Which patients with dystonia benefit from deep brain stimulation? A metaregression of individual patient outcomes

Andrews, Caroline; Avilés-Olmos, Icíar; Hariz, Marwan; Foltynie, Thomas

doi:10.1136/jnnp.2010.207993

Cited by 179 publications

(144 citation statements)

References 110 publications

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“…Of particularly interest for our presented cohort is how classification could guide expectations regarding outcome following DBS. Prognostic factors for outcome following pallidal DBS remain largely unclear, though one clear finding is that, taken collectively, dystonia previously categorised as "secondary" is less responsive than dystonia previously catergorised as primary [12]. However, even this apparently clear cut relationship has it's exceptions (e.g.…”

Section: Accepted Manuscriptmentioning

confidence: 85%

Classification of dystonia in childhood

Lumsden

Gimeno

Lin

2016

Parkinsonism & Related Disorders

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Objective:The most recent international consensus update on dystonia classification proposed a system based on 2 axes, clinical characteristics and aetiology. We aimed to apply this system to Children and Young People (CAYP) selected for movement disorder surgery, and determine if meaningful groupings of cases could be extracted. Methods:The 2013 Consensus Committee classification system for dystonia was retrospectively applied to 145 CAYP with dystonic movement disorders. Twostep cluster analysis was applied to the resulting categorisations to identify groupings of CAYP with similar characteristics. ResultsClassification resulted in a total of 43 unique groupings of categorisation.Cluster analysis detected 4 main clusters of CAYP, comparable to previously used patient groupings. ConclusionsThe 2013 consensus update on dystonia classification can be applied to CAYP with dystonia. The large number of categories provides a wealth of information for the clinician, and also facilitates data driven grouping into clinically meaningful subgroups.

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Section: Accepted Manuscriptmentioning

confidence: 85%

Classification of dystonia in childhood

Lumsden

Gimeno

Lin

2016

Parkinsonism & Related Disorders

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“…Due to the fact that several studies indicate a weaker effect of DBS for the treatment of secondary dystonia in comparison to primary forms,18, 19 conventional treatment regimen with botulinum toxin was preferred.…”

Section: Discussionmentioning

confidence: 99%

Cervical dystonia caused by focal putaminal ischemia

Dinkelbach

Hartmann

Mathys

et al. 2015

Ann Clin Transl Neurol

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We describe a 48‐year‐old woman with putaminal gliosis and a sphenoid wing meningioma at the left, who developed dystonia restricted to cervical regions. We propose the following causal chain: the meningioma led to an occlusion of a lenticulo‐striatal branch of the middle cerebral artery that caused ventral putaminal ischemia and finally resulting in symptomatic dystonia. The previously reported relevance of the infarcted regions to the pathophysiology of dystonia supports this assumption. Implications for the diagnostic procedure of dystonia will be discussed.

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“…Deep brain stimulation (DBS) has demonstrated variable effectiveness in secondary dystonias caused by inborn errors of metabolism (e.g., homocystinuria, PKAN, GM1-gangliosidosis) (Andrews et al 2010;Aydin et al 2011). The present report of DBS in a now 22-year-old man with a diagnosis of X-linked adrenoleukodystrophy (X-ALD, OMIM# 300100) (Vidailhet et al 2005) is its first reported application in X-ALD.…”

Section: Reportmentioning

confidence: 99%

Deep Brain Stimulation and Dantrolene for Secondary Dystonia in X-Linked Adrenoleukodystrophy

et al. 2014

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Deep brain stimulation (DBS) has been used to treat secondary dystonias caused by inborn errors of metabolism with varying degrees of effectiveness. Here we report for the first time the application of DBS as treatment for secondary dystonia in a 22-year-old male with X-linked adrenoleukodystrophy (X-ALD). The disease manifested at age 6 with ADHD, tics, and dystonic gait, and deteriorated to loss of ambulation by age 11, and speech difficulties, seizures, and characteristic adrenal insufficiency by age 16. DBS in the globus pallidus internus was commenced at age 18. However, after 25 months, no improvement in dystonia was observed (Burke-Fahn-Marsden (BFM) scores of 65.5 and 62 and disability scores of 28 and 26, pre-and post-DBS, respectively) and the DBS device was removed. Treatment with dantrolene reduced skeletal muscle tone and improved movement (Global Dystonia Rating Scores from 5 to 1 and BFM score 42). Therefore, we conclude that DBS was a safe but ineffective intervention in our case with longstanding dystonia, whereas treatment of spasticity with dantrolene did improve the movement disorder in this young man with X-ALD. ReportDeep brain stimulation (DBS) has demonstrated variable effectiveness in secondary dystonias caused by inborn errors of metabolism (e.g., homocystinuria, PKAN, GM1-gangliosidosis) (Andrews et al. 2010;Aydin et al. 2011). The present report of DBS in a now 22-year-old man with a diagnosis of X-linked adrenoleukodystrophy (X-ALD, OMIM# 300100) (Vidailhet et al. 2005) is its first reported application in X-ALD.The patient presented at age 6 with ADHD, tics, and a dystonic gait. Neuroimaging revealed symmetrical occipito-parietal demyelination and abnormal signal intensities in the basal ganglia and thalamus (Fig. 1). Diagnosis was confirmed by elevated very long chain fatty acids in plasma. He lost the ability to ambulate within 5 years, and was wheelchair-bound by age 11. His speech deteriorated and was barely intelligible by age 16. Seizure disorder and adrenal insufficiency were effectively controlled with carbamazepine and clobazam, and hydrocortisone, respectively. Initially the dystonia affected only the lower extremities, but quickly generalized. At age 11 years, mild athetoid movements of the head, neck, and extremities were observed. He held the neck in a flexed position, but was able to extend it on request. He had a tendency to elevate the right upper extremity, with his right wrist in a flexed position and fingers extended. There was mild intermittent flexion of the wrist. Knees were held in an extended position, with ankles plantar flexed and feet inverted (left greater than right). Tone was difficult to assess due to the dystonia. Deep tendon reflexes were brisk, especially at the knees; plantar responses were difficult to assess due to dystonia. Hoffman's sign was absent.For dystonia, botox, levodopa/carbidopamine, trihexyphenidyl, pergolide, and pramipexole were ineffective. Intrathecal baclofen to reduce muscle tone was refused by the patient.Repeat gadoli...

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Which patients with dystonia benefit from deep brain stimulation? A metaregression of individual patient outcomes

Cited by 179 publications

References 110 publications

Classification of dystonia in childhood

Classification of dystonia in childhood

Cervical dystonia caused by focal putaminal ischemia

Deep Brain Stimulation and Dantrolene for Secondary Dystonia in X-Linked Adrenoleukodystrophy

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