Bilateral Hypoglossal Nerve Palsy in an Adult Patient With Repaired Tetralogy of Fallot.

INTRODUCTION: Spontaneous cervical artery dissection (sCAD) is a major cause of ischemic stroke in young and middle-aged adults, but the occurrence of multiple sCADs could suggest the presence of an underlying arteriopathy. Bilateral hypoglossal nerve palsy caused by extracranial internal carotid artery dissections have been rarely described. We present the case of a 3-vessel sCAD in a patient with repaired tetralogy of Fallot (ToF).

CASE REPORT: A 50-year-old man was admitted to our stroke unit for a sudden onset of left cervical pain, mild left ptosis, dysarthria, and dysphagia. His medical history was remarkable for surgically repaired ToF. Neurological examination showed left Claude-Bernard-Horner syndrome and bilateral hypoglossal palsy. Diffusion-weighted magnetic resonance imaging excluded parenchymal lesions while magnetic resonance angiography showed a pattern of bilateral extracranial internal carotid and right vertebral dissection.

CONCLUSIONS: This is the first case of multiple-vessel sCAD, and more associated with an unusual clinical presentation, in a patient with surgically repaired ToF, supporting the hypothesis that defective large artery elastogenesis plays a role in this congenital disorder, and suggesting the importance of continuous long-term vascular follow-up.