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Inappropriate intracranial hemodynamics in the natural course of MELAS

Junko Nishioka1, Yukihiro Akita, Shuichi Yatsuga

  • 1Department of Pediatrics and Child Health, Kurume University School of Medicine, Kurume City, Fukuoka, Japan.

Brain & Development
|August 1, 2007
PubMed

Insights

Mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes (MELAS) patients show abnormal brain blood flow that worsens over time. This hypoperfusion, particularly in the posterior cingulate cortex, suggests a link to mitochondrial angiopathy.

Area of Science:

  • Neurology
  • Neuroimaging
  • Genetics

Background:

  • Mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes (MELAS) is a genetic disorder.
  • Intracranial hemodynamic abnormalities are observed in MELAS, but their progression and relation to disease course are not fully understood.

Purpose of the Study:

  • To clarify regional cerebral blood flow (rCBF) changes in the natural course of MELAS.
  • To understand the pathogenic mechanisms, including cytopathy and angiopathy, in MELAS.

Main Methods:

  • Serial brain single photon emission computed tomography (SPECT) studies analyzed with Statistical Parametric Mapping (SPM) 99.
  • Analysis of 5 MELAS patients with A3243G mutation over a maximum 10-year interval.
  • Voxel-by-voxel analysis of functional images using statistical methods.

Main Results:

  • Inappropriate intracranial hemodynamics were present in acute and interictal phases, worsening with disease progression.
  • Consistent hypoperfusion in the posterior cingulate cortex was observed in MELAS patients.
  • These hemodynamic changes may be linked to mitochondrial angiopathy.

Conclusions:

  • Intracranial hemodynamic abnormalities are a common feature in the natural course of MELAS.
  • Progressive hypoperfusion, especially in the posterior cingulate cortex, is characteristic of MELAS.
  • Findings suggest a significant role for mitochondrial angiopathy in MELAS pathogenesis.

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