Monarch Disease Ontology identifier MONDO_0016796 (mitochondrial DNA depletion syndrome, encephalomyopathic form) is a group of mitochondrial DNA maintenance syndrome diseases characterized by predominantly neuromuscular manifestations with typically infantile onset of hypotonia, lactic acidosis, psychomotor delay, progressive hyperkinetic-dystonic movement disorders, external ophtalmoplegia, sensosineural hearing loss, generalized seizures and variable renal tubular dysfunction. It may be associated with a broad range of other clinical features. Also known as: mtDNA depletion syndrome, encephalomyopathic form.