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Brain Advance Access originally published online on April 6, 2004
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Brain, Vol. 127, No. 6, 1302-1312, 2004
© 2004 Guarantors of Brain
doi: 10.1093/brain/awh157

Longitudinal study of MRS metabolites in Rasmussen encephalitis

R. M. Wellard1, R. S. Briellmann1,3, J. C. Wilson5, R. M. Kalnins1, D. P. Anderson1,4, P. Federico1, G. C. A. Fabinyi1, I. E. Scheffer2,3, A. S. Harvey1 and G. D. Jackson1,3

1 Brain Research Institute and 2 Epilepsy Research Institute, Heidelberg West, 3 University of Melbourne, Parkville, 4 Brain Sciences Institute, Swinburn University of Technology, Hawthorn, Victoria, and 5 Institute for Glycomics, Griffith University (Gold Coast Campus), Queensland, Australia

Correspondence to: Professor Graeme Jackson, Director, Brain Research Center, Austin Health, Neurosciences Building, Banksia St, Heidelberg West 3081, Australia. E-mail: g.jackson{at}brain.org.au

This study analyses the evolution of metabolite changes in an 8-year-old boy with focal Rasmussen encephalitis. Five MRI examinations, including magnetic resonance spectroscopy (MRS) were performed over 9 months. Following complex partial status, T2-weighted imaging showed transient dramatic signal increase in the left superior temporal gyrus and mesial temporal structures. Subsequent scans showed resolution of the swelling and signal normalization, with development of slight focal atrophy. MRS after status showed a reduction in N-acetylaspartate, total creatine and trimethylamines. Subsequent scans showed complete resolution of these metabolite abnormalities, followed later by development of further abnormal metabolite values. Lactate and glutamine/glutamate were elevated after status. After surgery, ex vivo high-field 1H and 31P MRS confirmed metabolite abnormalities (elevated choline and decreased aspartate, N-acetylaspartate, [1H]glutamate together with altered [31P]phospholipid ratios. These findings suggested active disease process in the anterior region of the excised superior temporal gyrus. We conclude that Rasmussen encephalitis is a combination of progressive encephalitic damage and fluctuating seizure effects, in which neuronal injury and recovery can occur. MRS measurements at a single time point should consider the fluctuating metabolite profile related to seizure activity.

Key Words: Rasmussen; chronic localized encephalitis; epilepsy; magnetic resonance spectroscopy; MRS

Abbreviations: Cho= total choline; CPS = complex partial seizure; Cr = creatine + phosphocreatine; GABA = {gamma} aminobutyric acid; gCOSY = gradient correlated spectroscopy; Glx = glutamine + glutamate; Lac = lactate; mI = myoinositol; MRS = magnetic resonance spectroscopy; NA = N-acetylaspartate + N-acetylaspartyl glutamate; NAA = N-acetylaspartate; PRESS = point resolved spectroscopy; ROI = region of interest; SPECT = single photon emission computed tomography; SPS = simple partial seizure.

Received October 29, 2003. Revised January 16, 2004. Accepted January 19, 2004.


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