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    MRI Characteristics of Globus Pallidus Infarcts in Isolated Methylmalonic Acidemia | American Journal of Neuroradiology  
    

















    





























    
  
  




    
    

  

      


    

      

  
  
  

    

  

    

  

                
    

      

  

    

  
      
  
  

    


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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 January 2015,  36 (1) 194-201; DOI: https://doi.org/10.3174/ajnr.A4087 

  
  
  



  


  
  



			

		

	
	
 	
	  
  

		
		

			

			  

  
      
  
  

    
  


  
  



  
      
  
  

    

  

    

  
      
  
  

    
References
  1. 1.↵


    1. Pagon  RA, 
    2. Adam  MP, 
    3. Bird  TD, et al.
    1. 

    2. Manoli  I, 
    3. Venditti  CP
    . Methylmalonic Acidemia. In: Pagon  RA, Adam  MP, Bird  TD, et al., eds. GeneReviews. Seattle: University of Washington, Seattle; 2005
  2. 2.↵


    1. 

    2. Rosenberg  LE, 
    3. Lilljeqvist  AC, 
    4. Hsia  YE
    . Methylmalonic aciduria: an inborn error leading to metabolic acidosis, long-chain ketonuria and intermittent hyperglycinemia. N Engl J Med 1968;278:1319–22
    CrossRefPubMedWeb of Science
  3. 3.↵


    1. 

    2. Matsui  SM, 
    3. Mahoney  MJ, 
    4. Rosenberg  LE
    . The natural history of the inherited methylmalonic acidemias. N Engl J Med 1983;308:857–61
    CrossRefPubMedWeb of Science
  4. 4.↵


    1. 

    2. Fenton  WA, 
    3. Gravel  RA, 
    4. Rosenblatt  DS
    . Disorders of Propionate and Methylmalonate Metabolism. New York: McGraw-Hill; 2013
  5. 5.↵


    1. 

    2. Willard  HF, 
    3. Rosenberg  LE
    . Inherited methylmalonyl CoA mutase apoenzyme deficiency in human fibroblasts: evidence for allelic heterogeneity, genetic compounds, and codominant expression. J Clin Invest 1980;65:690–98
    CrossRefPubMedWeb of Science
  6. 6.↵


    1. 

    2. Ando  T, 
    3. Rasmussen  K, 
    4. Wright  JM, et al
    . Isolation and identification of methylcitrate, a major metabolic product of propionate in patients with propionic acidemia. J Biol Chem 1972;247:2200–04
    Abstract/FREE Full Text
  7. 7.↵


    1. 

    2. Hauser  NS, 
    3. Manoli  I, 
    4. Graf  JC, et al
    . Variable dietary management of methylmalonic acidemia: metabolic and energetic correlations. Am J Clin Nutr 2011;93:47–56
    Abstract/FREE Full Text
  8. 8.↵


    1. 

    2. Touati  G, 
    3. Valayannopoulos  V, 
    4. Mention  K, et al
    . Methylmalonic and propionic acidurias: management without or with a few supplements of specific amino acid mixture. J Inherit Metab Dis 2006;29:288–98
    CrossRefPubMedWeb of Science
  9. 9.↵


    1. 

    2. Hörster  F, 
    3. Baumgartner  MR, 
    4. Viardot  C, et al
    . Long-term outcome in methylmalonic acidurias is influenced by the underlying defect (mut°, mut-, cblA, cblB). Pediatr Res 2007;62:225–30
    CrossRefPubMedWeb of Science
  10. 10.↵


    1. 

    2. Kruszka  PS, 
    3. Manoli  I, 
    4. Sloan  JL, et al
    . Renal growth in isolated methylmalonic acidemia. Genet Med 2013;15:990–96
    CrossRefPubMed
  11. 11.↵


    1. 

    2. O'Shea  CJ, 
    3. Sloan  JL, 
    4. Wiggs  EA, et al
    . Neurocognitive phenotype of isolated methylmalonic acidemia. Pediatrics 2012;129:e1541–51
    Abstract/FREE Full Text
  12. 12.↵


    1. 

    2. Bindu  PS, 
    3. Kovoor  JM, 
    4. Christopher  R
    . Teaching neuroimages: MRI in methylmalonic acidemia. Neurology 2010;74:e14
    CrossRef
  13. 13.↵


    1. 

    2. Campeau  PM, 
    3. Valayannopoulos  V, 
    4. Touati  G, et al
    . Management of West syndrome in a patient with methylmalonic aciduria. J Child Neurol 2010;25:94–97
    Abstract/FREE Full Text
  14. 14.↵


    1. 

    2. de Sousa  C, 
    3. Piesowicz  AT, 
    4. Brett  EM, et al
    . Focal changes in the globi pallidi associated with neurological dysfunction in methylmalonic acidaemia. Neuropediatrics 1989;20:199–201
    CrossRefPubMed
  15. 15.↵


    1. 

    2. Heidenreich  R, 
    3. Natowicz  M, 
    4. Hainline  BE, et al
    . Acute extrapyramidal syndrome in methylmalonic acidemia: “metabolic stroke” involving the globus pallidus. J Pediatr 1988;113:1022–27
    CrossRefPubMedWeb of Science
  16. 16.↵


    1. 

