Objective:

We report a case of rapidly progressive orthostatic syncope secondary to a cerebellopontine angle epidermoid cyst.

Background:

Episodic loss of consciousness carries a broad differential diagnosis and has high prevalence in pediatric population. Given prevalence of benign causes of syncope and the sensitivity of careful history-taking, extensive diagnostic workup is infrequently pursued. When typical patterns are not recognized, further diagnostic evaluation is indicated.

Design/Methods:

N/a

Results:

A 14-year-old male with history of eosinophilic esophagitis, resolved iatrogenic adrenal insufficiency, obsessive compulsive disorder, major depressive disorder, generalized anxiety disorder, Tourette’s syndrome, episodic sleep paralysis presented with rapidly progressive orthostatic symptoms. Symptoms began with orthostatic presyncope and progressed to syncope with occasional syncopal convulsions. Serial exams by multiple subspecialities did not elicit abnormal findings. Cardiac monitoring during stereotyped episodes showed sinus rhythm with occasional sinus tachycardia. Inter-event cardiac monitoring revealed rare premature atrial and ventricular contractions not temporally associated with syncope or symptoms. Echocardiogram showed tiny coronary-pulmonary artery fistula and dilated aortic sinotubular junction and was otherwise normal. Routine EEG was normal, and prolonged video EEG for spell capture found high voltage delta slowing without epileptiform potentials. MRI showed left cerebellopontine angle nonenhancing hyperintense T2 with hypointense T1 mass with associated restricted diffusion and pontine mass effect. MRI and pathological microscopic examination most consistent with epidermal inclusion cyst. Cyst was resected and post-operative course complicated by cerebral salt wasting but syncopal events resolved.

Conclusions:

Nervous system structural etiologies of orthostatic intolerance and syncope are rare across the lifespan. The frequency of benign causes of syncope presents a diagnostic difficulty, and not all structural differences are accompanied by neurological deficits on physical exam. When typical disease presentations are not recognized, a high index of suspicion is required to diagnosis these rare causes of secondary syncope.