Muscle biopsy was performed in two patients with Hallervorden-Spatz di
sease and increased serum creatine kinase levels, Morphological analys
is showed myopathic signs such as subsarcolemmal accumulation of myelo
id structures, dense bodies and debris, endomysial macrophage activati
on, focal necrosis and fiber splitting, We emphasize the finding of mu
scle involvement in Hallervorden-Spatz disease, like in other forms of
neuroacanthocytosis.