Reversible brain dysfunction in MELAS: MEG, and (1)H MRS analysis.
This case report describes a follow up investigation of a patient with impaired word discrimination due to mitochondrial encephalopathy with lactic acidosis and stroke-like syndrome (MELAS) using proton magnetic resonance spectroscopy ((1)H MRS) and auditory evoked magnetic fields (AEFs). The initial (1)H MRS showed no N-acetyl aspartate (NAA) and marked accumulation of lactate (Lac) in the stroke-like lesion of MELAS, which was silent in neural activity according to AEFs. The follow up investigations, however, demonstrated that NAA reappeared, that the formerly increased Lac signal was significantly reduced, and that the magnitude of AEFs of the lesion was markedly increased. Metabolic and functional changes in (1)H MRS and AEFs reflected the neurological recovery very well. The stroke-like lesion was shown, using AEFs and (1)H MRS, to be able to function properly, although brain tissue of the lesion initially had severe damage due to mitochondrial dysfunction.
Kamada
K
K
Department of Neurosurgery, School of Medicine, Hokkaido University, Kita-ku, Kita-15, Nishi-7, Sapporo, 060-8638, Japan. kamady1@qc4.so-net.ne.jp
Takeuchi
F
F
Houkin
K
K
Kitagawa
M
M
Kuriki
S
S
Ogata
A
A
Tashiro
K
K
Koyanagi
I
I
Mitsumori
K
K
Iwasaki
Y
Y
D000328:Adult; D001921:Brain / Q000378:metabolism* / Q000503:physiopathology*; D006801:Humans; D017241:MELAS Syndrome / Q000378:metabolism* / Q000503:physiopathology*; D009682:Magnetic Resonance Spectroscopy; D015225:Magnetoencephalography; D008297:Male