Leigh syndrome is a progressive neurodegenerative mitochondrial disorder that typically presents in the first year of life with psychomotor regression, vomiting, diarrhea, dysphagia, failure to thrive, dystonia, ataxia, and ophthalmoparesis. Death due to respiratory failure usually occurs by two to three years of life. Brain MRI can show symmetrical T2 hyperintense lesions in the basal ganglia, periaqueductal grey, and cerebral peduncles or brainstem.

Ceroid lipofuscinosis spares white matter relative to gray matteR