Bilateral pallidotomy for generalized dystonia

Abstract

-Objective: To evaluate the efficacy and safety of bilateral pallidotomies in five patients with generalized dystonia. Background: Generalized dystonias are frequently a therapeutic challenge, with poor responses to pharmacological treatment. GPi (globus pallidus internus) pallidotomies for Parkinson's disease ameliorate all kinds of dyskinesias/dystonia, and recent studies reported a marked improvement of refractory dystonias with this procedure. Methods: Five patients with generalized dystonias refractory to medical treatment were selected; one posttraumatic and four idiopathic. The decision to perform bilateral procedures was based on the predominant axial involvement in these patients. Dystonia severity was assessed with the Burke-FahnMarsden Dystonia Scale (BFM). Simultaneous procedures were performed in all but one patient, who had a staged procedure. They were reevaluated with the same scale (BFM) by an unblinded rater at 1, 2, 3, 30, 60, 90, 120 and 180 days post-operatively. Results: The four patients with idiopathic dystonia showed a progressive improvement up to three months; the patient with posttraumatic dystonia relapsed at three months. One patient had a marked improvement, being able to discontinue all the medications. A mean decrease in the BFM scores of 52,58% was noted. One patient had a trans-operative motor seizure followed by a transient hemiparesis secondary to rack hemorrhage; other was lethargic up to three days after the procedure. Conclusions: Our results show that bilateral GPi pallidotomies may be a safe and effective approach to medically refractory generalized dystonias; it can also be speculated that the posttraumatic subgroup may not benefit with this procedure.KEY WORDS: dystonia, pallidotomy, stereotactic surgery. Palidotomia bilateral para distonias generalizadasRESUMO -As distonias generalizadas são freqüentemente um desafio terapêutico, com pobres respostas aos tratamentos farmacológicos. As cirurgias estereotáxicas, como a palidotomia, têm sido utilizadas com êxito no tratamento da doença de Parkinson e estudos recentes relatam importante melhora das distonias generalizadas, refratárias ao tratamento farmacológico, com a palidotomia bilateral. O objetivo dos autores foi avaliar a eficácia e segurança da palidotomia bilateral em cinco pacientes com distonia generalizada. Foram selecionados cinco pacientes com distonia generalizada, predominante axial, refratários ao tratamento farmacológico (quatro idiopáticas e uma pós-traumática). A severidade da distonia foi avaliada através da escala de Burke-Fahn-Marsden (BFM), no 1º, 2º, e 3º dia após a cirurgia e nos dias 30, 60, 90, 120 e 180 do pós-operatório. Quatro pacientes com distonia idiopática tiveram uma progressiva melhora dentro de 3 meses após a cirurgia e o paciente com distonia pós-traumática teve uma piora da distonia após 3 meses da cirurgia. Um dos pacientes teve uma melhora acentuada do quadro de distonia, ficando livre das medicações. Em média ocorreu redução de 52,58 % dos escores da escala de BFM. Um dos...

Safety and efficacy of unilateral and bilateral pallidotomy for primary dystonia

“…Previous studies on bilateral pallidotomy for dystonia have reported excellent results with a BFMDRS score improvement of 50%–90%, which is consistent with our findings (74.0% improvement of BFMDRS) 10,12,14,23,28,29 . A recent study by Gross et al reported that bilateral pallidotomy using laser interstitial thermal therapy in two dystonia patients led to a 40.4% and 45.7% BFMDRS score improvement at 1‐year follow‐up 22 .…”

“…The current literature on the efficacy of pallidotomy is derived from small case studies and reports using objective Previous studies on bilateral pallidotomy for dystonia have reported excellent results with a BFMDRS score improvement of 50%-90%, which is consistent with our findings (74.0% improvement of BFMDRS). 10,12,14,23,28,29 A recent study by Gross et al reported that bilateral pallidotomy using laser interstitial thermal therapy in two dystonia patients led to a 40.4% and 45.7% BFMDRS score improvement at 1-year follow-up. 22 Contrastingly, Lin et al reported BFMDRS score improvement of only 13% after bilateral pallidotomy at 1-year follow-up in 18 patients with generalized dystonia.…”

