Safety and efficacy of high-dose enteral, intravenous, and transdermal clonidine for the acute management of severe intractable childhood dystonia and status dystonicus: An illustrative case-series

Nakou, Vasiliki; Williamson, Kathleen A.; Lumsden, Daniel E.; Tomlin, Steve; Kaminska, Margaret; Lin, Jean-Pierre

doi:10.1016/j.ejpn.2017.07.007

Cited by 20 publications

(12 citation statements)

References 20 publications

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“…As an alternative to benzodiazepines, clonidine is a treatment option for severe dystonia in children . The role of clonidine in acute dystonic exacerbation management has been emphasized . The main advantages of clonidine are the lack of respiratory depressant effects and the availability of multiple routes of administration.…”

Section: Discussionmentioning

confidence: 99%

Prestatus and status dystonicus in children and adolescents

Garone

Graziola

Nicita

et al. 2019

Develop Med Child Neuro

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Aim To critically analyse the management of status dystonicus and prestatus dystonicus in children and adolescents, in order to examine clinical features, acute management, and risk of relapse in a paediatric cohort. Method Clinical, demographic, and therapeutic features were analysed according to disease severity. Risk of subsequent relapse was estimated through Kaplan–Meier curves. Results Thirty‐four patients (eight females, 26 males) experiencing 63 episodes of acute dystonia exacerbations at a tertiary referral Italian hospital were identified. Mean age at status dystonicus presentation was 9 years 11 months (11y at inclusion in the study). Onset of dystonia dated back to infancy in most cases. Fourteen patients experienced two or more episodes. Infections were the most common trigger (48%). Benzodiazepines were the most commonly used drugs for acute management. Stereotactic pallidotomy was performed in six cases during status dystonicus, and in two additional patients it was electively performed after medical management. The probability of survival free from status dystonicus relapses was 78% after 4 months and 61% after 27 months. Interpretation Dystonia exacerbations are potentially life‐threating emergencies, with a considerable risk of relapse. Nevertheless, no obvious factors for relapse risk stratification exist. Pallidotomy is a feasible option in medical refractory status dystonicus for patients with limited deep brain stimulation applicability, but the risk of recurrence is elevated. What this paper adds Acute exacerbations may affect up to 10% of children with dystonia. Infections are the most common precipitant factor. In about 30% of the cases, intensive care unit admission is needed. Subsequent relapses are common, reaching 25% risk at 1 year. Pallidotomy can be considered in medical‐refractory cases with no deep brain stimulation applicability.

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Section: Discussionmentioning

confidence: 99%

Prestatus and status dystonicus in children and adolescents

Garone

Graziola

Nicita

et al. 2019

Develop Med Child Neuro

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“…So far, there are only a few reports of evolution after SD with improvement of dystonia and even fewer on the resolution of SD for a longer period . Although algorithms have been proposed to treat SD, no consensus is available regarding the optimal strategy . Patients were treated with DBS for SD episodes following failure of medical management, usually transferred directly from remote hospital ICU to internal ICU.…”

Section: Discussionmentioning

confidence: 99%

Deep brain stimulation treated dystonia‐trajectory via status dystonicus

et al. 2018

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“…Clonidine can also help with sleep induction, calming, and reducing anxiety. Other than tic disorders, and treating psychiatric comorbidity, clonidine can be useful in status dystonicus, such as patients with GNAO1 dyskinetic exacerbations . In patients with tic disorders, clonidine may be used only at night (50–100 μg), or in a twice‐daily regimen.…”

Section: Symptomatic Therapiesmentioning

confidence: 99%

“…In patients with tic disorders, clonidine may be used only at night (50–100 μg), or in a twice‐daily regimen. In emergency settings, such as status dystonicus, clonidine can be used more regularly, such as 50 μg every 2 hours (dose equivalents up to 2.5 μg/kg/hr) with increased doses at night, to induce sedation. Oral clonidine can be effective and reduce the need for intravenous sedation in status dystonicus.…”

Section: Symptomatic Therapiesmentioning

confidence: 99%

“…Other than tic disorders, and treating psychiatric comorbidity, clonidine can be useful in status dystonicus, such as patients with GNAO1 dyskinetic exacerbations. [121][122][123] In patients with tic disorders, clonidine may be used only at night (50-100 μg), or in a twice-daily regimen. In emergency settings, such as status dystonicus, clonidine can be used more regularly, such as 50 μg every 2 hours (dose equivalents up to 2.5 μg/kg/hr 121 ) with increased doses at night, to induce sedation.…”

Section: Alpha Agonistsmentioning

confidence: 99%

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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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Safety and efficacy of high-dose enteral, intravenous, and transdermal clonidine for the acute management of severe intractable childhood dystonia and status dystonicus: An illustrative case-series

Abstract: High dose clonidine administered via different routes can be used in the acute management of severe exacerbations of dystonia. Its use in our cohort was not associated with significant cardio-respiratory depression even at doses as high as 9 mcg/kg/h.

Cited by 20 publications

References 20 publications

Prestatus and status dystonicus in children and adolescents

Prestatus and status dystonicus in children and adolescents

Deep brain stimulation treated dystonia‐trajectory via status dystonicus

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

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