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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

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
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/1817-1745.92845

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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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