Hallervorden-Spatz syndrome

Despite the massive iron deposition in the globus pallidus and substantia nigra and the likely autosomal recessive genetic transmission of Hallervorden-Spatz syndrome (HSS), the basic pathophysiology is still unknown. There is no specific treatment. However, therapy with levodopa/carbidopa or bromocriptine is often effective for controlling the dystonic posture and in less degree tremors. No therapy for intellectual deterioration is of value. The clinical course is variable; the mean duration of illness is 11 years. Some children have a rapidly progressive course and die within 2 years of onset.