MR imaging and proton MR spectroscopy in adult Krabbe disease

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 imag es showed selective, increased signal intensity on T2-weighted sequences al ong the corticospinal tracts, most prominently in the proband and barely de tectable in her brother. Proton MR spectroscopy showed increased choline an d myo-inositol in the affected white matter. In the second family, the clin ical presentation differed in that the signs of pyramidal tract involvement mere asymmetrical, with concomitant asymmetry on MR images in one. In adul ts, Krabbe disease may present on MR imaging with selective pyramidal fiber involvement.