Document Detail


Hallervorden-Spatz disease: clinical and MRI study of 11 cases diagnosed in life.
MedLine Citation:
PMID:  1447570     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
The diagnosis of Hallervorden-Spatz disease (HSD) has usually been made post mortem, although the recent description of characteristic abnormalities in the globus pallidus has suggested the possibility of an in vivo diagnosis. We present the clinical histories, neurological features and MRI findings of 11 patients, diagnosed as having HSD. Generalized dystonia with predominance of oromandibular involvement, behavioural changes followed by dementia and retinal degeneration were present in all the patients. MRI pallidal abnormalities consisted of decreased signal intensity in T2-weighted images, compatible with iron deposits, and of a small area of hyperintensity in its internal segment ("eye of the tiger" sign). We propose that the combination of these neurological signs with these MRI findings could be considered as highly suggestive of a diagnosis of HSD in living patients.
Authors:
L Angelini; N Nardocci; V Rumi; C Zorzi; L Strada; M Savoiardo
Publication Detail:
Type:  Case Reports; Journal Article; Research Support, Non-U.S. Gov't    
Journal Detail:
Title:  Journal of neurology     Volume:  239     ISSN:  0340-5354     ISO Abbreviation:  J. Neurol.     Publication Date:  1992 Oct 
Date Detail:
Created Date:  1992-12-29     Completed Date:  1992-12-29     Revised Date:  2007-11-15    
Medline Journal Info:
Nlm Unique ID:  0423161     Medline TA:  J Neurol     Country:  GERMANY    
Other Details:
Languages:  eng     Pagination:  417-25     Citation Subset:  IM    
Affiliation:
Department of Child Neurology, Istituto Nazionale Neurologico C. Besta, Milan, Italy.
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MeSH Terms
Descriptor/Qualifier:
Adolescent
Adult
Child
Dystonia / diagnosis
Female
Follow-Up Studies
Globus Pallidus / radiography
Humans
Magnetic Resonance Imaging
Male
Neuropsychological Tests
Pantothenate Kinase-Associated Neurodegeneration / diagnosis*,  radiography
Retinal Degeneration / diagnosis
Tomography, X-Ray Computed

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