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Year : 2016  |  Volume : 26  |  Issue : 1  |  Page : 131-134
Atypical MR lenticular signal change in infantile isovaleric acidemia


1 Department of Pediatric Radiology, Government Medical College, Srinagar, Jammu and Kashmir, India
2 Department of Pediatrics, Government Medical College, Srinagar, Jammu and Kashmir, India
3 Department of Radiology, Government Medical College, Srinagar, Jammu and Kashmir, India

Correspondence Address:
Dr. Nisar A Wani
Department of Pediatric Radiology, Government Medical College, Srinagar, Jammu and Kashmir
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0971-3026.178362

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Isovaleric acidemia (IVA) is an inborn error of branched chain amino acid metabolism that may manifest as acute neonatal metabolic acidosis or as chronic intermittent form with developmental delay or recurrent episodes of acute metabolic acidosis. Early diagnosis is the key to prevent morbidity and mortality. Brain imaging abnormalities are rarely described in IVA. We report a case of chronic intermittent IVA with acute presentation in a 4-month-old infant who presented with acute metabolic acidosis. Brain magnetic resonance imaging (MRI) revealed symmetric signal intensity changes in bilateral lentiform nuclei with an unreported T1-weighted (T1W) symmetric hyperintense ring-like appearance in bilateral putamen.


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