| CASE REPORT |
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| Year : 2011 | Volume
: 6
| Issue : 2 | Page : 146-148 |
Striatal necrosis in type 1 glutaric aciduria: Different stages in two siblings
Anitha Sen1, Rajesh Subramonia Pillay2
1 Department of Radiodiagnosis, Devi Scans, Medical College PO, Thiruvananthapuram, Kerala, India
2 Department of Radiodiagnosis, Kerala Institute of Medical Sciences, Thiruvananthapuram, Kerala, India
Correspondence Address:
Anitha Sen
Department of Radiodiagnosis, Devi Scans, Medical College PO, Thiruvananthapuram - 695 011, Kerala
India
 Source of Support: None, Conflict of Interest: None  | Check |
DOI: 10.4103/1817-1745.92845
Two siblings born of a consanguineous marriage with history of neurologic deterioration were imaged. Imaging features are classical of glutaric aciduria type 1 (GA-1), acute (striatal necrosis) stage in younger sibling, and chronic stage in older sibling. GA-1 is an autosomal recessive disease with typical imaging features. Greater awareness about this condition among clinicians and radiologists is essential for early diagnosis and prevention of its catastrophic consequences. Striatal necrosis with stroke-like signal intensity on imaging correlates with clinical stage of patients.
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