Alpha-Synuclein (originally called precursor of the non-Abeta component of
Alzheimer's disease amyloid-NACP) is a presynaptic nerve terminal
protein and is now known to be a major component of Lewy bodies (LBs) in
Parkinson's disease. Previous studies have shown that LBs are occasionally found in patients with
Hallervorden-Spatz disease (HSD), a hereditary or sporadic
neuroaxonal dystrophy. Therefore, an immunocytochemical examination of the brain tissues from two patients with HSD for
alpha-synuclein/NACP was performed. In both cases, LBs were observed in the substantia nigra, locus ceruleus and other subcortical nuclei. These LBs were strongly immunolabelled with anti-
alpha-synuclein/NACP. Moreover, abnormal
alpha-synuclein/NACP-immunoreactive structures in the neuronal somata and processes were found in the cerebral neocortex, hippocampus, basal ganglia, thalamus, pontine and inferior olivary nuclei, spinal grey matter, and peripheral sympathetic ganglia. Although numerous dystrophic axons (spheroids) were found throughout the brain, either none or only a few were positive for
alpha-synuclein/NACP. These findings suggest that widespread accumulation of
alpha-synuclein/NACP is a pathological feature in patients suffering from HSD with LBs, and that this phenomenon is unrelated to axonal spheroid formation.