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infection (e.g., encephalitis, meningitis, sepsis), toxic ingestion, possible nonaccidental injury, and inborn errors of metabolism (IEMs). Imaging Differential Diagnoses In terms of IEMs, bilateral basal ganglia T2 hyperintensity raises the possibility of a primary mitochondrial disorder, pyruvate dehydrogenase complex deficiency, biotin-thiamine-responsive basal ganglia disease

infection (e.g., encephalitis, meningitis, sepsis), toxic ingestion, possible nonaccidental injury, and inborn errors of metabolism (IEMs). Imaging Differential Diagnoses In terms of IEMs, bilateral basal ganglia T2 hyperintensity raises the possibility of a primary mitochondrial disorder, pyruvate dehydrogenase complex deficiency, biotin-thiamine-responsive basal ganglia disease

infection (e.g., encephalitis, meningitis, sepsis), toxic ingestion, possible nonaccidental injury, and inborn errors of metabolism (IEMs). Imaging Differential Diagnoses In terms of IEMs, bilateral basal ganglia T2 hyperintensity raises the possibility of a primary mitochondrial disorder, pyruvate dehydrogenase complex deficiency, biotin-thiamine-responsive basal ganglia disease

infection. Sagittal T1 shows mild global volume loss and ex vacuo dilation of the prepontine cistern and fourth ventricle (arrows). (B) Axial T2 shows patchy edema in the bifrontal subcortical white matter (arrows), with crowding of sulci and basal cisterns. (C) Sagittal T1 with severe cerebral edema

infection. Sagittal T1 shows mild global volume loss and ex vacuo dilation of the prepontine cistern and fourth ventricle (arrows). (B) Axial T2 shows patchy edema in the bifrontal subcortical white matter (arrows), with crowding of sulci and basal cisterns. (C) Sagittal T1 with severe cerebral edema

infection. Sagittal T1 shows mild global volume loss and ex vacuo dilation of the prepontine cistern and fourth ventricle (arrows). (B) Axial T2 shows patchy edema in the bifrontal subcortical white matter (arrows), with crowding of sulci and basal cisterns. (C) Sagittal T1 with severe cerebral edema

infection is usually asymptomatic or mild in children with mainly respiratory symptoms. However,Page 213Page

infection is usually asymptomatic or mild in children with mainly respiratory symptoms. However,Page 213Page

infection is usually asymptomatic or mild in children with mainly respiratory symptoms. However,Page 213Page

infections (e.g., parechovirus), infant formula preparation errors/hypernatremia, and IEMs including MSUD, glycine encephalopathy, decompensated urea cycle disorders. Final Diagnosis Maple syrup urine disease. Discussion Maple syrup urine disease results from dysfunction of the branched-chain amino acid dehydrogenase complex. It has an autosomal recessive inheritance