1naresh
Array
(
    [urn:ac.highwire.org:guest:identity] => Array
        (
            [runtime-id] => urn:ac.highwire.org:guest:identity
            [type] => guest
            [service-id] => ajnr-ac.highwire.org
            [access-type] => Controlled
            [privilege] => Array
                (
                    [urn:ac.highwire.org:guest:privilege] => Array
                        (
                            [runtime-id] => urn:ac.highwire.org:guest:privilege
                            [type] => privilege-set
                            [privilege-set] => GUEST
                        )

                )

            [credentials] => Array
                (
                    [method] => guest
                )

        )

)
1naresh
Array
(
    [urn:ac.highwire.org:guest:identity] => Array
        (
            [runtime-id] => urn:ac.highwire.org:guest:identity
            [type] => guest
            [service-id] => ajnr-ac.highwire.org
            [access-type] => Controlled
            [privilege] => Array
                (
                    [urn:ac.highwire.org:guest:privilege] => Array
                        (
                            [runtime-id] => urn:ac.highwire.org:guest:privilege
                            [type] => privilege-set
                            [privilege-set] => GUEST
                        )

                )

            [credentials] => Array
                (
                    [method] => guest
                )

        )

)
1naresh
Array
(
    [urn:ac.highwire.org:guest:identity] => Array
        (
            [runtime-id] => urn:ac.highwire.org:guest:identity
            [type] => guest
            [service-id] => ajnr-ac.highwire.org
            [access-type] => Controlled
            [privilege] => Array
                (
                    [urn:ac.highwire.org:guest:privilege] => Array
                        (
                            [runtime-id] => urn:ac.highwire.org:guest:privilege
                            [type] => privilege-set
                            [privilege-set] => GUEST
                        )

                )

            [credentials] => Array
                (
                    [method] => guest
                )

        )

)
1naresh
Array
(
    [urn:ac.highwire.org:guest:identity] => Array
        (
            [runtime-id] => urn:ac.highwire.org:guest:identity
            [type] => guest
            [service-id] => ajnr-ac.highwire.org
            [access-type] => Controlled
            [privilege] => Array
                (
                    [urn:ac.highwire.org:guest:privilege] => Array
                        (
                            [runtime-id] => urn:ac.highwire.org:guest:privilege
                            [type] => privilege-set
                            [privilege-set] => GUEST
                        )

                )

            [credentials] => Array
                (
                    [method] => guest
                )

        )

)
1naresh
Array
(
    [urn:ac.highwire.org:guest:identity] => Array
        (
            [runtime-id] => urn:ac.highwire.org:guest:identity
            [type] => guest
            [service-id] => ajnr-ac.highwire.org
            [access-type] => FreeToRead
            [privilege] => Array
                (
                    [urn:ac.highwire.org:guest:privilege] => Array
                        (
                            [runtime-id] => urn:ac.highwire.org:guest:privilege
                            [type] => privilege-set
                            [privilege-set] => GUEST
                        )

                )

            [credentials] => Array
                (
                    [method] => guest
                )

        )

)


  
    





















































































































    Proton MR Spectroscopy of Mitochondrial Diseases: Analysis of Brain Metabolic Abnormalities and Their Possible Diagnostic Relevance | American Journal of Neuroradiology  
    



















    





























    
  
  




    
    

  

      


    

      

  
  
  

    

  

    

  

                
    

      

  

    

  
      
  
  

    


Advertisement



  



  


  
  



  



    

  


  


  


  
  
  
  
  
  

  	      


    
      

    
  
    
        
    

  

    
                        

  

                
    

      

	  

		
		

			

			  

  
      
  
  

    

  
  
      
Research ArticleBRAIN

  
      

  
      
Proton MR Spectroscopy of Mitochondrial Diseases: Analysis of Brain Metabolic Abnormalities and Their Possible Diagnostic Relevance
  
    	
M. Cristina Bianchi, Michela Tosetti, Roberta Battini, Maria L. Manca, Michelangelo Mancuso, Giovanni Cioni, Raffaello Canapicchi and Gabriele Siciliano

  
    	
American Journal of Neuroradiology November 2003,  24 (10) 1958-1966; 

  
  
  



  


  
  



			

		

	
	
 	
	  
  

		
		

			

			  

  
      
  
  

    
  


  
  



  
      
  
  

