A case of delayed acute intestinal pseudo-obstruction after MELAS crisis in mitochondrial encephalomyopathy with lactic acidosis and stroke-like episodes

TL;DR

A 24-year-old woman with MELAS developed delayed intestinal pseudo-obstruction after a neurological crisis, which improved with jejunal decompression.

Contribution

Highlights delayed acute intestinal pseudo-obstruction as a rare MELAS complication and effective jejunal decompression as a treatment.

Findings

MELAS can lead to delayed acute intestinal pseudo-obstruction after a neurological crisis.

Jejunal decompression successfully improved bowel function in this patient.

Conservative measures failed to resolve the intestinal obstruction.

Abstract

Mitochondrial encephalomyopathy with lactic acidosis and stroke-like episodes (MELAS) is a rare mitochondrial disorder primarily presenting with neurological manifestations, but it may also lead to gastrointestinal complications such as intestinal pseudo-obstruction, which can significantly complicate clinical management.

We report a 24-year-old woman with genetically confirmed MELAS (mtDNA A3243G mutation and TRPM3 c.2878G > T heterozygous variant) who was admitted with acute confusion and generalized convulsive seizures. Neuroimaging revealed multiple low-density lesions on CT and abnormal signals in the bilateral occipital lobes and basal ganglia on MRI. Although her neurological symptoms stabilized after emergency treatment, she subsequently developed recurrent episodes of intestinal obstruction characterized by vomiting and decreased bowel sounds. Conservative measures were unsuccessful, and jejunal decompression was performed, resulting in significant improvement in bowel function and successful transition to normal oral intake.

This case illustrates delayed acute intestinal pseudo-obstruction (AIPO) as an uncommon but clinically important complication of MELAS, and shows that timely jejunal decompression can be an effective therapeutic intervention in selected patients.

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