Initial experiences with proton MR spectroscopy in treatment monitoring of mitochondrial encephalopathy.

Yonsei Medical Journal
Seung-Koo LeeYoung Mock Lee

Abstract

Mitochondrial encephalopathy (ME) is a rare disorder of energy metabolism. The disease course can roughly be evaluated by clinical findings. The purpose of this study was to evaluate metabolic spectral changes using proton MR spectroscopy (MRS), and to establish a way to monitor ME by neuroimaging. Proton MRS data were retrospectively reviewed in 12 patients with muscle biopsy-confirmed ME (M : F = 7 : 5, Mean age = 4.8 years). All received 1H-MRS initially and also after a ketogenic diet and mitochondrial disease treatment cocktail (follow up average was 10.2 months). Changes of N-acetylaspartate/ creatine (NAA/Cr) ratio, choline/creatine (Cho/Cr) ratio, and lactate peak in basal ganglia at 1.2 ppm were evaluated before and after treatment. Findings on conventional T2 weighted MR images were also evaluated. On conventional MRI, increased basal ganglia T2 signal intensity was the most common finding with ME (n = 9, 75%), followed by diffuse cerebral atrophy (n = 8, 67%), T2 hyperintense lesions at pons and midbrain (n = 4, 33%), and brain atrophy (n = 2, 17%). Lactate peak was found in 4 patients; 2 had disappearance of the peak on follow up MRS. Quantitative analysis showed relative decrease of Cho/Cr ratio on follow up MRS (p...Continue Reading

References

Jan 4, 1998·Journal of Neurology, Neurosurgery, and Psychiatry·P F Chinnery, D M Turnbull
Jun 17, 1998·Archives of Neurology·S G PavlakisD Lustbader
Apr 5, 2002·Muscle & Nerve·Harald E MöllerGerhard Schuierer
Apr 3, 2003·Movement Disorders : Official Journal of the Movement Disorder Society·Jay C EricksonMarc P Difazio
Nov 11, 2003·Journal of the Neurological Sciences·Stephen J KolbSteven L Galetta
Jan 28, 2004·Neurologic Clinics·Nicola Longo
May 11, 2005·Archives of Neurology·Héctor Manuel Barragán-CamposJacques Chiras
Aug 1, 2006·Pediatric Neurology·Hoon-Chul KangSi Hoon Han

❮ Previous
Next ❯

Citations

Aug 9, 2013·Brain Injury : [BI]·Rafael Rodriguez-RojasPhilip DeFina
Nov 28, 2015·Neurochemical Research·Erica B SherryIn-Young Choi
Mar 16, 2013·Developmental Medicine and Child Neurology·Saskia KoeneJan A M Smeitink
Oct 4, 2017·Brain : a Journal of Neurology·Hannah E SteelePatrick F Chinnery
Jan 24, 2018·Journal of Medical Genetics·Isaure de BeaurepaireNathalie Boddaert
Mar 10, 2016·EMBO Molecular Medicine·Werner Jh KoopmanJan Am Smeitink
Jul 22, 2018·Essays in Biochemistry·Mario MascalchiRenzo Guerrini

❮ Previous
Next ❯

Methods Mentioned

BETA
imaging techniques
enzyme

Software Mentioned

SpectroView

Related Concepts

Related Feeds

Basal Ganglia

Basal Ganglia are a group of subcortical nuclei in the brain associated with control of voluntary motor movements, procedural and habit learning, emotion, and cognition. Here is the latest research.