MELAS: Clinical and pathologic correlations with MRI, xenon/CT, and MR spectroscopy.

Abstract:

:We describe the clinical, imaging, and pathologic findings in a patient with mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes (MELAS). The patient experienced her first stroke-like episode at age forty-four. Brain MRI, obtained at symptom onset, at 3 weeks, and at 1 year, revealed migrating T2-weighted hyperintensities in the temporal/parietal and occipital cortices and later revealed atrophy. Abnormal cerebrovascular reserve was evident on xenon/CT four days after the first MRI. MR spectroscopy at 1 year revealed increased lactate in both the occipital and temporal lobes. Histologic sections demonstrated spongy degeneration of the cortex that was most prominent at the crests of the gyri. Electron microscopy of the blood vessels showed increased numbers of abnormal mitochondria within the vascular smooth muscle and in endothelial cells. We hypothesize that the stroke-like episodes in MELAS may be due to impaired autoregulation secondary to the impaired metabolic activity of mitochondria in the endothelial and smooth muscle cells of blood vessels.

journal_name

Neurology

journal_title

Neurology

authors

Clark JM,Marks MP,Adalsteinsson E,Spielman DM,Shuster D,Horoupian D,Albers GW

doi

10.1212/wnl.46.1.223

subject

Has Abstract

pub_date

1996-01-01 00:00:00

pages

223-7

issue

1

eissn

0028-3878

issn

1526-632X

journal_volume

46

pub_type

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