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CC BY-NC-ND 4.0 · Arq Neuropsiquiatr 2016; 74(05): 426-427
DOI: 10.1590/0004-282X20160049
IMAGES IN NEUROLOGY

Hypertrophic olivary degeneration: unveiling the triangle of Guillain-Mollaret

Degeneração olivar hipertrófica: descobrindo o triângulo de Guillain-Mollaret
William Alves Martins
1   Pontifícia Universidade Católica do Rio Grande do Sul, Hospital São Lucas, Departamento de Neurologia, Porto Alegre RS, Brasil;
,
Luiz Carlos Porcello Marrone
1   Pontifícia Universidade Católica do Rio Grande do Sul, Hospital São Lucas, Departamento de Neurologia, Porto Alegre RS, Brasil;
3   Pontifícia Universidade Católica do Rio Grande do Sul, Instituto do Cérebro, Porto Alegre RS, Brasil.
,
Ricardo Bernardi Soder
1   Pontifícia Universidade Católica do Rio Grande do Sul, Hospital São Lucas, Departamento de Neurologia, Porto Alegre RS, Brasil;
3   Pontifícia Universidade Católica do Rio Grande do Sul, Instituto do Cérebro, Porto Alegre RS, Brasil.
,
Jaderson Costa da Costa
1   Pontifícia Universidade Católica do Rio Grande do Sul, Hospital São Lucas, Departamento de Neurologia, Porto Alegre RS, Brasil;
2   Pontifícia Universidade Católica do Rio Grande do Sul, Faculdade de Medicina, Departamento de Medicina Interna, Porto Alegre RS, Brasil;
3   Pontifícia Universidade Católica do Rio Grande do Sul, Instituto do Cérebro, Porto Alegre RS, Brasil.
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A 28-year-old woman presented with hemi-dystonic movements on the left. Four years earlier, a ruptured vascular malformation caused a hemorrhagic stroke on the left cerebellar hemisphere ([Figure 1B]), evacuated by surgery. MRI revealed right hypertrophic olivary degeneration (HOD) ([Figure 1A]) and reduction of cerebellar-rubro pathways ([Figure 2]).

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Figure 1 (A) Axial T2-weighted image showing enlargement and hyperintensity of the right olivary nuclei, compatible to hypertrophic olivary degeneration; (B) Coronal T2-weighted image displaying reduced left cerebellar hemisphere associated to hemosiderin.
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Figure 2 (A) Atrophy of right red nucleus (arrow) and substantia nigra; (B) Tractography image showing decreased volume of the right central tegmental tract (arrowheads).

HOD is a trans-synaptic degeneration affecting the dentate-rubro-olivary pathway or Guillain-Mollaret’s triangle[1]. A lesion in any part of this synaptic pathway may lead to HOD ([Figure 3]), including stroke, cranioencephalic trauma, neurodegenerative diseases, inflammatory illnesses, among others[2] , [3]. This unique synaptic collapse is associated to palatal myoclonus, parkinsonian features and dystonia[1] , [2].

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Figure 3 Anatomical view of the Triangle of Guillain-Mollaret. Initially, the axons from the dentate nucleus of cerebellum travel through the superior cerebellar peduncle (SCP) and decussate in the brachium conjunctivum to terminate into the contralateral red nucleus (Rn). Then, the central tegmental tract (CTT) leaves the red nucleus in the direction of the ipsilateral inferior olivary nuclei (On), situated downwardly at the medulla oblongata. At last, the inferior cerebellar peduncle (ICP) connects the ION to the opposite dentate nucleus (Dn), closing this complex pathway known as dentate-rubral-olivary circuitry.

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Conflict of interest:

There is no conflicts of interest to declare.

  • References

  • 1 Guillain G, Mollaret P. Deux-cas de myoclonies synchrones et rhythmes velopharyngo-laringo-oculo-diaphragmatiques. Rev Neurol. 1931;2:545-66.
  • 2 Martins WA, Schilling LCP, Neto FK, Becker J. Hypertrophic olivary degeneration secondary to Neuro-Behçet’s disease. Clin Neuroradiol. 2015 Mar 24 [Epub ahead of print]. doi:10.1007/s00062-015-0384-0
  • 3 Siebert E, Harms L, Herbst M. Posttraumatic bilateral hypertrophic olivary degeneration. Neurol Sci. 2013;34(10):1829-30. doi:10.1007/s10072-013-1309-9

Address for correspondence

William Alves Martins
Departmento de Neurologia, Hospital São Lucas – PUCRS; Avenida Ipiranga 6690/220; 00610-000 Porto Alegre RS
Brasil   

Publication History

Received: 22 August 2015

Accepted: 09 December 2015

Article published online:
06 September 2023

© 2023. Academia Brasileira de Neurologia. This is an open access article published by Thieme under the terms of the Creative Commons Attribution-NonDerivative-NonCommercial License, permitting copying and reproduction so long as the original work is given appropriate credit. Contents may not be used for commecial purposes, or adapted, remixed, transformed or built upon. (https://creativecommons.org/licenses/by-nc-nd/4.0/)

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  • References

  • 1 Guillain G, Mollaret P. Deux-cas de myoclonies synchrones et rhythmes velopharyngo-laringo-oculo-diaphragmatiques. Rev Neurol. 1931;2:545-66.
  • 2 Martins WA, Schilling LCP, Neto FK, Becker J. Hypertrophic olivary degeneration secondary to Neuro-Behçet’s disease. Clin Neuroradiol. 2015 Mar 24 [Epub ahead of print]. doi:10.1007/s00062-015-0384-0
  • 3 Siebert E, Harms L, Herbst M. Posttraumatic bilateral hypertrophic olivary degeneration. Neurol Sci. 2013;34(10):1829-30. doi:10.1007/s10072-013-1309-9

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Zoom Image
Figure 1 (A) Axial T2-weighted image showing enlargement and hyperintensity of the right olivary nuclei, compatible to hypertrophic olivary degeneration; (B) Coronal T2-weighted image displaying reduced left cerebellar hemisphere associated to hemosiderin.
Zoom Image
Figure 2 (A) Atrophy of right red nucleus (arrow) and substantia nigra; (B) Tractography image showing decreased volume of the right central tegmental tract (arrowheads).
Zoom Image
Figure 3 Anatomical view of the Triangle of Guillain-Mollaret. Initially, the axons from the dentate nucleus of cerebellum travel through the superior cerebellar peduncle (SCP) and decussate in the brachium conjunctivum to terminate into the contralateral red nucleus (Rn). Then, the central tegmental tract (CTT) leaves the red nucleus in the direction of the ipsilateral inferior olivary nuclei (On), situated downwardly at the medulla oblongata. At last, the inferior cerebellar peduncle (ICP) connects the ION to the opposite dentate nucleus (Dn), closing this complex pathway known as dentate-rubral-olivary circuitry.