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Research ArticlePEDIATRICS
Open Access

MRI Characteristics of Globus Pallidus Infarcts in Isolated Methylmalonic Acidemia

E.H. Baker, J.L. Sloan, N.S. Hauser, A.L. Gropman, D.R. Adams, C. Toro, I. Manoli and C.P. Venditti
American Journal of Neuroradiology September 2014, DOI: https://doi.org/10.3174/ajnr.A4087
E.H. Baker
From the Department of Radiology and Imaging Sciences (E.H.B.), Clinical Center, and Genetics and Molecular Biology Branch (J.L.S., I.M., C.P.V.), Medical Genetics Branch (D.R.A.), and Undiagnosed Diseases Program (C.T.), National Human Genome Research Institute, National Institutes of Health, Bethesda, Maryland; Medical Genetics and Metabolism Department (N.S.H.), Children's Hospital Central California, Madera, California; and Department of Neurology (A.L.G.), Children's National Medical Center, Washington, DC.
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J.L. Sloan
From the Department of Radiology and Imaging Sciences (E.H.B.), Clinical Center, and Genetics and Molecular Biology Branch (J.L.S., I.M., C.P.V.), Medical Genetics Branch (D.R.A.), and Undiagnosed Diseases Program (C.T.), National Human Genome Research Institute, National Institutes of Health, Bethesda, Maryland; Medical Genetics and Metabolism Department (N.S.H.), Children's Hospital Central California, Madera, California; and Department of Neurology (A.L.G.), Children's National Medical Center, Washington, DC.
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N.S. Hauser
From the Department of Radiology and Imaging Sciences (E.H.B.), Clinical Center, and Genetics and Molecular Biology Branch (J.L.S., I.M., C.P.V.), Medical Genetics Branch (D.R.A.), and Undiagnosed Diseases Program (C.T.), National Human Genome Research Institute, National Institutes of Health, Bethesda, Maryland; Medical Genetics and Metabolism Department (N.S.H.), Children's Hospital Central California, Madera, California; and Department of Neurology (A.L.G.), Children's National Medical Center, Washington, DC.
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A.L. Gropman
From the Department of Radiology and Imaging Sciences (E.H.B.), Clinical Center, and Genetics and Molecular Biology Branch (J.L.S., I.M., C.P.V.), Medical Genetics Branch (D.R.A.), and Undiagnosed Diseases Program (C.T.), National Human Genome Research Institute, National Institutes of Health, Bethesda, Maryland; Medical Genetics and Metabolism Department (N.S.H.), Children's Hospital Central California, Madera, California; and Department of Neurology (A.L.G.), Children's National Medical Center, Washington, DC.
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D.R. Adams
From the Department of Radiology and Imaging Sciences (E.H.B.), Clinical Center, and Genetics and Molecular Biology Branch (J.L.S., I.M., C.P.V.), Medical Genetics Branch (D.R.A.), and Undiagnosed Diseases Program (C.T.), National Human Genome Research Institute, National Institutes of Health, Bethesda, Maryland; Medical Genetics and Metabolism Department (N.S.H.), Children's Hospital Central California, Madera, California; and Department of Neurology (A.L.G.), Children's National Medical Center, Washington, DC.
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C. Toro
From the Department of Radiology and Imaging Sciences (E.H.B.), Clinical Center, and Genetics and Molecular Biology Branch (J.L.S., I.M., C.P.V.), Medical Genetics Branch (D.R.A.), and Undiagnosed Diseases Program (C.T.), National Human Genome Research Institute, National Institutes of Health, Bethesda, Maryland; Medical Genetics and Metabolism Department (N.S.H.), Children's Hospital Central California, Madera, California; and Department of Neurology (A.L.G.), Children's National Medical Center, Washington, DC.
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I. Manoli
From the Department of Radiology and Imaging Sciences (E.H.B.), Clinical Center, and Genetics and Molecular Biology Branch (J.L.S., I.M., C.P.V.), Medical Genetics Branch (D.R.A.), and Undiagnosed Diseases Program (C.T.), National Human Genome Research Institute, National Institutes of Health, Bethesda, Maryland; Medical Genetics and Metabolism Department (N.S.H.), Children's Hospital Central California, Madera, California; and Department of Neurology (A.L.G.), Children's National Medical Center, Washington, DC.
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C.P. Venditti
From the Department of Radiology and Imaging Sciences (E.H.B.), Clinical Center, and Genetics and Molecular Biology Branch (J.L.S., I.M., C.P.V.), Medical Genetics Branch (D.R.A.), and Undiagnosed Diseases Program (C.T.), National Human Genome Research Institute, National Institutes of Health, Bethesda, Maryland; Medical Genetics and Metabolism Department (N.S.H.), Children's Hospital Central California, Madera, California; and Department of Neurology (A.L.G.), Children's National Medical Center, Washington, DC.
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Abstract

BACKGROUND: Bilateral infarcts confined to the globus pallidus are unusual and occur in conjunction with only a few disorders, including isolated methylmalonic acidemia, a heterogeneous inborn error of metabolism. On the basis of neuroradiographic features of metabolic strokes observed in a large cohort of patients with methylmalonic acidemia, we have devised a staging system for methylmalonic acidemia–related globus pallidus infarcts.

MATERIALS AND METHODS: Forty patients with isolated methylmalonic acidemia and neurologic symptoms underwent clinical brain MR imaging studies, which included 3D-T1WI. Infarcted globus pallidus segments were neuroanatomically characterized, and infarct volumes were measured.

RESULTS: Globus pallidus infarcts were present in 19 patients; all were bilateral, and most were left-dominant. A neuroanatomic scoring system based on the infarct patterns was devised; this revealed a 5-stage hierarchical susceptibility to metabolic infarct, with the posterior portion of the globus pallidus externa being the most vulnerable. Globus pallidus infarct prevalence by methylmalonic acidemia class was the following: cblA (5/7, 71%), cblB (3/7, 43%), mut° (10/22, 45%), and mut- (1/4, 25%). Tiny lacunar infarcts in the pars reticulata of the substantia nigra, previously unrecognized in methylmalonic acidemia, were found in 17 patients, 13 of whom also had a globus pallidus infarct.

CONCLUSIONS: The staged pattern of globus pallidus infarcts in isolated methylmalonic acidemia suggests a nonuniform, regionally specific cellular susceptibility to metabolic injury, even for patients having milder biochemical phenotypes. In support of this hypothesis, the delineation of lacunar infarcts in the pars reticulata of the substantia nigra, a tissue functionally and histologically identical to the globus pallidus interna, supports the concept of cell-specific pathology.

Abbreviations

bFFE
balanced fast-field echo
GP
globus pallidus
GPe
globus pallidus externa
GPi
globus pallidus interna
MMA
methylmalonic acidemia
MUT
methylmalonyl-coenzyme A mutase
SNr
pars reticulata of the substantia nigra
  • © 2015 American Society of Neuroradiology

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MRI Characteristics of Globus Pallidus Infarcts in Isolated Methylmalonic Acidemia
E.H. Baker, J.L. Sloan, N.S. Hauser, A.L. Gropman, D.R. Adams, C. Toro, I. Manoli, C.P. Venditti
American Journal of Neuroradiology Sep 2014, DOI: 10.3174/ajnr.A4087
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Cite this article
E.H. Baker, J.L. Sloan, N.S. Hauser, A.L. Gropman, D.R. Adams, C. Toro, I. Manoli, C.P. Venditti
MRI Characteristics of Globus Pallidus Infarcts in Isolated Methylmalonic Acidemia
American Journal of Neuroradiology Sep 2014, DOI: 10.3174/ajnr.A4087

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