MELAS syndrome: an acute stroke-like episode complicated by renal tubular acidosis

MELAS, a mitochondrially inherited multisystem disorder, can present with acute stroke-like episodes. The literature thus far supports the use of L-arginine therapy in acute MELAS flares to alleviate and shorten the duration of symptoms. This is the case of a patient who presented with ataxia and worsening confusion on a background of genetically confirmed MELAS syndrome. In this instance, intravenous L-arginine therapy, along with corticosteroids, was administered in keeping with best practice. However, in a metabolically vulnerable patient, L-arginine therapy resulted in a further deterioration in his clinical status and the development of a non-anion gap metabolic acidosis.