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Elizabeth C. Dooling, MD; William C. Schoene, MD; Edward P. Richardson, Jr, MD

Arch Neurol. 1974;30(1):70-83.

Abstract
Two sisters with Hallervorden-Spatz syndrome (HSS) were treated with anti-Parkinson drugs. One showed the typical neuropathological lesions at death. The surviving sister has been treated with an iron-chelater without sustained effect, and with levodopa with improvement of motor abnormalities. Of 64 cases thought to represent HSS and studied post mortem, a group of 42 forms a distinct clinicopathologic entity. The remaining cases differ considerably from these, making their classification difficult at present. Hallervorden-Spatz syndrome may be the result of an inborn error of metabolism. Future studies of patients with HSS should include catecholamine analyses of cerebrospinal fluid and brain tissue and a search for viral infection, as iron studies have not been helpful.

Author Affiliations
Boston

From the Charles S. Kubik Laboratory for Neuropathology, James Homer Wright Pathology Laboratories, Massachusetts General Hospital, and the departments of neurology-neuro-pathology and pathology, Harvard Medical School, Boston.

Footnotes
Accepted for publication June 14, 1973.

Reprint requests to Massachusetts General Hospital, Boston, MA 02114 (Dr. Dooling).

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