Neuropathologic characteristics of spinal cord lesions in sporadic Creutzfeldt-Jakob disease

We investigated the neuropathologic features of spinal cord lesions in 23 patients with sporadic Creutzfeldt-Jakob disease (sCJD), paying particular attention to neuronal loss and gliosis, pyramidal tract degeneration and prion protein (PrP) deposition. The study included 9 cases of subacute spongiform encephalopathy, 13 cases of panencephalopathic-type sCJD and 1 case of sporadic fatal insomnia (sFI). In the spinal gray matter, although gliosis was present in some patients with disease of relatively long duration, the number of neurons, including large motor neurons, was well preserved regardless of disease duration. Pyramidal tract degeneration was observed in some patients with disease lasting more than 14 months but not in the patient with sFI. PrP deposition was present in the spinal cord of all sCJD patients, and was identified predominantly in the posterior horn, particularly in the substantia gelatinosa, regardless of disease duration or disease classification based on cerebral pathology. Relatively prominent PrP deposition was also observed in Clarke's column. The density of PrP deposition in the sCJD spinal cord was not associated with disease duration or neuronal degeneration. Our results indicate that PrP deposition in the spinal cord is an early pathologic event in sCJD and may remain to the end stage. Although no VV1, VV2 or MV2 cases were included in our study, we suggest that stereotypic accumulation of PrP is a consistent pathologic feature of sCJD and that the spinal cord remains relatively resistant to the pathologic process of sCJD, at least in patients with MM1 sCJD.