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American Journal of Neuroradiology 21:1478-1482 (8 2000)
© 2000 American Society of Neuroradiology

ARTICLE

MR Imaging and Proton MR Spectroscopy in Adult Krabbe Disease

Laura Farina^,a, Alberto Bizzi^a, Gaetano Finocchiaro^a, Davide Pareyson^a, Angelo Sghirlanzoni^a, Barbara Bertagnolio^a, Mario and Savoiardo^a, and SakkuBai Naidu^a, Bhim S. Singhal^a and David A. Wenger^a

^a From the Departments of Neuroradiology (L.F., A.B., M.S.), Neuro-Oncology and Gene Therapy (G.F.), Neurology (D.P., A.S.), and Biochemistry and Genetics (B.B.), Istituto Nazionale Neurologico, Milan, Italy; the Department of Pediatrics and Neurology, Johns Hopkins Medical Institutions and the Neurogenetics Unit, Kennedy Krieger Institute, Baltimore, MD (S.N.); the Department of Neurology, Bombay Hospital, Medical Research Centre, Bombay, India (B.S.S.); and the Department of Neurology, Jefferson Medical College, Philadelphia PA (D.A.W.).

Summary: We present the MR imaging findings in four patients (two pairs of siblings from two unrelated families) with adult Krabbe disease. In the first family, clinical presentation mimicked familial spastic paraplegia. Their MR images showed selective, increased signal intensity on T2-weighted sequences along the corticospinal tracts, most prominently in the proband and barely detectable in her brother. Proton MR spectroscopy showed increased choline and myo-inositol in the affected white matter. In the second family, the clinical presentation differed in that the signs of pyramidal tract involvement were asymmetrical, with concomitant asymmetry on MR images in one. In adults, Krabbe disease may present on MR imaging with selective pyramidal fiber involvement.

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