Safety and efficacy of unilateral and bilateral pallidotomy for primary dystonia

“…Previous studies on bilateral pallidotomy for dystonia have reported excellent results with a BFMDRS score improvement of 50%–90%, which is consistent with our findings (74.0% improvement of BFMDRS) 10,12,14,23,28,29 . A recent study by Gross et al reported that bilateral pallidotomy using laser interstitial thermal therapy in two dystonia patients led to a 40.4% and 45.7% BFMDRS score improvement at 1‐year follow‐up 22 .…”

“…The current literature on the efficacy of pallidotomy is derived from small case studies and reports using objective Previous studies on bilateral pallidotomy for dystonia have reported excellent results with a BFMDRS score improvement of 50%-90%, which is consistent with our findings (74.0% improvement of BFMDRS). 10,12,14,23,28,29 A recent study by Gross et al reported that bilateral pallidotomy using laser interstitial thermal therapy in two dystonia patients led to a 40.4% and 45.7% BFMDRS score improvement at 1-year follow-up. 22 Contrastingly, Lin et al reported BFMDRS score improvement of only 13% after bilateral pallidotomy at 1-year follow-up in 18 patients with generalized dystonia.…”

Bilateral pallidotomy for acquired or heredodegenerative generalized dystonia in children

“…Ten patients (8 male and 2 female) were included in this study (Table 1). The mean ± SD (range) age of the patients was 11.1 ± 4.1 (5)(6)(7)(8)(9)(10)(11)(12)(13)(14)(15)(16)(17) years. The mean duration between disease onset and surgical intervention was 3.9 ± 3.0 (1-9) years.…”

“…Most reports of the use of pallidotomy for AGD are in the form of short series. 12,17,29,32,44 The poor results of pallidotomy in patients with AGD may be a reason for the lack of available studies in the literature. Many studies collectively reported the outcomes of isolated dystonia and AGD.…”

Bilateral Pallidotomy for Dystonia: A Systematic Review

“…However, due to scarcity of literature, non‐English papers were also assessed. Finally, 33 papers met our inclusion criteria and were included in this review 15,17‐48 . The reasons to exclude the other 133 articles were the following: congress abstracts (n = 37), described no patients (n = 25), full text not available (n = 18), unilateral pallidotomy (n = 15), Parkinson's disease (n = 12), no (motor) outcomes (n = 9), patients described before (n = 7), thalamotomy or other lesions (n = 6), no dystonia patients (n = 2), and DBS only (n = 2) ( FIGURE S1).…”

Safety and efficacy of unilateral and bilateral pallidotomy for primary dystonia

“…Previous studies on bilateral pallidotomy for dystonia have reported excellent results with a BFMDRS score improvement of 50%–90%, which is consistent with our findings (74.0% improvement of BFMDRS) 10,12,14,23,28,29 . A recent study by Gross et al reported that bilateral pallidotomy using laser interstitial thermal therapy in two dystonia patients led to a 40.4% and 45.7% BFMDRS score improvement at 1‐year follow‐up 22 .…”

“…The current literature on the efficacy of pallidotomy is derived from small case studies and reports using objective Previous studies on bilateral pallidotomy for dystonia have reported excellent results with a BFMDRS score improvement of 50%-90%, which is consistent with our findings (74.0% improvement of BFMDRS). 10,12,14,23,28,29 A recent study by Gross et al reported that bilateral pallidotomy using laser interstitial thermal therapy in two dystonia patients led to a 40.4% and 45.7% BFMDRS score improvement at 1-year follow-up. 22 Contrastingly, Lin et al reported BFMDRS score improvement of only 13% after bilateral pallidotomy at 1-year follow-up in 18 patients with generalized dystonia.…”