    

  

    

  
      
  
  

    
References
  1. ↵
    Jackson MJ, Schaefer JA, Johnson MA, Morris AAM, Turnbull DM, Bindoff LA. Presentation and clinical investigation of mitochondrial respiratory chain disease: a study of 51 patients. Brain 1995;118:339–357
    Abstract/FREE Full Text
  2. Chimmery PF, Turnbull DM. Clinical features, investigation, and management of patients with defects of mitochondrial DNA. J Neurol Neurosurg Psychiatry 1997;63:559–563
    FREE Full Text
  3. ↵
    Di Mauro S, Bonilla E, De Vivo DC. Does the patient have a mitochondrial encephalomyopathy? J Child Neurol 1999;14:23–35
  4. ↵
    Moats RA, Watson L, Shonkt T, et al. Added value of automated clinical proton MR spectroscopy of the brain. J Comput Assist Tomogr 1995;19:480–491
    PubMed
  5. ↵
    Wang ZJ, Zimmermann RA. Proton MR spectroscopy of pediatric brain metabolic disorders. Neuroimaging Clin North Am 1998;8:781–807
  6. ↵
    Grodd W, Krageloh-Mann I, Petersen D, Trefz FK, Harzer K. In vivo assessment of N-acetylaspartate in brain spongy degeneration (Canavan’s disease) by proton spectroscopy. Lancet 1990;336:437–438
    PubMedWeb of Science
  7. ↵
    Stöckler S, Hanefeld F, Frahm J. Creatine replacement therapy in guanidinoacetate methyltransferase deficiency, a novel inborn error of metabolism. Lancet 1996;348:789–790
    CrossRefPubMedWeb of Science
  8. ↵
    Bianchi MC, Tosetti M, Fornai F, et al. Reversible brain creatine deficiency in two sisters with normal blood creatine level. Ann Neurol 2000;47:511–513
    CrossRefPubMedWeb of Science
  9. ↵
    Kruse B, Barker PB, Van Zijl PC, Duyn JH, Moonen CT, Moser HW. Multislice proton magnetic resonanace spectroscopic imaging in X-linked adrenoleukodystrophy. Ann Neurol 1994;36:595–608
    CrossRefPubMedWeb of Science
  10. ↵
    Ross BD, Jacobson S, Villamil F, et al. Subclinical hepatic encephalopathy: proton MR spectroscopic abnormalities. Radiology 1994;193:457–463
    PubMedWeb of Science
  11. ↵
    Detre JA, Wang Z, Bogdan AR, et al. Regional variation in brain lactate in Leigh syndrome by localized 1H magnetic resonance spectroscopy. Ann Neurol 1991;29:218–221
    CrossRefPubMedWeb of Science
  12. Matthews PM, Andermann F, Silver K, Carpati G, Arnold DL. Proton MR spectroscopic characterization of differences in regional brain metabolic abnormalities in mitochondrial encephalomyopathies. Neurology 1993;43:2484–2490
    Abstract/FREE Full Text
  13. ↵
    Barkovich AJ, Good WV, Koch TK, Berg BO. Mitochondrial disorders: analysis of their clinical and imaging characteristics. AJNR Am J Neuroradiol 1993;14:1119–1137
    Abstract/FREE Full Text
  14. Kuwabara T, Watanabe H, Tanaka K, et al. Mitochondrial encephalomyopathy: elevated visual cortex lactate unresponsive to photic stimulation—a localized 1H-MRS study. Neurology 1994;44:557–559
    Abstract/FREE Full Text
  15. Castillo M, Kwock L, Green C. MELAS syndrome: imaging and proton MR spectroscopic findings. AJNR Am J Neuroradiol 1995;16:233–239
    Abstract/FREE Full Text
  16. Clark JM, Marks MP, Adalsteinsson E, et al. MELAS: clinical and pathologic correlations with MRI, xenon/CT and MR spectroscopy. Neurology 1996;46:223–227
    Abstract/FREE Full Text
  17. Kapellar P, Fazekas F, Offenbacher H, et al. Magnetic resonance imaging and spectroscopy of progressive cerebral involvement in Kearns-Sayre syndrome. J Neurol Sci 1996;135:126–130
    CrossRefPubMedWeb of Science
  18. ↵
    Pavlakis SG, Kingsley PB, Kaplan GP, Stacpoole PW, Oshea M, Lustbader D. Magnetic resonance spectroscopy: use in monitoring MELAS treatment. Arch Neurol 1998;55:849–852
    CrossRefPubMedWeb of Science
  19. ↵
    Mancuso M, Bianchi MC, Santorelli FM, et al. Encephalomyopathy with multiple mitochondrial DNA deletions and multiple symmetric lipomatosis: further evidence of a possible association. J Neurol 1999;246:1197–1198
    PubMed
  20. ↵
    Santorelli FM, Siciliano G, Casali C, et al. Mitochondrial tRNA (Cys) gene mutation (A5814G): a second family with mitochondrial encephalopathy. Neuromusc Disord 1997;7:156–159
    CrossRefPubMedWeb of Science
  21. ↵
    Webb PG, Sailasuta N, Kohler SJ, et al. Automated single-voxel proton MRS: technical development and multisite verification. Magn Reson Med 1994;31:365–373
    PubMedWeb of Science
  22. ↵
    Mascalchi M, Tosetti M, Plasmati R, et al. Proton magnetic resonance spectroscopy in an Italian family with spinocerebellar ataxia type 1. Ann Neurol 1998;43:244–252
    CrossRefPubMedWeb of Science
  23. ↵
