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Images in Neurology
January 2011

Unilateral White Matter Involvement in Krabbe Disease

Author Affiliations

Author Affiliations: Departments of Neurology (Drs Lemmens and Robberecht) and Radiology (Dr Demaerel), University Hospitals Leuven, Leuven, Belgium; Vesalius Research Center, VIB, and Laboratory for Neurobiology, Section of Experimental Neurology, School of Medicine, University of Leuven, Leuven (Drs Lemmens and Robberecht); and the Department of Neurology, General Hospital Sint Maarten, Duffel (Dr Piessens), Belgium.

Arch Neurol. 2011;68(1):130-131. doi:10.1001/archneurol.2010.331

A 43-year-old man with no relevant medical history visited an ear, nose, and throat specialist because of deafness in the left ear. He was diagnosed with otosclerosis for which a stapedectomy was performed, resulting in resolution of symptoms. During the diagnostic evaluation, a magnetic resonance image of the brain was performed; based on the findings, he was sent to a neurologist. He had no clear neurological concerns. Findings of neurological examination were unremarkable. His father had died of a heart attack at the age of 45 years. One of his sisters sustained a myocardial infarction at the age of 46 years; the other was diagnosed with cancer and died at age 24 years. His 2 brothers died in early infancy of unknown cause.