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MR Imaging of a Group I Case of Hallervorden-Spatz Disease
J Comput Assist Tomogr 12:851-853, Mutoh,K.,et al, 1988
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Article Abstract
Magnetic resonance(MR)imaging of a case of Hallervorden-Spatz disease(HSD) using a 1.5 T system is described.The patient showed progressive spastic diplegia with equinovarus deformity of the feet,dystonic postural movements,dysarthria,dysphagia,mental deterioration,optic nerve atrophy, and peripheral neuropathy.These clinical features were compatible with HDS.Symmetrical,decreased signal intensity was seen on both proton density weighted and T2-weighted spin echo images in the globus pallidus as well as in the substantia nigra(Group I).This MR finding suggests an increased iron deposition in these subcortical nuclei,which is characteristic of HSD.The characteristic MR imaging,together with the relevant clinical features,was considered to be useful for establishing the diagnosis of HSD.
 
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dysarthria
dysphagia
equinovarus
gait disorder
genetic neurologic disorders
globus pallidus
globus pallidus,lesion of
globus pallidus,lesion of,bilateral
Hallervorden Spatz disease
intellectual deterioration
iron,brain
MRI
MRI,abnormal
neuropathy
optic atrophy
spastic diplegia
substantia nigra
visual acuity,decreased

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