ORIGINAL

Guillain-Mollaret Triangle: anatomy applied to hypertrophic olivary degeneration

Trígono de Guillain Mollaret: anatomia aplicada à degeneração olivar hipertrófica

  • Pedro Henrique Alves Reis 1    Pedro Henrique Alves Reis 1
  • Julia Basolli Gomiero 1    Julia Basolli Gomiero 1
  • Laura Campos Dornellas 1    Laura Campos Dornellas 1
  • Maria Fernanda Stefanoni Gobbi 1    Maria Fernanda Stefanoni Gobbi 1
  • Milene Castro de Sousa 1    Milene Castro de Sousa 1
  • Giovanna Delcole 2    Giovanna Delcole 2
  • Paulo Henrique Pires de Aguiar 1    Paulo Henrique Pires de Aguiar 1
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Resumo

Introdução: a via denteado-rubro olivar, conhecida como triângulo de Guillain-Mollaret (TGM), é associada à distonias cerebelares como degeneração olivar hipertrófica (DOH). DOH se apresenta com sintomas como tremor palatal, ataxia e nistagmos. Compreender a anatomia do TGM é crucial para o diagnóstico acurado e o planejamento terapêutico. Objetivo: relacionar a anatomia do TGM com a apresentação clínica das distonias e explorar opções terapêuticas. Métodos: realizar a análise de espécimes de cadáver e revisão de literatura no PubMed/MEDLINE. Resultados: DOH afeta o núcleo olivar inferior (NOI) e é caracterizada por mudanças hipertróficas após uma perda de aferências. O TGM consiste em vias eferentes do núcleo denteado (ND), pelo pedúnculo cerebelar superior (PCS), que decussam em direção ao núcleo rubro (NR), e conexões do NR ao NOI ipsilateral via trato tegumentar central (TTC). As lesões no ND, PCS contralateral ou TTC ipsilateral podem causar DOH. O DOH é diagnosticado por meio de ressonância nuclear magnética, que revela aumento do núcleo afetado com hipersinal em T2. Conclusão: o conhecimento aprofundado da anatomia do TGM é vital para evitar lesões cirúrgicas que causem DOH. Os neurorradiologistas devem reconhecer as características da DOH para evitar erros diagnósticos. O DBS pode ser uma opção terapêutica para casos específicos, porém sua eficácia ainda é questionada.


Palavras-chave

Distúrbios distônicos; Tremor; Anatomia; Ataxia; Fossa craniana posterior

Abstract

Introduction: the Dentate-Rubro-Olivary pathway, known as Guillain-Mollaret Triangle (GMT), is associated with cerebellar dystonias such as Hypertrophic Olivary Degeneration (HOD). HOD presents itself with symptoms like palatal tremor, ataxia, and nystagmus. Understanding the anatomy of GMT is crucial for accurate diagnosis and treatment planning. Objective: to correlate Guillain-Mollaret Triangle anatomy with dystonia presentation and explore therapeutic options. Methods: to perform the analysis of cadaver specimens and a literature review on PubMed/MEDLINE. Results: Hypertrophic Olivary Degeneration (HOD) affects the Inferior Olivary Nucleus (ION) and is characterized by hypertrophic changes following afferent loss. The Guillain-Mollaret Triangle (GMT) consists of efferent pathways from the Dentate Nucleus (DN) through the superior cerebellar peduncle (SCP) that decussate in the midbrain to the Red Nucleus (RN), and connections from the RN to the ipsilateral ION via Central Tegmental Tract (CTT). Lesions in the contralateral DN, SCP, or ipsilateral CTT can cause HOD. HOD is diagnosed through magnetic resonance imaging, revealing an increased size of the affected nucleus with hyperintense signal in T2-weighted images. Conclusion: Thorough understanding of GMT anatomy is vital for avoiding surgical injuries in HOD. Neuroradiologists should recognize HOD characteristics to prevent misdiagnosis. DBS may be an option for resistant HOD cases but its effectiveness is unsubstantiated.


Keywords

Dystonia; Tremor; Anatomy; Ataxia; Cranial fossa, posterior

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1Centro Universitário Faculdade de Medicina do ABC, Santo André, SP, Brasil.

2Neurosurgery Department, Hospital Santa Paula, São Paulo, SP, Brasil.

 

Received Apr 16, 2024

Accepted May 28, 2024

JBNC  Brazilian Journal of Neurosurgery

JBNC
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  •   e-ISSN (online version): 2446-6786
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