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January 04, 2022; 98 (1) Resident & Fellow Section

Teaching NeuroImage: Bilateral Nucleus Tractus Solitarius Lesions in Neurogenic Respiratory Failure

View ORCID ProfileBindu Parayil Sankaran, Saskia B. Wortman, Michel A. Willemsen, Shanti Balasubramaniam
First published August 10, 2021, DOI: https://doi.org/10.1212/WNL.0000000000012614
Bindu Parayil Sankaran
From the Genetic Metabolic Disorders Service (B.P.S., S.B.), Western Sydney Genetics Program, The Children's Hospital at Westmead, Sydney; The Children's Hospital at Westmead Clinical School (B.P.S.), Sydney Medical School, The Faculty of Medicine and Health, The University of Sydney, NSW, Australia; Department of Pediatric Neurology (S.B.W., M.A.W.), Amalia Children's Hospital, Radboud University Nijmegen Medical Center, Nijmegen, the Netherlands; Department of Pediatrics (S.B.W.), University Children's Hospital, Salzburg, Austria; and Discipline of Genetic Medicine (S.B.), Sydney Medical School, University of Sydney, NSW, Australia.
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  • ORCID record for Bindu Parayil Sankaran
Saskia B. Wortman
From the Genetic Metabolic Disorders Service (B.P.S., S.B.), Western Sydney Genetics Program, The Children's Hospital at Westmead, Sydney; The Children's Hospital at Westmead Clinical School (B.P.S.), Sydney Medical School, The Faculty of Medicine and Health, The University of Sydney, NSW, Australia; Department of Pediatric Neurology (S.B.W., M.A.W.), Amalia Children's Hospital, Radboud University Nijmegen Medical Center, Nijmegen, the Netherlands; Department of Pediatrics (S.B.W.), University Children's Hospital, Salzburg, Austria; and Discipline of Genetic Medicine (S.B.), Sydney Medical School, University of Sydney, NSW, Australia.
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Michel A. Willemsen
From the Genetic Metabolic Disorders Service (B.P.S., S.B.), Western Sydney Genetics Program, The Children's Hospital at Westmead, Sydney; The Children's Hospital at Westmead Clinical School (B.P.S.), Sydney Medical School, The Faculty of Medicine and Health, The University of Sydney, NSW, Australia; Department of Pediatric Neurology (S.B.W., M.A.W.), Amalia Children's Hospital, Radboud University Nijmegen Medical Center, Nijmegen, the Netherlands; Department of Pediatrics (S.B.W.), University Children's Hospital, Salzburg, Austria; and Discipline of Genetic Medicine (S.B.), Sydney Medical School, University of Sydney, NSW, Australia.
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Shanti Balasubramaniam
From the Genetic Metabolic Disorders Service (B.P.S., S.B.), Western Sydney Genetics Program, The Children's Hospital at Westmead, Sydney; The Children's Hospital at Westmead Clinical School (B.P.S.), Sydney Medical School, The Faculty of Medicine and Health, The University of Sydney, NSW, Australia; Department of Pediatric Neurology (S.B.W., M.A.W.), Amalia Children's Hospital, Radboud University Nijmegen Medical Center, Nijmegen, the Netherlands; Department of Pediatrics (S.B.W.), University Children's Hospital, Salzburg, Austria; and Discipline of Genetic Medicine (S.B.), Sydney Medical School, University of Sydney, NSW, Australia.
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Teaching NeuroImage: Bilateral Nucleus Tractus Solitarius Lesions in Neurogenic Respiratory Failure
Bindu Parayil Sankaran, Saskia B. Wortman, Michel A. Willemsen, Shanti Balasubramaniam
Neurology Jan 2022, 98 (1) e103-e104; DOI: 10.1212/WNL.0000000000012614

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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.

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Figure Brain MRI

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

References

  1. 1.↵
    1. Wortmann SB,
    2. van Hasselt PM,
    3. Baric I, et al.
    Eyes on MEGDEL: distinctive basal ganglia involvement in dystonia deafness syndrome. Neuropediatrics. 2015;46(2):98-103.
    OpenUrlCrossRefPubMed
  2. 2.↵
    1. Cutsforth-Gregory JK,
    2. Benarroch EE
    . Nucleus of the solitary tract, medullary reflexes, and clinical implications. Neurology. 2017;88(12):1187-1196.
    OpenUrlAbstract/FREE Full Text

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