Optic Nerve Enlargement in Krabbe's Disease
Blaise V. Jonesa,
Todd F. Barrona and
Javad Towfighia
a From the Department of Radiology, Neuroradiology (B.V.J.), Children's Hospital Medical Center, Cincinnati, OH, and the Departments of Pediatrics, Pediatric Neurology (T.F.B.), and Pathology (J.T.), M.S. Hershey Medical Center, Penn State Geisinger Health System, Hershey, PA.
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FIG 1. ;t1Case 1: MR imaging of enlarged optic nerves and white matter lesions in a 5-month-old male patient with Krabbe's disease.
A, Axial T1-weighted image shows symmetrical enlargement of the prechiasmatic intracranial optic nerves (arrow).
B, Sagittal T1-weighted image shows symmetrical enlargement of the prechiasmatic intracranial optic nerves (arrow).
C, Axial T2-weighted image shows symmetrical enlargement of the prechiasmatic intracranial optic nerves (arrow).
D, Axial T2-weighted image through the posterior fossa shows abnormal hyperintense foci in the region of the dentate nuclei (arrows), with some surrounding hypointensity.
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FIG 2. ;t1Case 2: Gross anatomy and histopathologic presentation of optic nerves in an 11-month-old female patient with Krabbe's disease.
A, Photograph of the base of the brain obtained at autopsy with the pons and posterior fossa contents removed. Arrows point to the symmetrically enlarged optic nerves.
B, Photomicrograph of a section from the optic nerve (hematoxylin and eosin stains; original magnification, x100) shows numerous multinucleated globoid cells (arrows).
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