FDG-PET study of patients with Leigh syndrome

FDG-PET study of patients with Leigh syndrome

Abstract We conducted a [18 F]fluorodeoxyglucose positron emission tomography (FDG-PET) study in five patients (median age 11 (range 4–13) years) with Leigh syndrome to evaluate its usefulness for understanding the functional brain dysfunction in this disease and in future drug trials. Four patients...

Full description

Saved in:
Bibliographic Details
Published in:Journal of the neurological sciences Vol. 362; pp. 309 - 313
Main Authors: Haginoya, Kauzhiro, Kaneta, Tomohiro, Togashi, Noriko, Hino-Fukuyo, Naomi, Kobayashi, Tomoko, Uematsu, Mitsugu, Kitamura, Taro, Inui, Takehiko, Okubo, Yukimune, Takezawa, Yusuke, Anzai, Mai, Endo, Wakaba, Miyake, Noriko, Saitsu, Hirotomo, Matsumoto, Naomichi, Kure, Shigeo
Format: Journal Article
Language:English
Published: Netherlands Elsevier B.V 15-03-2016
Subjects:
Adolescent
Basal Ganglia - diagnostic imaging
Cerebellum - diagnostic imaging
Child
Child, Preschool
FDG-PET
Fluorodeoxyglucose F18 - metabolism
Humans
Leigh Disease - diagnostic imaging
Leigh syndrome
mitochondrial disease
neuroimaging
Neurology
PET
Positron-Emission Tomography
FDG-PET
Leigh syndrome
positron emission tomography
mitochondrial disease
PET
neuroimaging
Online Access:Get full text
Tags: Add Tag
No Tags, Be the first to tag this record!
Description
Summary:Abstract We conducted a [18 F]fluorodeoxyglucose positron emission tomography (FDG-PET) study in five patients (median age 11 (range 4–13) years) with Leigh syndrome to evaluate its usefulness for understanding the functional brain dysfunction in this disease and in future drug trials. Four patients were found to have reported mitochondrial DNA gene mutations. The brain T2-weighted magnetic resonance imaging (MRI) showed high-intensity areas in the putamen bilaterally in five patients, caudate bilaterally in four, thalamus bilaterally in two, and brainstem in one. Cerebellar atrophy was observed in older two patients. For disease control, seven ag e -matched epilepsy patients who had normal MRI and FDG-PET studies were selected. For semiquantitative analysis of the lesions with decreased18 F-FDG uptake, the mean standard uptake value (SUV) was calculated in regions of interest (ROIs) placed in each brain structure. We compared the SUV of nine segments (the frontal, temporal, parietal, and occipital lobes, thalami, basal ganglia, mid-brain, pons, and cerebellum) between patients with Leigh syndrome and controls. The glucose uptake was decreased significantly in the cerebellum and basal ganglia, which could explain the ataxia and dystonia in patients with Leigh syndrome. Although this study had some limitations, FDG-PET might be useful for evaluating the brain dysfunction and treatment efficacy of new drugs in patients with Leigh syndrome. Further study with more patients using advanced methods to quantify glucose uptake is needed before drawing a conclusion.
Bibliography:ObjectType-Article-1
SourceType-Scholarly Journals-1
ObjectType-Feature-2
content type line 23
ISSN:0022-510X
1878-5883
DOI:10.1016/j.jns.2016.02.008