Globus pallidus internus deep brain stimulation for the treatment of status dystonicus in tardive dystonia

Trezza, Andrea; Antonini, Angelo; Sganzerla, Erik P.; Landi, Andrea

doi:10.1007/s00701-016-2887-0

Cited by 11 publications

(3 citation statements)

References 10 publications

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“…In 2012, the largest SD series was published: 68 patients undergoing 89 SD episodes allowed drawing some conclusion on the clinical expression, management strategies, and outcome of SD . During the past 5 years, isolated case reports and a small case series of SD have been published: a fatal refractory case in ataxia telangiectasia, 2 patients with Sex Determining Region Y‐box 2 (SOX2)‐Anophthalmia syndrome, a child with familial idiopathic hypoparathyroidism, 2 NBIA/DYT‐PANK2 patients, a chromosomopathy (deletion of the long arm of chromosome 22), a DYT/CHOR‐GCDH (glutaric aciduria type I), epileptic encephalopathy, idiopathic bilateral striatal necrosis, 3 idiopathic isolated dystonia, additional dyskinetic JCP patients, a 19‐year‐old boy affected by mental retardation and severe behavior disorder treated with high doses of haloperidol, tardive dystonia acutely worsened by the depletion of deep brain stimulation (DBS) implantable pulse generator, dystonia secondary to kernicterus, a series of 5 DYT‐TOR1A positive (DYT1) patients, and an isolated case report of a DYT‐TOR1A patient with SD as a result of implantable pulse generator depletion …”

Section: Historical Review and Current Definitionsmentioning

confidence: 99%

Rethinking status dystonicus

Ruiz-López

Fasano

2017

Movement Disorders

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Status dystonicus is a movement disorder emergency that has been a source of controversy in terms of terminology, phenomenology, and management since it was first described in 1982. Here we argue that the current use of the term status dystonicus falls well short of the precision needed for either clinical or academic use. We performed a critical review on this topic, describing possible pathophysiological mechanisms and areas of uncertainties. This review also addresses the problems derived by the extreme clinical heterogeneity of this condition, as the lack of an objective criterion useful for the definition, or the fact that status dystonicus may present not only in the context of a known dystonic syndrome. We propose a new possible definition that includes not only dystonia but also other hyperkinetic movements in the wide range of movement disorders that can be seen during an episode. The new definition keeps the term status dystonicus and highlights the fact that this is a medical emergency based on the impairment of bulbar and/or respiratory function requiring hospital admission as the principal feature. Furthermore, the new definition should not consider as necessary unspecific features as patient's condition at baseline, the distribution of dystonia, occurrence of systemic symptoms such as fever or laboratory findings. We hope that this proposal will stimulate the debate on this subject among our peers, further developing a clinical and pathophysiological understanding of status dystonicus. © 2017 International Parkinson and Movement Disorder Society.

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Section: Historical Review and Current Definitionsmentioning

confidence: 99%

Rethinking status dystonicus

Ruiz-López

Fasano

2017

Movement Disorders

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“…For symptomatic management, we find sedative agents, particularly oral clonidine, at frequent and high doses as a valuable tool for children with severe movement disorder exacerbations. Emergent DBS can be useful and life‐saving, as exemplified by several reports of GNAO1‐related movement disorders, but is limited by availability around the world.…”

Section: Symptomatic Therapiesmentioning

confidence: 99%

Current therapies and therapeutic decision making for childhood‐onset movement disorders

2019

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Movement disorders differ in children to adults. First, neurodevelopmental movement disorders such as tics and stereotypies are more prevalent than parkinsonism, and second, there is a genomic revolution which is now explaining many early‐onset dystonic syndromes. We outline an approach to children with movement disorders starting with defining the movement phenomenology, determining the level of functional impairment due to abnormal movements, and screening for comorbid psychiatric conditions and cognitive impairments which often contribute more to disability than the movements themselves. The rapid improvement in our understanding of the etiology of movement disorders has resulted in an increasing focus on precision medicine, targeting treatable conditions and defining modifiable disease processes. We profile some of the key disease‐modifying therapies in metabolic, neurotransmitter, inflammatory, and autoimmune conditions and the increasing focus on gene or cellular therapies. When no disease‐modifying therapies are possible, symptomatic therapies are often all that is available. These classically target dopaminergic, cholinergic, alpha‐adrenergic, or GABAergic neurochemistry. Increasing interest in neuromodulation has highlighted that some clinical syndromes respond better to DBS, and further highlights the importance of “disease‐specific” therapies with a future focus on individualized therapies according to the genomic findings or disease pathways that are disrupted. We summarize some pragmatic applications of symptomatic therapies, neuromodulation techniques, and some rehabilitative interventions and provide a contemporary overview of treatment in childhood‐onset movement disorders. © 2019 International Parkinson and Movement Disorder Society

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“…of clinical studies have shown the effectiveness of deep brain stimulation (DBS) of the globus pallidus internus (GPi) in cases of drug-resistant SD. 5,6,23,32,37,42,44,45 However, one shortcoming of DBS is the nonnegligible risk of hardware-related complications in the pediatric dystonia population. 28 In addition, several reports have described the onset of severe or fatal SD as a consequence of a DBS depleted battery or lead dislocation.…”

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confidence: 99%

Deep brain stimulation versus pallidotomy for status dystonicus: a single-center case series

Levi

Zorzi

Messina

et al. 2021

Journal of Neurosurgery

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OBJECTIVEFirst-line pharmacological therapies have shown limited efficacy in status dystonicus (SD), while surgery is increasingly reported as remediable in refractory cases. In this context, there is no evidence regarding which neurosurgical approach is the safest and most effective. The aim of this study was to assess the clinical outcomes and surgery-related complications of globus pallidus internus deep brain stimulation (GPi DBS) and pallidotomy for the treatment of drug-resistant SD.METHODSThe authors reviewed the records of patients with drug-resistant SD who had undergone GPi DBS or pallidotomy at their institution between 2003 and 2017. The severity of the dystonia was evaluated using the Barry-Albright Dystonia (BAD) Scale. Surgical procedures were performed bilaterally in all cases.RESULTSFourteen patients were eligible for inclusion in the study. After surgery, the mean follow-up was 40.6 ± 30 months. DBS ended the dystonic storm in 87.5% of cases (7/8), while pallidotomy had a success rate of 83.3% (5/6). No significant differences were observed between the two techniques in terms of failure rates (risk difference DBS vs pallidotomy −0.03, 95% CI −0.36 to 0.30), SD mean resolution time (DBS 34.8 ± 19 days, pallidotomy 21.8 ± 20.2 days, p > 0.05), or BAD scores at each postoperative follow-up (p > 0.05). The long-term hardware complication rate after DBS was 37.5%, whereas no surgery-related complications were noted following pallidotomy.CONCLUSIONSThe study data suggest that DBS and pallidotomy are equally safe and effective therapies for drug-resistant SD. The choice between the two techniques should be tailored on a case-by-case basis, depending on factors such as the etiology and evolution pattern of the underlying dystonia and the clinical conditions at the moment of SD onset. Given the limitation of the low statistical power of this study, further multicentric investigations are needed to confirm its findings.

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Globus pallidus internus deep brain stimulation for the treatment of status dystonicus in tardive dystonia

Cited by 11 publications

References 10 publications

Rethinking status dystonicus

Rethinking status dystonicus

Current therapies and therapeutic decision making for childhood‐onset movement disorders

Deep brain stimulation versus pallidotomy for status dystonicus: a single-center case series

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