Iron accumulation in the substantia nigra of autosomal recessive juvenile parkinsonism (ARJP).

Autosomal recessive juvenile parkinsonism (ARJP) is a distinct clinical and genetic entity characterized by highly selective neuronal death in the substantia nigra (SN) and locus coeruleus neurons without Lewy body formation. The mechanism of neuronal death of ARJP is still unknown. Our study demonstrated that iron staining was more intense in ARJP than in both controls and sporadic Parkinson's disease (PD), and there were differences in the pattern of distribution of iron staining between ARJP and PD. In addition neurites of SN in ARJP showed intense iron staining. Thus, we postulate that oxidative stress may play an important role in the neurodegeneration that occurs in ARJP.