+ Site Statistics
+ Search Articles
+ PDF Full Text Service
How our service works
Request PDF Full Text
+ Follow Us
Follow on Facebook
Follow on Twitter
Follow on LinkedIn
+ Subscribe to Site Feeds
Most Shared
PDF Full Text
+ Translate
+ Recently Requested

Hallervorden-Spatz syndrome in two siblings diagnosed by the clinical features and magnetic resonance imaging (MRI)

Hallervorden-Spatz syndrome in two siblings diagnosed by the clinical features and magnetic resonance imaging (MRI)

Journal of Korean Medical Science 8(5): 329-333

Hallervorden-Spatz syndrome (HSS) is a heredodegenerative disorder characterized by both progressive pyramidal and extrapyramidal signs, dysarthric speech, and mental deterioration. No diagnostic biochemical test is yet available, and diagnosis of HSS can be confirmed only at autopsy by the characteristic neuropathology including abnormal iron storage, disordered myelination, and loss of brain substance. We present two siblings with clinical features consistent with HSS, in whom magnetic resonance imaging (MRI) demonstrated the deposition of iron in the globus pallidus and the substantia nigra thus allowing an antemortem diagnosis of HSS.

Please choose payment method:

(PDF emailed within 0-6 h: $19.90)

Accession: 046210036

Download citation: RISBibTeXText

PMID: 8305140

DOI: 10.3346/jkms.1993.8.5.329

Related references

Hallervorden-Spatz syndrome in two siblings diagnosed by clinical features and magnetic resonance imaging. Journal of the Medical Association of Thailand 83(12): 1535-1540, 2000

Hallervorden-Spatz syndrome: clinical and magnetic resonance imaging correlations. Annals of Neurology 24(5): 692-694, 1988

Clinical neuropsychological and magnetic resonance imaging in hallervorden spatz syndrome. Annals of Neurology 22(1): 146-147, 1987

Magnetic resonance imaging and Hallervorden-Spatz syndrome. Cns Spectrums 8(6): 420, 2003

Dexmedetomidine for a patient with Hallervorden-Spatz syndrome during magnetic resonance imaging: a case report. Journal of Anesthesia 27(6): 963-964, 2013

Presence of the Eye-of-the-tiger Sign on Magnetic Resonance Imaging in a Subject of Atypical Hallervorden-Spatz Syndrome Lacking Pantothenate Kinase 2 Mutation. 2011

Follow-up magnetic resonance imaging in Hallervorden-Spatz disease. Clinical Neurology and Neurosurgery 94 Suppl: S57-S60, 1992

Nuclear magnetic resonance in the Hallervorden-Spatz syndrome. Digitale Bilddiagnostik 4(2): 66-68, 1984

A case of Hallervorden-Spatz disease with magnetic resonance imaging data. Revista de Investigacion Clinica; Organo del Hospital de Enfermedades de la Nutricion 50(3): 249-253, 1998

The role of magnetic resonance imaging in the diagnosis of Hallervorden-Spatz disease. Rinsho Shinkeigaku 30(9): 972-977, 1990

Hallervorden spatz disease antemortem diagnosis by magnetic resonance imaging. American Journal of Human Genetics 49(4 Suppl.): 170, 1991

Magnetic resonance imaging in pathologically proven Hallervorden-Spatz disease. Neurology 39(3): 440-442, 1989

Magnetic resonance imaging and differential diagnosis in hallervorden spatz disease. Nervenarzt 63(2): 105-107, 1992

Hallervorden Spatz syndrome: magnetic resonance findings. Case report. Arquivos de Neuro-Psiquiatria 62(3a): 730-732, 2004

Contribution of nuclear magnetic imaging in the diagnosis of Hallervorden-Spatz syndrome. Archives Francaises de Pediatrie 50(1): 35-37, 1993