Bilateral pallidotomy for acquired or heredodegenerative generalized dystonia in children

“…Ten patients (8 male and 2 female) were included in this study (Table 1). The mean ± SD (range) age of the patients was 11.1 ± 4.1 (5)(6)(7)(8)(9)(10)(11)(12)(13)(14)(15)(16)(17) years. The mean duration between disease onset and surgical intervention was 3.9 ± 3.0 (1-9) years.…”

“…Most reports of the use of pallidotomy for AGD are in the form of short series. 12,17,29,32,44 The poor results of pallidotomy in patients with AGD may be a reason for the lack of available studies in the literature. Many studies collectively reported the outcomes of isolated dystonia and AGD.…”

Bilateral Pallidotomy for Dystonia: A Systematic Review

“…However, due to scarcity of literature, non‐English papers were also assessed. Finally, 33 papers met our inclusion criteria and were included in this review 15,17‐48 . The reasons to exclude the other 133 articles were the following: congress abstracts (n = 37), described no patients (n = 25), full text not available (n = 18), unilateral pallidotomy (n = 15), Parkinson's disease (n = 12), no (motor) outcomes (n = 9), patients described before (n = 7), thalamotomy or other lesions (n = 6), no dystonia patients (n = 2), and DBS only (n = 2) ( FIGURE S1).…”

Safety and efficacy of unilateral and bilateral pallidotomy for primary dystonia

“…Previous studies on bilateral pallidotomy for dystonia have reported excellent results with a BFMDRS score improvement of 50%–90%, which is consistent with our findings (74.0% improvement of BFMDRS) 10,12,14,23,28,29 . A recent study by Gross et al reported that bilateral pallidotomy using laser interstitial thermal therapy in two dystonia patients led to a 40.4% and 45.7% BFMDRS score improvement at 1‐year follow‐up 22 .…”

“…The current literature on the efficacy of pallidotomy is derived from small case studies and reports using objective Previous studies on bilateral pallidotomy for dystonia have reported excellent results with a BFMDRS score improvement of 50%-90%, which is consistent with our findings (74.0% improvement of BFMDRS). 10,12,14,23,28,29 A recent study by Gross et al reported that bilateral pallidotomy using laser interstitial thermal therapy in two dystonia patients led to a 40.4% and 45.7% BFMDRS score improvement at 1-year follow-up. 22 Contrastingly, Lin et al reported BFMDRS score improvement of only 13% after bilateral pallidotomy at 1-year follow-up in 18 patients with generalized dystonia.…”

Bilateral pallidotomy for acquired or heredodegenerative generalized dystonia in children

“…Ten patients (8 male and 2 female) were included in this study (Table 1). The mean ± SD (range) age of the patients was 11.1 ± 4.1 (5)(6)(7)(8)(9)(10)(11)(12)(13)(14)(15)(16)(17) years. The mean duration between disease onset and surgical intervention was 3.9 ± 3.0 (1-9) years.…”

“…Most reports of the use of pallidotomy for AGD are in the form of short series. 12,17,29,32,44 The poor results of pallidotomy in patients with AGD may be a reason for the lack of available studies in the literature. Many studies collectively reported the outcomes of isolated dystonia and AGD.…”

Bilateral Pallidotomy for Dystonia: A Systematic Review

“…However, due to scarcity of literature, non‐English papers were also assessed. Finally, 33 papers met our inclusion criteria and were included in this review 15,17‐48 . The reasons to exclude the other 133 articles were the following: congress abstracts (n = 37), described no patients (n = 25), full text not available (n = 18), unilateral pallidotomy (n = 15), Parkinson's disease (n = 12), no (motor) outcomes (n = 9), patients described before (n = 7), thalamotomy or other lesions (n = 6), no dystonia patients (n = 2), and DBS only (n = 2) ( FIGURE S1).…”