Hallervorden-Spatz Disease

HALLERWARDEN-SPATZ DISEASE (Hallervorden, Spatz), a special type of familial extrapyramidal disease, characterized by gradually developing changes in the shape of the foot in the sense of pes varus and slowly developing immobility of the lower extremities (sometimes also of the trunk muscles, neck, and upper extremities), leading, in the end, to contractures. Subsequently, speech disorders and progressive dementia develop, sometimes also atrophy of certain muscles (peroneal and interosseous), choreatic and athetoid movements, and swallowing disorders. Pyramidal symptoms are not observed. The disease develops at the age of 8-10 years; its duration is 8-12 years. Anatomically-pathologically, characteristic dark brown, rust-colored staining of the globi pallidi and the zona reticularis of the substantia nigra was found, without clearly visible other changes in these parts; furthermore, microscopically-intense reaction to iron in the specified centers; then deposition of pigment, changes in nerve cells, swelling of axis cylinders, significant increase in glia, and development of large cells with large pale nuclei. In addition, in the cortex, changes in ganglion cells (mainly in the 3rd layer).