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Images in Neurology |

Unilateral White Matter Involvement in Krabbe Disease

Robin Lemmens, MD, PhD; Frans Piessens, MD; Philippe Demaerel, MD, PhD; Wim Robberecht, MD, PhD
Arch Neurol. 2011;68(1):130-131. doi:10.1001/archneurol.2010.331.
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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.

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Figure.

Unilateral white matter changes. Four sequential fluid-attenuated inversion recovery images are shown, identifying diffuse white matter changes in the right hemisphere. Follow-up magnetic resonance imaging (not shown) 1 year later was unchanged with respect to the white matter changes.

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