    2. Korf  B, 
    3. Wallman  JK, 
    4. Levy  HL
    . Bilateral lucency of the globus pallidus complicating methylmalonic acidemia. Ann Neurol 1986;20:364–66
    CrossRefPubMedWeb of Science
  17. 17.↵


    1. 

    2. Larnaout  A, 
    3. Mongalgi  MA, 
    4. Kaabachi  N, et al
    . Methylmalonic acidaemia with bilateral globus pallidus involvement: a neuropathological study. J Inherit Metab Dis 1998;21:639–44
    CrossRefPubMed
  18. 18.↵


    1. 

    2. Michel  SJ, 
    3. Given  CA  2nd., 
    4. Robertson  WC  Jr.
    . Imaging of the brain, including diffusion-weighted imaging in methylmalonic acidemia. Pediatr Radiol 2004;34:580–82
    PubMed
  19. 19.↵


    1. 

    2. Roodhooft  AM, 
    3. Baumgartner  ER, 
    4. Martin  JJ, et al
    . Symmetrical necrosis of the basal ganglia in methylmalonic acidaemia. Eur J Pediatr 1990;149:582–84
    CrossRefPubMedWeb of Science
  20. 20.↵


    1. 

    2. Trinh  BC, 
    3. Melhem  ER, 
    4. Barker  PB
    . Multi-slice proton MR spectroscopy and diffusion-weighted imaging in methylmalonic acidemia: report of two cases and review of the literature. AJNR Am J Neuroradiol 2001;22:831–33
    Abstract/FREE Full Text
  21. 21.↵


    1. 

    2. Yeşildağ  A, 
    3. Ayata  A, 
    4. Baykal  B, et al
    . Magnetic resonance imaging and diffusion-weighted imaging in methylmalonic acidemia. Acta Radiol 2005;46:101–03
    FREE Full Text
  22. 22.↵


    1. 

    2. Işikay  S, 
    3. Temel  L, 
    4. Keskin  M
    . Imaging findings associated with methylmalonic aciduria. Pediatr Neurol 2014;50:435–36
    CrossRefPubMed
  23. 23.↵


    1. 

    2. Sousa  R, 
    3. Hrinya Tannery  N, 
    4. Lafer  EM
    . Two new monoclonal antibodies provide immunohistochemical evidence for the unique biochemical similarity of the mouse globus pallidus, entopeduncular nucleus and substantia nigra pars reticulata. Neuroscience 1990;34:403–10
    CrossRefPubMedWeb of Science
  24. 24.↵


    1. 

    2. Nambu  A
    . Globus pallidus internal segment. Prog Brain Res 2007;160:135–50
    CrossRefPubMed
  25. 25.↵


    1. 

    2. McAuliffe  M, 
    3. Lalonde  FM, 
    4. McGarry  D, et al
    . Medical image processing, analysis and visualization in clinical research. In: Proceedings of the 14th IEEE Symposium on Computer-Based Medical Systems, Washington, DC. July 26–27, 2001:381–86
  26. 26.↵


    1. 

    2. Nambu  A
    . Somatotopic organization of the primate basal ganglia. Front Neuroanat 2011;5:26
    CrossRefPubMed
  27. 27.↵


    1. 

    2. Forno  LS
    . Reaction of the substantia nigra to massive basal ganglia infarction. Acta Neuropathol 1983;62:96–102
    CrossRefPubMedWeb of Science
  28. 28.↵


    1. 

    2. Nakane  M, 
    3. Teraoka  A, 
    4. Asato  R, et al
    . Degeneration of the ipsilateral substantia nigra following cerebral infarction in the striatum. Stroke 1992;23:328–32
    Abstract/FREE Full Text
  29. 29.↵


    1. 

    2. McKee  AC, 
    3. Levine  DN, 
    4. Kowall  NW, et al
    . Peduncular hallucinosis associated with isolated infarction of the substantia nigra pars reticulata. Ann Neurol 1990;27:500–04
    CrossRefPubMedWeb of Science
  30. 30.↵


    1. 

    2. François  C, 
    3. Grabli  D, 
    4. McCairn  K, et al
    . Behavioural disorders induced by external globus pallidus dysfunction in primates. II. Anatomical study. Brain 2004;127:2055–70
    Abstract/FREE Full Text
  31. 31.↵


    1. 

    2. Grabli  D, 
    3. McCairn  K, 
    4. Hirsch  EC, et al
    . Behavioural disorders induced by external globus pallidus dysfunction in primates. I. Behavioural study. Brain 2004;127:2039–54
    Abstract/FREE Full Text
  32. 32.↵


    1. 

    2. Kooistra  CA, 
    3. Heilman  KM
    . Motor dominance and lateral asymmetry of the globus pallidus. Neurology 1988;38:388–90
    CrossRef
  


  
  



  




  


  
  



  
      
  
  

    
  


  
  



  
      
  
  

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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 Jan 2015, 36 (1) 194-201; DOI: 10.3174/ajnr.A4087 

  
  
  



  


  
  



  
      
  
  

    

  
    
  
  
    
  
  
    
  


  


  
  



  
      
  
  

    
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