    Bianchi MC, Tosetti M, Moretti P, et al. Evaluation of herpes encephalitis and diffuse astrocytoma by means of magnetic resonance imaging and proton magnetic resonance spectroscopy. Int J Neuroradiol 1999;5:16–21
  24. ↵
    Sparaco M, Bonilla E, Di Mauro S, Powers JM. Neuropathology of mitochondrial encephalomyopathies due to mitochondrial DNA defects. J Neuropathol Exp Neurol 1993;52:1–10
    CrossRefPubMedWeb of Science
  25. ↵
    Matthews PM, Tampieri D, Berkovic SF, et al. Magnetic resonance imaging shows specific abnormalities in the MELAS syndrome. Neurology 1991;41:1043–1046
    Abstract/FREE Full Text
  26. Valanne L, Ketonen L, Majander A, Suomalainen A, Pihko H. Neuroradiologic findings in children with mitochondrial disorders. AJNR Am J Neuroradiol 1998;19:369–377
    Abstract
  27. ↵
    Wray SH, Provenzale JM, Johns DR, Thulborn KR. MR of the brain in mitochondrial myopathy. AJNR Am J Neuroradiol 1995;16:1167–1173
    Abstract
  28. ↵
    Ross BD, Blum S. Neurospectroscopy. In: Greenberg JO, ed. Neuroimaging. McGraw-Hill Companies Inc.;1999 :727–774
  29. ↵
    Lopez-Villegas D, Lenkinski RE, Wehrli SL, Ho WZ, Douglas SD. Lactate production by human monocytes/macrophages determined by proton MR spectroscopy. Magn Reson Med 1995;34:32–38
    PubMed
  30. ↵
    Bitsch A, Bruhn H, Vougioukas V, et al. Inflammatory CNS demyelination: histopathologic correlation with in vivo quantitative proton MR spectroscopy. AJNR Am J Neuroradiol 1999;20:1619–1627
    Abstract/FREE Full Text
  31. ↵
    Ross BD. A biochemistry primer for neuroradiologist. In: Advanced Imaging Symposium: Preparing the Neuroradiologist for the New Millenium. Oak Brook, IL: American Society of Neuroradiology,2000 :13–27
  32. ↵
    Urenjak J, Wiliams SR, Gadian DG, Noble M. Proton nuclear magnetic resonance spectroscopy unambigously identifies differentneuronal cell types. J Neurosci 1993;13:981–989
    Abstract
  33. ↵
    Vion-Dury J, Nicoli P, Salvan AM, et al. Reversal of brain metabolic alterations with zidovudine detected by proton localized magnetic resonance spectroscopy. Lancet 1995;345:60–61
    PubMed
  34. ↵
    Agris PF, Campbell ID. Proton nuclear magnetic resonance of intact friend leukemia cells: phosphorylcholine increase during differentiation. Science 1982;216:1325–1327
    Abstract/FREE Full Text
  35. ↵
    De Stefano N, Matthews PM, Ford B, Genge A, Karpati G, Arnold DL. Short-term dichloroacetate treatment improves indices of cerebral mitochondrial disorders. Neurology 1995;45:1193–1198
    Abstract/FREE Full Text
  36. ↵
    Bruhn H, Kruse B, Korenke GC, et al. Proton NMR Spectroscopy of cerebral metabolic alterations in infantile peroxisomal disorders. J Comput Assist Tomogr 1992;16:335–344
    PubMedWeb of Science
  37. Kuesel AC, Donnelly SM, Halliday W. Mobile lipids and metabolic heterogeneity of brain tumors as detectable by ex vivo 1H-MR spectroscopy. NMR Biomed 1994;7:172–180
    PubMed
  38. ↵
    Kuesel AC, Sutherland GR, Halliday W. 1H-MR spectroscopy of high grade astrocytomas: mobile lipid accumulation in necrotic tissue. NMR Biomed 1994;7:49–55
  39. ↵
    Felber SR, Sperl W, Chemelli A, Murr C, Wendel U. Maple syrup urine disease: metabolic decompensation monitored by proton magnetic resonance imaging and spectroscopy. Ann Neurol 1993;33:396–401
    PubMed
  40. ↵
    Patel TB, Debuysere MS, Barron LL, et al. Studies on the regulation of the branched chain alpha-keto acid dehydrogenase in the perfused rat liver. J Biol Chem 1981;256:9009–9015
    Abstract/FREE Full Text
  41. Waymack WW, DebuysereMS, Olson MS. Studies on the activation and inactivation of the branched chain alpha-keto acid dehydrogenase in the perfused rat heart. J Biol Chem 1980;255:9773–9781
    Abstract/FREE Full Text
  42. Matuda S, Kitano A, Sakaguchi Y, et al. Pyruvate dehydrogenase subcomplex with lipoamide dehydrogenase deficiency in a patient with lactic acidosis and branched chain ketoaciduria. Clin Chim Acta 1984;140:59–64
    CrossRefPubMedWeb of Science
  43. ↵
    Taylor J, Robinson BH, Sherwood WG. A defect in branched-chain aminoacid metabolism in a patient with congenital lactic acidosis due to dihydrolipoyl dehydrogenase deficiency. Pediatr Res 1978;12:60–62
    PubMedWeb of Science
  44. ↵
    Craigen WJ. Leigh disease with deficiency of lipoamide dehydrogenase: treatment with dichloroacetate. Pediatr Neurol 1996;4:69–71
  45. ↵
    Larsson NG, Luft R. Revolution in mitochondrial medicine. FEBS Lett 1999;455:199–202
    CrossRefPubMedWeb of Science
  


