ArticleThe British journal of radiology2014
Bilateral hypertrophic olivary nucleus degeneration on magnetic resonance imaging in children with Leigh and Leigh-like syndrome.
Article in The British journal of radiology, 2014. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 10 papers, 1 of them a synthesis that pooled it.
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Who cites it
10 citing papers in PubMed, 1 synthesis or guideline pooled it.
- The Guillain-Mollaret triangle: a key player in motor coordination and control with implications for neurological disorders.Neurosurgical review · 2023Pooled it
- Leigh Syndrome: A Comprehensive Review of the Disease and Present and Future Treatments.Biomedicines · 2025Review
- The first case report of spinocerebellar ataxia type-40 in India: novel phenotypic and radiological (bilateral olivary degeneration) features and a comprehensive review of this remarkable radiological sign.Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology · 2022Article
- Hypertrophic olivary degeneration: a description of four cases of and a literature analysis.Quantitative imaging in medicine and surgery · 2022Article
- Biallelic variants in HPDL cause pure and complicated hereditary spastic paraplegia.Brain : a journal of neurology · 2021Article
- Hypertrophic olivary degeneration: A clinico-radiologic study.Parkinsonism & related disorders · 2016Article
- The neuroimaging of Leigh syndrome: case series and review of the literature.Pediatric radiology · 2016Review
- Magnetic resonance imaging differential diagnosis of brainstem lesions in children.World journal of radiology · 2016Article
- Palatal Tremor inMovement disorders clinical practice · 2015Article
- Mutations in TTC19: expanding the molecular, clinical and biochemical phenotype.Orphanet journal of rare diseases · 2015Article
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12 authors.
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Abstract
objectiveBilateral 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.
methodsThis 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.
resultsThe 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.
conclusionBilateral 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. ADVANCES IN KNOWLEDGE: 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.
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