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Metabolic stroke in childhood: Diagnostic approach and suggestions for therapy

Lookup NU author(s): Professor Rita HorvathORCiD, Professor Hanns Lochmuller

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Abstract

The central nervous system is particularly vulnerable to certain metabolic abnormalities that may result in energy failure and subsequent neuronal cell death with clinical presentation as strokelike episodes. The mitochondrion and its electron transport chain machinery provide the main source of ATP for neurons and the most frequent causes of metabolic stroke are associated with the disorders of mitochondria. Mitochondrial diseases are a heterogeneous group of conditions affecting different organs of the body with variable severity. Both inborn errors (e.g. organic acidurias) and mitochondrial cytopathies can lead to stroke by vascular or cytotoxic mechanisms, thereby making a differential diagnosis complicated when using standard clinical investigations or neuroimaging. This article will provide an overview of various metabolic strokes in childhood with a special emphasis on the most frequent mitochondrial diseases. © 2010 IOS Press and the authors.

Publication metadata

Author(s): Horvath R, Lochmüller H, Acsadi G

Publication type: Review

Publication status: Published

Journal: Journal of Pediatric Neurology

Year: 2010

Volume: 8

Issue: 3

Pages: 321-332

Print publication date: 09/06/2010

ISSN (print): 1304-2580

ISSN (electronic): 1875-9041

URL: http://dx.doi.org/10.3233/JPN-2010-0404

DOI: 10.3233/JPN-2010-0404

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