms incude recurrent neck or throat pain, foreign body sensation and symptoms mimicking maxillofacial, oropharyngeal disorder and/or neuralgias.³ We present a case of Eagle Syndrome in a young male who presented with recurrent stroke.

Case report

A 34-year-old gentleman presented with left sided weakness in left upper limb and left lower limb, power 4/5 with gait imbalance since 5 days. He had a history of acute ischemic stroke in right middle cerebral artery (MCA) territory with suspected atherogenic right internal carotid artery (ICA) occlusion (40- 50%) with hypoplastic right A1 anterior cerebral artery (ACA) segment 3 months prior to this admission [Fig1a, b]. At this time, patient presented with gait ataxia, left upper limb and lower limb power 3/5 and facial asymmetry. On examination he was conscious, oriented, following commands. He had significant left hemiparesis. Exhaustive Young Stroke workup and evaluation for cardioembolic stroke was negative. The vascular imaging was reviewed, and a diagnosis of Eagle Syndrome was made with focal tortuosity and stenosis in right mid cervical ICA, closely abutting right elongated styloid process (Fig2). There was a suspicion of dissection because of which ear nose and throat surgeon’s (ENT) opinion was then taken, and patient was suggested styloidectomy as a definitive management.

Discussion

There is an increased risk of cerebral infarction in young adults particularly in developing countries thought to be due to increased incidence of smoking and other risk factor.⁵ There are many causes and risk factors of stroke in children and young adults as illustrated in Table 1. The common causes such as cardioembolic stroke, arterial dissection, migraine and certain ethnicity is also seen in Young Stroke^5,6 along with oral contraceptive users and drug abusers. Diseases such as Anti-phospholipid syndrome, inherited coagulopathy, Veno arterial shunting, rheumatic heart disease is also a known etiology. Structural etiology can be ruled out in the young by echocardiography. Coagulopathy and vascular disease including Moya-Moya, reversible cerebral vasoconstriction (RCVS), systemic lupus erythematosus (SLE), mitochondrial myopathy, mitochondrial encephalopathy, lactic acidosis, and stroke like episodes (MELAS) and leukoencephalopathy require extensive testing and imaging. In our case, the coagulation profile was normal eliminating various etiologies. Twenty-five percent of the population have a Patient Foramen Ovale⁵ which is also speculated in Young Stroke according to several studies. Although the evidence is unclear, migraine particularly migraine with aura, is associated with increased risk of stroke in young population as well. The mechanism and pathophysiology remain unclear.⁷

Hyper-homocysteinemia, a genetic mutation, is thought to cause abnormal blood clotting as well as deficiencies of folate and vitamins B6 and B12. This is thought to be related to coagulopathies and small vessel disease and is an emerging cause of stroke in young. Hyper-homocysteinemia is seen more in young men with stroke, four times more often than females.⁵ Hypercoagulable workup also includes investigation of protein C and S levels. Even though it concurs with less percentage of stroke in young, even mild deficiency of these proteins is responsible for about 4-12% of stroke in young

In this case study, we diagnosed recurrent ischemic stroke due to Eagle Syndrome. Stroke in Young itself is a diagnostic dilemma. Anatomical variations like elongated styloid process are considered even more rarely as the etiology. Our patient had a presenting complaint of left sided weakness along with gait imbalance with recurrent right sided neck pain. CT angiography was normal but in the same sequence bony elongation of styloid process was seen, dissection was suspected, and surgical intervention was recommended. Even though with thorough investigation, in as many as in 35% of cases, the etiology of stroke remains unclear.⁸

Table 1: Causes and risk factors for ischemic stroke in children and young adults

MELAS: Mitochondrial encephalopathy, lactic acidosis and stroke like episodes