Abstract
A 16-year-old boy with known Leigh/mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes (MELAS) overlap syndrome ( MTND5 T12706C mutation) developed worsening dysphagia. Examination revealed reduced palatal movements and dysarthria. MRI showed prior strokes, basal ganglia lesions, and new right > left medullary lesions (figure). He was discharged on gastrostomy tube feeds to decrease aspiration risk. Leigh syndrome is a neurodegenerative mitochondrial disorder with necrotizing basal ganglia and brainstem lesions.1 MTND5 mutation causes rare Leigh/MELAS overlap syndrome with clinical and neuroradiologic characteristics of both.2 Though dorsal medullary lesions are common in Leigh syndrome, they are rarely asymmetric or associated with dysphagia.1
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