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CC BY 4.0 · Arq Neuropsiquiatr 2023; 81(03): 263-270
DOI: 10.1055/s-0043-1764416
Original Article

Exploring clinical outcomes in patients with idiopathic/inherited isolated generalized dystonia and stimulation of the subthalamic region

Explorando os desfechos clínicos em pacientes com distonia idiopática/hereditária generalizada isolada submetidos a estimulação da região subtalâmica
Clarice Listik 
1   Universidade de São Paulo, Center for Movement Disorders, Faculty of Medicine, Department of Neurology, São Paulo SP, Brazil.
,
Jorge Dornellys Lapa 
2   Universidade de São Paulo, Faculty of Medicine, Neurosurgery Division, Departament of de Neurology, São Paulo SP, Brazil.
,
Sara Carvalho Barbosa Casagrande 
1   Universidade de São Paulo, Center for Movement Disorders, Faculty of Medicine, Department of Neurology, São Paulo SP, Brazil.
,
Egberto Reis Barbosa 
1   Universidade de São Paulo, Center for Movement Disorders, Faculty of Medicine, Department of Neurology, São Paulo SP, Brazil.
,
Ricardo Iglesio 
2   Universidade de São Paulo, Faculty of Medicine, Neurosurgery Division, Departament of de Neurology, São Paulo SP, Brazil.
,
Fabio Godinho 
2   Universidade de São Paulo, Faculty of Medicine, Neurosurgery Division, Departament of de Neurology, São Paulo SP, Brazil.
,
Kleber Paiva Duarte 
2   Universidade de São Paulo, Faculty of Medicine, Neurosurgery Division, Departament of de Neurology, São Paulo SP, Brazil.
,
Manoel Jacobsen Teixeira 
2   Universidade de São Paulo, Faculty of Medicine, Neurosurgery Division, Departament of de Neurology, São Paulo SP, Brazil.
,
Rubens Gisbert Cury 
1   Universidade de São Paulo, Center for Movement Disorders, Faculty of Medicine, Department of Neurology, São Paulo SP, Brazil.
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Abstract

Background Deep Brain Stimulation (DBS) is an established treatment option for refractory dystonia, but the improvement among the patients is variable.

Objective To describe the outcomes of DBS of the subthalamic region (STN) in dystonic patients and to determine whether the volume of tissue activated (VTA) inside the STN or the structural connectivity between the area stimulated and different regions of the brain are associated with dystonia improvement.

Methods The response to DBS was measured by the Burke-Fahn-Marsden Dystonia Rating Scale (BFM) before and 7 months after surgery in patients with generalized isolated dystonia of inherited/idiopathic etiology. The sum of the two overlapping STN volumes from both hemispheres was correlated with the change in BFM scores to assess whether the area stimulated inside the STN affects the clinical outcome. Structural connectivity estimates between the VTA (of each patient) and different brain regions were computed using a normative connectome taken from healthy subjects.

Results Five patients were included. The baseline BFM motor and disability subscores were 78.30 ± 13.55 (62.00–98.00) and 20.60 ± 7.80 (13.00–32.00), respectively. Patients improved dystonic symptoms, though differently. No relationships were found between the VTA inside the STN and the BFM improvement after surgery (p = 0.463). However, the connectivity between the VTA and the cerebellum structurally correlated with dystonia improvement (p = 0.003).

Conclusions These data suggest that the volume of the stimulated STN does not explain the variance in outcomes in dystonia. Still, the connectivity pattern between the region stimulated and the cerebellum is linked to outcomes of patients.

Resumo

Antecedentes A estimulação cerebral profunda (ECP) é um tratamento estabelecido para distonias refratárias. Porém, a melhora dos pacientes é variável.

Objetivo O objetivo do estudo foi descrever os desfechos da ECP da região do núcleo subtalâmico (NST) e determinar se o volume de tecido ativado (VTA) dentro do NST ou se a conectividade estrutural entre a área estimulada e diferentes regiões cerebrais estão associadas a melhora da distonia.

Métodos A resposta da ECP em pacientes com distonia generalizada isolada de etiologia hereditária/idiopática foi mensurada pela escala de Burke-Fahr-Marsden Dystonia Rating Scale (BFM) antes e 7 meses após a cirurgia. A soma dos volumes do NST nos dois hemisférios foi correlacionada com a melhora nos escores do BFM para avaliar se a área estimulada dentro do NST afeta o desfecho clínico. A conectividade estrutural estimada entre o VTA de cada paciente e as diferentes regiões cerebrais foram computadas usando um conectoma normativo retirado de indivíduos saudáveis.

Resultados Cinco pacientes com idade de 40,00 ± 7,30 anos foram incluídos. O BFM motor e de incapacidade basal eram de 78,30 ± 13,55 (62,00–98,00) e 20,60 ± 7,80 (13,00–32,00), respectivamente. Os pacientes melhoraram com a cirurgia, mas com variabilidade. Não houve relação entre o VTA dentro do NST e a melhora do BFM após a cirurgia (p = 0.463). Entretanto, a conectividade estrutural entre o VTA e o cerebelo correlacionaram com a melhora da distonia (p = 0.003).

Conclusão Os dados sugerem que o VTA dentro do NST não explica a variabilidade do desfecho clínico na distonia. Porém, o padrão de conectividade entre a região estimulada e o cerebelo foi relacionada com o desfecho dos pacientes.

Keywords

Deep Brain Stimulation - Dystonia - Cerebellum - Subthalamic Nucleus - Connectome

Palavras-chave

Estimulação Encefálica Profunda - Distonia - Cerebelo - Núcleo Subtalâmico - Conectoma

Authors' Contributions

CL: data curation, formal analysis, investigation, writing – original draft preparation; DJL, RI: formal analysis, writing – original draft preparation; SCBC: data curation, investigation; ERB: supervision, writing – review and editing; FG, KPD: writing – review and editing; MJT: methodology, writing – review and editing; RGC: methodology, project administration, supervision, writing – review and editing.




Publication History

Received: 07 May 2022

Accepted: 10 November 2022

Article published online:
14 April 2023

© 2023. Academia Brasileira de Neurologia. This is an open access article published by Thieme under the terms of the Creative Commons Attribution 4.0 International License, permitting copying and reproduction so long as the original work is given appropriate credit (https://creativecommons.org/licenses/by/4.0/)

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