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MELAS syndrome: an acute stroke-like episode complicated by renal tubular acidosis
  1. Dearbhail Ni Cathain,
  2. Emmet Browne,
  3. Kieran Skehan and
  4. Karl Boyle
  1. Beaumont Hospital, Dublin, Ireland
  1. Correspondence to Dr Dearbhail Ni Cathain; dearbhailnicathain{at}


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.

  • fluid electrolyte and acid-base disturbances
  • unwanted effects/adverse reactions
  • pharmacology and therapeutics
  • stroke

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  • Contributors DNC identified the case for reporting and wrote the body of the report with contribution from EB and KS to the clinical presentation and investigations performed. KB was the consultant supervisor for the report and reviewed the piece with amendments/edits, where appropriate.

  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

  • Competing interests None declared.

  • Provenance and peer review Not commissioned; externally peer reviewed.

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