|First Submitted Date||September 10, 2011|
|First Posted Date||September 13, 2011|
|Last Update Posted Date||July 2, 2017|
|Start Date||August 26, 2011|
|Primary Completion Date||Not Provided|
|Current Primary Outcome Measures
|Original Primary Outcome Measures||Not Provided|
|Change History||Complete list of historical versions of study NCT01432899 on ClinicalTrials.gov Archive Site|
|Current Secondary Outcome Measures
||This protocol has 5 Objectives, each with its own primary and secondary objectives. They are too numerous to list.|
|Original Secondary Outcome Measures||Not Provided|
|Current Other Outcome Measures||Not Provided|
|Original Other Outcome Measures||Not Provided|
|Brief Title||Studying Childhood-Onset Hemidystonia|
|Official Title||Characteristics and Mechanism of Childhood-Onset Hemidystonia|
- Childhood-onset dystonia is caused by a brain injury. It causes muscle contractions and twisting movements that the person with dystonia cannot control. The term hemidystonia is used when only one side of the body is affected. When dystonia starts during childhood, the brain and nerves may not develop normally. People with hemidystonia can become disabled because of the unwanted postures and twisting that dystonia causes. More research is needed to determine how dystonia affects brain development.
- To study brain function in people with hemidystonia.
In childhood-onset hemidystonia, motor and sensory abnormalities pose a great challenge to voluntary movement. In an effort to inform future methods of treatment of this disorder, this study seeks to (1) characterize involuntary muscle activity, (2) explore neurophysiologic mechanisms of involuntary muscle activity, (3) characterize abnormalities of sensation, (4) assess performance on two sensorimotor tasks (voluntary postural control, and a reach-to-grasp arm movement), and (5) explore brain abnormalities using imaging in childhood-onset hemidystonia.
The dystonia group will consist of 40 individuals (age 7-40 years) with childhood-onset hemidystonia. The control group will consist of 40 individuals within the same age range with no neurological disorders.
This is a cross-sectional study, in which multiple assessments will be performed over a short period. Results in the dystonia group will be compared to results in the control group. Due to the laterality of brain injury in hemidystonia, outcomes from both arms and cortical hemispheres will be compared to each other. Outcomes within the dystonia group will be correlated with validated dystonia rating scales as well as with the age at the time of injury.
In Objective 1, we will analyze the timing of involuntary electromyographic (EMG) activity and kinematics triggered in the dystonic arm during various tasks. In Objective 2, we will investigate various types of intracortical inhibition (short intracortical inhibition, long intracortical inhibition, contralateral silent period, and ipsilateral silent period) and intracortical facilitation in motor cortical regions controlling the dystonic wrist flexor during voluntary contraction of the wrist extensor using transcranial magnetic stimulation (TMS). In addition, we will perform a coherence analysis between EMG activity in the wrist flexor and extensor muscles and between electroencephalographic (EEG) activity in the primary motor cortex and the contralateral wrist muscles. In Objective 3, we will test tactile sense (spatial and temporal), vibration sense, and stereognosis of the hand. In Objective 4, we will analyze seated limits of stability (voluntary postural control), and the timing and amplitude of EMG activity and kinematics in the arm during a reaching and grasping arm movement. In Objective 5, we will obtain brain scans with structural magnetic resonance imaging (MRI), diffusion tensor imaging (DTI), resting state functional connectivity MRI, and functional MRI during a simple task.
|Study Design||Observational Model: Case-Control
Time Perspective: Prospective
|Target Follow-Up Duration||Not Provided|
|Sampling Method||Not Provided|
|Study Population||Not Provided|
|Study Groups/Cohorts||Not Provided|
* Includes publications given by the data provider as well as publications identified by ClinicalTrials.gov Identifier (NCT Number) in Medline.
|Completion Date||Not Provided|
|Primary Completion Date||Not Provided|
Additional inclusion criteria for individuals with dystonia:
- Childhood-onset (before 13 years of age) hemidystonia in one wrist. Diagnosis of dystonia will be made based on the Hypertonia Assessment Tool (HAT)
-Presence of any neurological disorders
Individuals with Dystonia:
Additional exclusion criteria for TMS:
Additional exclusion criteria for MRI:
|Ages||7 Years to 40 Years (Child, Adult)|
|Accepts Healthy Volunteers||Yes|
|Contacts||Contact information is only displayed when the study is recruiting subjects|
|Listed Location Countries||United States|
|Removed Location Countries|
|Other Study ID Numbers||110246
|Has Data Monitoring Committee||Not Provided|
|U.S. FDA-regulated Product||Not Provided|
|IPD Sharing Statement||Not Provided|
|Responsible Party||National Institutes of Health Clinical Center (CC)|
|Study Sponsor||National Institutes of Health Clinical Center (CC)|
|PRS Account||National Institutes of Health Clinical Center (CC)|
|Verification Date||March 9, 2017|