Sensorimotor neuropathy in late-onset Krabbe disease progressing over 40 years after onset

We describe the case of a 51-year-old woman with Krabbe disease presenting with progressive spastic paraparesis since she was 10 years-of-age. At the age of 51 years, neurological evaluation showed progression of her sensorimotor neuropathy along with deterioration of nerve conduction, whereas no apparent progression of the bilateral pyramidal tract lesions was observed by magnetic resonance imaging. Sensorimotor neuropathy progresses over several decades in its long-term natural course in contrast to the absence of apparent progression of lesions in the central motor pathways.