Hallervorden-Spatz Disease: 2 Cases of Siblings.
10.3348/jkrs.1994.30.4.779
- Author:
Woo Sun KIM
;
In One KIM
;
Kyung Mo YEON
;
Jong Gi SONG
- Publication Type:Case Report
- MeSH:
Child;
Dysarthria;
Female;
Gait Disorders, Neurologic;
Globus Pallidus;
Humans;
Male;
Pantothenate Kinase-Associated Neurodegeneration*;
Siblings*;
Substantia Nigra;
Tremor
- From:Journal of the Korean Radiological Society
1994;30(4):779-781
- CountryRepublic of Korea
- Language:Korean
-
Abstract:
We report two patients with Hallervorden-Spatz disease, who were diagnosed by same MR findings of marked low signal intensity in the globus and substantia nigra. They presented with ataxic and spastic gait, intention tremor, delayed mental development, and dysarthria. They were 7 year-old male and 8 yea r-old female siblings, who were healthy until 3 years of age when they suffered from progressive symptoms. T2-weighted images showed marked low signal intensity in the globus pallidus and substantia nigra indicating an increased irondeposition, and it might suggest Hallervorden-Spatz disease.
