Detection of 14-3-3 protein in the cerebrospinal fluid in mitochondrial encephalopathy with lactic acidosis and stroke-like episodes.
We describe a 13-year-old boy with mitochondrial encephalopathy with lactic acidosis and stroke-like episodes (MELAS) who experienced a stroke-like episode resulting in severe mental regression and quadriplegia. We tested 14-3-3 protein in the cerebrospinal fluid (CSF) of the patient four times around a stroke-like episode in a magnetic resonance imaging (MRI) study. Detection of the protein in the CSF was well correlated with the clinical course and range of damage of the brain lesion on MRI. Interestingly, 14-3-3 CSF protein was detected at the beginning of mitochondrial encephalopathy without new MRI abnormalities, suggesting that it is a sensitive brain marker. We conclude that 14-3-3 CSF protein is a useful biological marker of brain disruption in MELAS as well as other neurological disorders.
Fujii
Katsunori
K
Department of Pediatrics, Chiba University Graduate School of Medicine, 1-8-1 Inohana, Chiba-shi, Chiba 260-8670, Japan. kfujii@faculty.chiba-u.jp
Tanabe
Yuzo
Y
Kobayashi
Kazuhiko
K
Uchikawa
Hideki
H
Kohno
Yoichi
Y
D048948:14-3-3 Proteins / Q000032:analysis / Q000134:cerebrospinal fluid*; D000293:Adolescent; D015415:Biomarkers; D001921:Brain / Q000378:metabolism* / Q000473:pathology / Q000503:physiopathology*; D006801:Humans; D017241:MELAS Syndrome / Q000134:cerebrospinal fluid* / Q000175:diagnosis* / Q000503:physiopathology; D008279:Magnetic Resonance Imaging; D008297:Male; D008928:Mitochondria / Q000378:metabolism; D011237:Predictive Value of Tests; D020521:Stroke / Q000134:cerebrospinal fluid / Q000209:etiology / Q000473:pathology
D048948:14-3-3 Proteins; D015415:Biomarkers