title: Proton magnetic resonance spectroscopy in Steele-Richardson-Olszewski syndrome.
abstract: Proton magnetic resonance spectroscopy, localized to the lentiform nucleus, was carried out in nine patients with a clinical diagnosis of Steele-Richardson-Olszewski syndrome (SRO) and in eight healthy age-matched controls. Three of the nine SRO patients had a so-called "eye of the tiger sign" with high signal in the globus pallidus surrounded by a ring of low signal on T2 weighted magnetic resonance imaging previously only reported in Hallervorden-Spatz disease. One of these patients had pathologically proven SRO at postmortem. The SRO group showed a significant reduction in the median concentration from N-acetyl groups (median, 6.87 mM; range, 4.92-10.59 mM; p < 0.015) compared with the control group (median, 9.85 mM; range, 9.26-11.0 mM). The N-acetylaspartate concentration was significantly reduced in seven of the nine patients studied. The reduction of the N-acetylaspartate-creatine ratio from the lentiform nucleus in the SRO group may reflect neuronal loss, occurring predominantly in the globus pallidus. Proton magnetic resonance spectroscopy may be a useful, noninvasive technique to help differentiate the various parkinsonian syndromes.
authors.affiliation: National Hospital for Neurology and Neurosurgery, London, U.K.
authors.forename: C A
authors.initials: CA
authors.lastname: Davie
authors.forename: G J
authors.initials: GJ
authors.lastname: Barker
authors.forename: C
authors.initials: C
authors.lastname: Machado
authors.forename: D H
authors.initials: DH
authors.lastname: Miller
authors.forename: A J
authors.initials: AJ
authors.lastname: Lees
mesh_terms: D000328:Adult; D000368:Aged; D001224:Aspartic Acid / Q000031:analogs & derivatives* / Q000032:analysis; D001479:Basal Ganglia / Q000737:chemistry / Q000473:pathology*; D016022:Case-Control Studies; D002794:Choline / Q000032:analysis; D003401:Creatine / Q000032:analysis; D003937:Diagnosis, Differential; D005917:Globus Pallidus / Q000473:pathology; D006801:Humans; D008279:Magnetic Resonance Imaging; D009682:Magnetic Resonance Spectroscopy; D008875:Middle Aged; D013494:Supranuclear Palsy, Progressive / Q000175:diagnosis*
chemical_list: D001224:Aspartic Acid; C000179:N-acetylaspartate; D003401:Creatine; D002794:Choline