Cranial MRI changes may precede symptoms in Hallervorden-Spatz syndrome

Hayflick, Susan J.; Penzien, Johann M.; Michl, Wolfgang; Sharif, Uzma M.; Rosman, N.Paul; Wheeler, Patricia G.

« Previous Next »Pediatric Neurology
Volume 25, Issue 2 , Pages 166-169, August 2001

Cranial MRI changes may precede symptoms in Hallervorden-Spatz syndrome

Susan J. Hayflick, MD
- Department of Molecular and Medical Genetics; Oregon Health Sciences University; Portland, OR, USA
- Department of Pediatrics; Oregon Health Sciences University; Portland, OR, USA
- Department of Neurology; Oregon Health Sciences University; Portland, OR, USA
- Communications should be addressed to: Dr. Hayflick; Oregon Health and Science University; Mail Code L103a; 3181 SW Sam Jackson Park Rd.; Portland, OR 97201
Johann M. Penzien, MD
- Division of Neuropediatrics; Zentralklinikum; Augsburg, Germany
Wolfgang Michl, MD
- Division of Pediatric Radiology; Zentralklinikum; Augsburg, Germany
Uzma M. Sharif, MD
- Department of Neurology; Tufts University School of Medicine; New England Medical Center; Boston, Massachusetts, USA
N.Paul Rosman, MD
- Department of Neurology; Tufts University School of Medicine; New England Medical Center; Boston, Massachusetts, USA
- Department of Pediatrics; Tufts University School of Medicine; New England Medical Center; Boston, Massachusetts, USA
Patricia G. Wheeler, MD
- Department of Pediatrics; Tufts University School of Medicine; New England Medical Center; Boston, Massachusetts, USA

Received 20 December 2000; accepted 3 April 2001.

Abstract

Two families are presented in which siblings of children affected with Hallervorden-Spatz syndrome exhibited characteristic cranial magnetic resonance imaging changes before developing clinical features of the disease. Linkage to a major locus on chromosome 20p supported the diagnosis of Hallervorden-Spatz syndrome. In some patients with Hallervorden-Spatz syndrome, iron is radiographically evident before the onset of clinical symptoms.