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Mitochondrial encephalomyopathy with corticosteroid dependence.

S S Gubbay1, G J Hankey, N T Tan

  • 1Royal Perth Hospital, WA.

The Medical Journal of Australia
|July 17, 1989
PubMed
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Corticosteroid therapy effectively manages mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes (MELAS). This treatment rapidly resolves neurological dysfunction and prevents relapses in MELAS patients, suggesting it as a standard therapy.

Area of Science:

  • Neurology
  • Genetics
  • Pediatrics

Background:

  • Mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes (MELAS) is a rare genetic disorder.
  • The case describes a 12-year-old boy with MELAS and his mother with asymptomatic mitochondrial myopathy.

Observation:

  • The patient presented with severe neurological symptoms including disorientation, dysphasia, hemianopia, and hemiparesis.
  • Cranial CT scan revealed infarction in the left posterior hemisphere and basal ganglia calcification.
  • Neurological dysfunction resolved within two weeks of initiating corticosteroid treatment.

Findings:

  • Attempts to reduce dexamethasone dosage led to repeated symptom exacerbations and neurological relapse.
  • The patient demonstrated a marked dependency on corticosteroids, with even minor dosage reductions causing adverse effects.

Related Experiment Videos

  • Reintroduction of corticosteroids rapidly resolved all symptoms and prevented further infarction.

    • Implications:

    • This case highlights a remarkable corticosteroid responsiveness in MELAS syndrome.
    • Corticosteroid therapy should be considered a standard treatment for MELAS.
    • Further evaluation is needed for corticosteroid use in other mitochondrial disorders.