Bilateral hypertrophic olivary nucleus degeneration on magnetic resonance imaging in children with Leigh and Leigh-like syndrome

Bindu, Parayil Sankaran; Taly, Arun B.; Kothari, Shawn; Govindaraju, C.; Arvinda, H R; Gayathri, Narayanappa; Bharath, M. M. Srinivas; Ranjith, D; Nagappa, Madhu; Sinha, Sanjib; Khan, Nahid Akthar; Thangaraj, Kumarasamy

doi:10.1259/bjr.20130478

Cited by 23 publications

(20 citation statements)

References 36 publications

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“…11 Recently, HOD and palatal tremor are reported in mitochondrial disorders, 12 particularly children with Leigh's phenotype. 13 Brain MRI in patients with POLG1-associated ataxia, including ours, show HOD along with abnormalities in thalami and basal ganglia. 1,8 Neuropathological findings corroborate with MRI observations.…”

Section: 9supporting

confidence: 50%

Palatal Tremor in POLG‐Associated Ataxia

Nagappa

Bindu

Taly

et al. 2015

Movement Disord Clin Pract

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Section: 9supporting

confidence: 50%

Palatal Tremor in POLG‐Associated Ataxia

Nagappa

Bindu

Taly

et al. 2015

Movement Disord Clin Pract

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“…SURF1 is a causative gene of Leigh syndrome, which is a severe neurodegenerative disorder associated with cytochrome c oxidase deficiency usually affecting infants [28]. Bilateral HOD is a characteristic finding of patients with SURF1 mutations [7, 8]. A nonsense mutation of TTC19 leads to mitochondrial respiratory chain complex III deficiency and neurodegeneration.…”

Section: Discussionmentioning

confidence: 99%

Hypertrophic olivary degeneration: A clinico-radiologic study

Konno

Broderick

Tacik

et al. 2016

Parkinsonism & Related Disorders

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Introduction The frequency and causes of hypertrophic olivary degeneration (HOD) are unknown. We compared the clinical and radiological characteristics of unilateral HOD and bilateral HOD. Methods We performed a search of a radiologic report database for patients who were radiologically diagnosed as having HOD. This database includes the patients examined at the Mayo Clinic in Florida and Arizona. We used the search terms “hypertrophic olivary degeneration,” “HOD,” and “olivary” in the reports recorded from 1995 to 2015. Pertinent medical records and magnetic resonance imaging (MRI) scans of the brain for those with HOD were reviewed retrospectively. Results We identified 142 MRI studies on 95 cases who had radiologically proven HOD, 39 cases had unilateral HOD and 56 with bilateral HOD. In symptomatic cases, the most common symptom was ataxia. Palatal tremor was observed in almost half of all HOD cases. While cerebrovascular diseases were the most frequent etiology in both types of HOD (n=24, 62% in unilateral; n=17, 30% in bilateral), more than half of bilateral HOD cases had an unknown etiology (52%, n=29), whereas only 13% (n=5) of the unilateral cases had an unknown etiology (χ2 test, P<0.001). The lesions of unilateral HOD had a tendency to improve radiologically over time, whereas those associated with bilateral HOD were likely to worsen (χ2 test, P<0.05). Conclusions Our study showed that bilateral HOD is more common than unilateral HOD. Half of bilateral HOD cases had no obvious cause and some worsened over time. This may implicate a possible primary neurodegenerative process.

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“…Typical MR findings include initial hypertrophy and T2 hyperintensity evolving over time to atrophy with residual hyperintensity on T2‐weighted images (Fig ). These changes have been reported to occur either unilaterally or bilaterally …”

Section: Introductionmentioning

confidence: 99%