Case Report: Heart-shape sign in bilateral medial medullary infarction: a case report and review of the litterature

Abstract* Introduction Bilateral medial medullary infarction (BMMI) is a rare ischemic lesion that typically presents as dysarthria, dysphagia, tetraplegia, and respiratory failure. It is known for its characteristic « heart-shaped » appearance on magnetic resonance imaging (MRI). The etiological profile of this disease has not been studied previously. Considering the unfavorable outcome of BMMI, it is crucial to investigate the clinical and etiological profile to optimize the management of these patients. Case presentation A 49-year-old north-african smoker male patient was admitted to the neurology department of the military hospital of Tunis-Tunisia 24 hours after acute-onset slurred speech, weakness and impaired sensation in all four limbs, predominantly in the lower limb. His family history was remarkable for young-onset brainstem ischemic stroke (IS) in a brother. Neurological examination a right hemiparesis with tactile hemihypoesthesia and right astereognosia (modified Rankin scale of 2). Cerebral MRI showed radiological aspect compatible with diagnosis of « heart-shaped » BMMI. A heterozygous mutation R506 of Leiden Factor V was identified. The patient’s condition remained stable at 15 months follow-up (Modified Rankins Scale at 2) and no recurrence of IS was noted under anticoagulation to date. Conclusions BMMI is a rare case of medial medullary infarction, accounting for less than 1% of IS cases. To our knowledge, this is the first case of « heart-shaped » BMMI linked to Leiden Factor V mutation.