Hallervorden-Spatz syndrome

K. F. Swaiman

Research output: Contribution to journalReview articlepeer-review

1 Scopus citations

Abstract

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.

Original languageEnglish (US)
Pages (from-to)148-152
Number of pages5
JournalInternational Pediatrics
Volume5
Issue number2
StatePublished - 1990

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