Predominant Spastic Paraparesis Associated With the D178N Mutation in PRNP

Spastic paraparesis is a very rare manifestation of prion diseases, occurring in both sporadic prion disease1 and inherited prion disease.2 The D178N mutation in the prion protein (PRNP) gene is associated with familial fatal insomnia (FFI) or familial Creutzfeldt-Jakob disease (fCJD),3 and in some cases, FFI and fCJD overlap.4,5 Herein, we describe a patient harboring the D178N mutation in PRNP with initially predominant spastic paraparesis.

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