Testai FD, Gorelick PB.
University of Illinois College of Medicineat Chicago, Chicago, IL, USA.
Arch Neurol 2010;67:19–24.
[2] Inherited metabolic disorders and stroke part 2: homocystinuria, organic acidurias, and urea cycle disorders.
Testai FD, Gorelick PB.
University of Illinois College of Medicineat Chicago, Chicago, IL, USA.
Arch Neurol 2010;67:148–53.
Editor’s note: These are two excellent articles that review the important inherited metabolic disorders associated with an increased risk of stroke. The first article covers Fabry’s disease and the mitochondrial disorder mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes (MELAS) [1], while the second article looks at homocystinuria, organic acidurias, and urea cycle disorders [2].The articles consider the epidemiology, biochemistry, and clinical features of each of the various disorders. For example, Fabry’s disease results from a deficiency in
α-galactosidase and is estimated to account for approximately 1% of cryptogenic strokes in patients aged
≤55 years.