Laminar cortical necrosis in mitochondrial disorders

Objectives

Laminar cortical necrosis, defined as focal or diffuse necrosis of one or more cortical lamina, represents an increasingly recognized neuropathological endpoint of vascular, endocrine, immunologic, metabolic, or toxic conditions, of which mitochondrial disorders (MIDs) are the third most frequent after cerebral ischemia and hypoxia.

Aims

To investigate the prevalence of laminar cortical necrosis in MIDs, types of MIDs associated with laminar cortical necrosis, and the morphological characteristics on imaging and autopsy.

Methods

Medline literature review for the terms “laminar cortical necrosis”, “cortical signal change”, “mitochondrial” and all acronyms of syndromatic MIDs.

Results

Among 139 hits for “laminar cortical necrosis”, 10 articles fulfilled the inclusion criteria (7%). Among the ten hits five were case series and the other five single case reports. The syndromic MID most frequently associated with laminar cortical necrosis is the MELAS syndrome, but was also described in a single patient each with Leigh syndrome, mitochondrial depletion syndrome, and mitochondrial spinocerebellar ataxia. The morphological and pathohistological features of laminar cortical necrosis in MIDs were not at variance from those in non-mitochondrial disorders.

Conclusions

In MIDs laminar cortical necrosis represents the histopathological and imaging endpoint of a stroke-like lesion. Though laminar cortical necrosis may have a wide pathophysiological background the histological and imaging characteristics do not vary between the different underlying conditions.