Teaching NeuroImage: Bilateral Nucleus Tractus Solitarius Lesions in Neurogenic Respiratory Failure
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A 7-year-old girl with MEGD(H)EL [3-methylglutaconic aciduria, dystonia-deafness, (hepatopathy), encephalopathy, Leigh-like syndrome, SERAC1]1 presented with worsening respiratory compromise. The evaluation showed type II respiratory failure (e.g., hypercapnic) necessitating mechanical ventilation. Cerebral MRI demonstrated progression of known changes in MEGD(H)EL (Figure, A and B) and symmetric nucleus tractus solitarius (NTS) involvement (Figure, C and D). She was ventilator dependent and subsequently died from the effect of the disease.
Brain MRI at age 2 years shows the classic “putaminal eye” sign (A, arrows). MRI at age 7 years shows progressive atrophy and gliosis of basal ganglia and cortical atrophy (B) and bilateral symmetrical signal changes of the nucleus tractus solitarius on fluid attenuated inversion recovery sequence (C, arrows) and T2-weighted images (D, arrows).
Bilateral NTS involvement is a rare occurrence in a neurologic setting.2 NTS plays a crucial role in the continuous modulation of chemoreceptor-mediated respiration and other respiratory reflexes.2 This case illustrates the neuroimaging correlation of central neurogenic respiratory failure.
Study Funding
The authors report no targeted funding.
Disclosure
B. Parayil Sankaran reports receipt of the clinical research fellowship from Mito Foundation Australia. S.B. Wortmann, M.A. Willemsen, and S. Balalsubramaniam report no disclosures relevant to the manuscript. Go to Neurology.org/N for full disclosures.
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Footnotes
Go to Neurology.org/N for full disclosures. Funding information and disclosures deemed relevant by the authors, if any, are provided at the end of the article.
Teaching slides http://links.lww.com/WNL/B485
- © 2021 American Academy of Neurology
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