  
  



  




  


  
  



  
      
  
  

    
  


  
  



  
      
  
  

     Back to top  


  
  



			

		

		
		

			

			  

  
        
In this issue

    
  
  

    
  


  
  



  
      
  
  

    

  

    

  
      
  
  

    


Advertisement



  



  


  
  



  



  


  
  



  
      
  
  

    

	  
  

		

          

            

  
      
  
  

     Print  


  
  



  
      
  
  

     Download PDF  


  
  



  
      
  
  

    
 Email Article
  


  
  



  
      
  
  

    
 Citation Tools
  


  
  



          

        

        
        

          

            



  
      
  
  

    

  

    

  
      
  
  

    

  
  
  
  
      
Proton MR Spectroscopy of Mitochondrial Diseases: Analysis of Brain Metabolic Abnormalities and Their Possible Diagnostic Relevance
  
    	
M. Cristina Bianchi, Michela Tosetti, Roberta Battini, Maria L. Manca, Michelangelo Mancuso, Giovanni Cioni, Raffaello Canapicchi, Gabriele Siciliano

  
    	
American Journal of Neuroradiology Nov 2003, 24 (10) 1958-1966; 

  
  
  



  


  
  



  
      
  
  

    

  
    
  
  
    
  
  
    
  


  


  
  



  
      
  
  

    
del.icio.us logo Twitter logo Facebook logo Mendeley logo
  


  
  



  



  


  
  



  
      
  
  

    
  


  
  



          

        

	
 	
	
	


  


  
  



  
        
Jump to section

    
  
  

    
  


  
  



  
      
  
  

    
  


  
  



			

		

	
	
 	
	
	


    

  



  

                
    

      

  

    

  
      
  
  

    


Advertisement