Adult-onset Hallervorden-Spatz syndrome presenting as cortical dementia

Citation
Ge. Cooper et al., Adult-onset Hallervorden-Spatz syndrome presenting as cortical dementia, ALZ DIS A D, 14(2), 2000, pp. 120-126
Citations number
24
Language
INGLESE
art.tipo
Article
Categorie Soggetti
Neurology,"Neurosciences & Behavoir
Journal title
ALZHEIMER DISEASE & ASSOCIATED DISORDERS
ISSN journal
0893-0341 → ACNP
Volume
14
Issue
2
Year of publication
2000
Pages
120 - 126
Database
ISI
SICI code
0893-0341(200004/06)14:2<120:AHSPAC>2.0.ZU;2-J
Abstract
The authors examined behavioral and pathophysiologic substrates in a patien t with adult-onset Hallervorden-Spatz syndrome who presented with insidious cognitive decline but no motor impairment. The authors combined longitudin al case history and serial neuropsychologic testing with functional neuroim aging (positron emission tomography), structural neuroimaging (magnetic res onance imaging), and brain tissue analyses. Serial assessments of a 29-year -old woman showed progressive dementia. Marked cognitive and behavioral def icits were seen on neuropsychologic testing, corresponding to striking cort ical abnormalities on positron emission tomography, magnetic resonance imag ing, and histopathologic studies. Typical motor manifestations of the disor der did not emerge until the patient was 34 years old, 5 years after the on set of cognitive symptoms. Hallervorden-Spatz syndrome should be considered in the differential diagnosis of progressive cortical dementia in a young adult, even in the absence of motor dysfunction.