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Bilateral hypertrophic olivary nucleus degeneration on magnetic resonance imaging in children with Leigh and Leigh-like syndrome
- Source :
- The British Journal of Radiology. 87:20130478
- Publication Year :
- 2014
- Publisher :
- British Institute of Radiology, 2014.
-
Abstract
- Bilateral hypertrophic olivary degeneration on brain MRI has been reported in a few metabolic, genetic and neurodegenerative disorders, including mitochondrial disorders. In this report, we sought to analyse whether bilateral symmetrical inferior olivary nucleus hypertrophy is specifically associated with mitochondrial disorders in children.This retrospective study included 125 children (mean age, 7.6 ± 5 years; male:female, 2.6:1) diagnosed with various metabolic and genetic disorders during 2005-2012. The routine MRI sequences (T1 weighted, T2 weighted and fluid-attenuated inversion-recovery sequences) were analysed for the presence of bilateral symmetrical olivary hypertrophy and central tegmental tract or dentate nuclei signal changes. The other imaging findings and the final diagnoses were noted.The cohort included patients with Leigh and Leigh-like syndrome (n = 25), other mitochondrial diseases (n = 25), Wilson disease (n = 40), Type 1 glutaric aciduria (n = 14), maple syrup urine disease (n = 13), giant axonal neuropathy (n = 5) and L-2 hydroxy glutaric aciduria (n = 3). Bilateral inferior olivary nucleus hypertrophy was noted in 10 patients, all of whom belonged to the Leigh and Leigh-like syndrome group.Bilateral hypertrophic olivary degeneration on MRI is relatively often, but not routinely, seen in children with Leigh and Leigh-like syndrome. Early detection of this finding by radiologists and physicians may facilitate targeted metabolic testing in these children.This article highlights the occurrence of bilateral hypertrophic olivary nucleus degeneration on MRI in children with Leigh and Leigh-like syndrome, compared with other metabolic disorders.
- Subjects :
- Male
Pathology
medicine.medical_specialty
Mitochondrial Diseases
Mitochondrial disease
Olivary Nucleus
Muscle hypertrophy
Cohort Studies
Diagnosis, Differential
Glutarates
Hepatolenticular Degeneration
Maple Syrup Urine Disease
Central tegmental tract
medicine
Inferior olivary nucleus
Humans
Radiology, Nuclear Medicine and imaging
Leigh disease
Child
Retrospective Studies
Full Paper
medicine.diagnostic_test
business.industry
Olivary degeneration
Magnetic resonance imaging
Hypertrophy
Syndrome
General Medicine
medicine.disease
Magnetic Resonance Imaging
Early Diagnosis
Giant Axonal Neuropathy
Female
Leigh Disease
Differential diagnosis
business
Subjects
Details
- ISSN :
- 1748880X and 00071285
- Volume :
- 87
- Database :
- OpenAIRE
- Journal :
- The British Journal of Radiology
- Accession number :
- edsair.doi.dedup.....73c94052f97dd7e6e3e8ecadb9b8c769