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Laminar cortical necrosis in mitochondrial disorders
1Krankenanstalt Rudolfstiftung, Vienna, Austria. fifigs1@yahoo.de
Clinical Neurology and Neurosurgery
|August 12, 2009
Summary
Laminar cortical necrosis is a neuropathological finding in mitochondrial disorders (MIDs). While its causes vary, the imaging and histological features of this condition are consistent across different underlying mitochondrial diseases.
Area of Science:
- Neuropathology
- Neuroimaging
- Mitochondrial Medicine
Background:
- Laminar cortical necrosis is a recognized neuropathological endpoint for various conditions.
- Mitochondrial disorders (MIDs) are the third most frequent cause of laminar cortical necrosis, following cerebral ischemia and hypoxia.
Purpose of the Study:
- To determine the prevalence of laminar cortical necrosis in MIDs.
- To identify specific MIDs associated with laminar cortical necrosis.
- To characterize the imaging and autopsy features of laminar cortical necrosis in MIDs.
Main Methods:
- A Medline literature review was conducted.
- Search terms included "laminar cortical necrosis", "cortical signal change", "mitochondrial", and acronyms of syndromic MIDs.
Main Results:
- Ten articles (7% of 139 hits) met inclusion criteria, comprising case series and single case reports.
- MELAS syndrome was the most frequently associated MID, with other cases involving Leigh syndrome, mitochondrial depletion syndrome, and mitochondrial spinocerebellar ataxia.
- Morphological and pathohistological features of laminar cortical necrosis in MIDs were similar to those in non-mitochondrial disorders.
Conclusions:
- Laminar cortical necrosis in MIDs represents the histopathological and imaging endpoint of a stroke-like lesion.
- Despite diverse pathophysiological backgrounds, the histological and imaging characteristics of laminar cortical necrosis remain consistent across different underlying conditions.
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