Dataset Information

Similarity of Involuntary Postures between Different Children with Dystonia.

ABSTRACT:

Background

Abnormal involuntary postures are characteristic of dystonia, but the specific postures observed clinically have not previously been categorized or enumerated. The objective of this study was to determine whether there is a set of specific postures that are common between different children with dystonia.

Methods

Videotapes were examined from all children who were seen in a pediatric movement disorders clinic over a 4-year period and had a diagnosis of nonpsychogenic dystonia. In total, 179 children were included in the video review.

Results

Visually similar postures were identified in 152 different children. Seven different common postures were identified. All 152 children exhibited at least 1 of these postures, and most had more than 1.

Conclusions

Involuntary postures in childhood dystonia exhibit unexpected similarities despite a wide range of underlying etiology, severity, and developmental experience. This is consistent with the hypothesis that childhood dystonia is a symptom that reflects a shared pathway of expression for multiple anatomic and functional abnormalities.

SUBMITTER: Sanger TD

PROVIDER: S-EPMC6407060 | biostudies-literature | 2017 Nov-Dec

REPOSITORIES: biostudies-literature

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Publications

Similarity of Involuntary Postures between Different Children with Dystonia.

Sanger Terence D TD Ferman Diana D

Movement disorders clinical practice 20170914 6

<h4>Background</h4>Abnormal involuntary postures are characteristic of dystonia, but the specific postures observed clinically have not previously been categorized or enumerated. The objective of this study was to determine whether there is a set of specific postures that are common between different children with dystonia.<h4>Methods</h4>Videotapes were examined from all children who were seen in a pediatric movement disorders clinic over a 4-year period and had a diagnosis of nonpsychogenic dy ...[more]

PMID: 30868098

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Project description:Background:The human hand can perform a range of manipulation tasks, from holding a pen to holding a hammer. The central nervous system (CNS) uses different strategies in different manipulation tasks based on task requirements. Attempts to compare postures of the hand have been made for use in robotics and animation industries. In this study, we developed an index called the posture similarity index to quantify the similarity between two human hand postures. Methods:Twelve right-handed volunteers performed 70 postures, and lifted and held 30 objects (total of 100 different postures, each performed five times). A 16-sensor electromagnetic tracking system captured the kinematics of individual finger phalanges (segments). We modeled the hand as a 21-DoF system and computed the corresponding joint angles. We used principal component analysis to extract kinematic synergies from this 21-DoF data. We developed a posture similarity index (PSI), that represents the similarity between posture in the synergy (Principal component) space. First, we tested the performance of this index using a synthetic dataset. After confirming that it performs well with the synthetic dataset, we used it to analyze the experimental data. Further, we used PSI to identify postures that are "representative" in the sense that they have a greater overlap (in synergy space) with a large number of postures. Results:Our results confirmed that PSI is a relatively accurate index of similarity in synergy space both with synthetic data and real experimental data. Also, more special postures than common postures were found among "representative" postures. Conclusion:We developed an index for comparing posture similarity in synergy space and demonstrated its utility by using synthetic dataset and experimental dataset. Besides, we found that "special" postures are actually "special" in the sense that there are more of them in the "representative" postures as identified by our posture similarity index.

Project description:BACKGROUND:The pathophysiology underlying different types of dystonia is not yet understood. We report microelectrode data from the globus pallidus interna (GPi) and globus pallidus externa (GPe) in children undergoing deep brain stimulation (DBS) for dystonia and investigate whether GPi and GPe firing rates differ between dystonia types. METHODS:Single pass microelectrode data were obtained to guide electrode position in 44 children (3.3-18.1?years, median 10.7) with the following dystonia types: 14 primary, 22 secondary Static and 8 progressive secondary to neuronal brain iron accumulation (NBIA). Preoperative stereotactic MRI determined coordinates for the GPi target. Digitised spike trains were analysed offline, blind to clinical data. Electrode placement was confirmed by a postoperative stereotactic CT scan. FINDINGS:We identified 263 GPi and 87 GPe cells. Both GPi and GPe firing frequencies differed significantly with dystonia aetiology. The median GPi firing frequency was higher in the primary group than in the secondary static group (13.5?Hz vs 9.6?Hz; p=0.002) and higher in the NBIA group than in either the primary (25?Hz vs 13.5?Hz; p=0.006) or the secondary static group (25?Hz vs 9.6?Hz; p=0.00004). The median GPe firing frequency was higher in the NBIA group than in the secondary static group (15.9?Hz vs 7?Hz; p=0.013). The NBIA group also showed a higher proportion of regularly firing GPi cells compared with the other groups (p<0.001). A higher proportion of regular GPi cells was also seen in patients with fixed/tonic dystonia compared with a phasic/dynamic dystonia phenotype (p<0.001). The GPi firing frequency showed a positive correlation with 1-year outcome from DBS measured by improvement in the Burke-Fahn-Marsden Dystonia Rating Scale (BFMDRS-m) score (p=0.030). This association was stronger for the non-progressive patients (p=0.006). INTERPRETATION:Pallidal firing rates and patterns differ significantly with dystonia aetiology and phenotype. Identification of specific firing patterns may help determine targets and patient-specific protocols for neuromodulation therapy. FUNDING:National Institute of Health Research, Guy's and St. Thomas' Charity, Dystonia Society UK, Action Medical Research, German National Academic Foundation.

Dataset Information

Similarity of Involuntary Postures between Different Children with Dystonia.

Background

Methods

Results

Conclusions

Publications

Similarity of Involuntary Postures between Different Children with Dystonia.

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