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Oct 2011

Patient With Unilateral White Matter Involvement Does Not Have Krabbe Disease

Author Affiliations

Author Affiliations: Department of Child Neurology, Department of Pediatrics, VU University Medical Center, Amsterdam, the Netherlands (Dr Van der Knaap); and Department of Neurology, Jefferson Medical College, Philadelphia, Pennsylvania (Dr Wenger).

Arch Neurol. 2011;68(10):1345. doi:10.1001/archneurol.2011.1028

We read with interest the article by Lemmens et al1 on unilateral white matter involvement in Krabbe disease. Leukodystrophies typically present with specific patterns of abnormalities on magnetic resonance imaging.2 Whereas unilateral white matter involvement is known in unusual cases of X-linked adrenoleukodystrophy,3 this is unheard of in Krabbe disease. Magnetic resonance imaging in adult Krabbe disease is characterized by preferential and often selective involvement of the pyramidal tracts over their entire extent.4 The patient described by Lemmens et al has patchy and partially multifocal white matter abnormalities in the deep cerebral white matter with a unilateral or highly asymmetrical distribution. Such abnormalities are usually stable.5 They may be seen in, for instance, chromosomal abnormalities and congenital infections.5-7 The white matter abnormalities in the present patient are not compatible with a diagnosis of Krabbe disease.