Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
Wilson disease is a genetic disorder resulting in copper accumulation in liver, brain and eye. The neurologic complications include dystonic syndrome, which is a prolonged and excessive muscle activation responsible for abnormal postures. Hand dystonia prevents daily life activities such as writing, which is particularly disabling, since writing is the only mean of communication in these patients with significant slurred speech. Treatment is limited and only partially effective.
Low frequency (\
This study investigates the handwriting performance of an homogeneous cohort of patients with Wilson disease and right handed dystonia, after one single inhibitory repetitive transcranial magnetic stimulation (rTMS). Fifteen patients with focal right hand dystonia will receive randomly either active or sham rTMS (1 Hz) to the left somatosensory cortex (SSC) in one single 20 minutes session. Handwriting performance will be measured immediately after this unique session (Visual analogic scale of subjective discomfort in writing, DPRE and WCRS scales, pen pressure and pen velocity measured on touchpad), compared to scores obtained 24 hours before the session. Three days later, the patient will receive the other session (sham rTMS or active rTMS) and the same parameters will be evaluated.
This is a single-center, randomized, crossover, prospective, clinical and double-blind study (the rTMS session is performed by the neurophysiologist, but the patient and the neurologist who will perform the handwriting evaluation are blind to the session).
Not provided
Not provided
Not provided
Not provided
| Label | Type | Description | Intervention Names |
|---|---|---|---|
| Active rTMS | Experimental | Repetitive Transcranial Magnetic stimulation: 1 Hz rTMS, delivered to left somatosensory cortex during rest. Intervention is delivered during 20 minutes in one single session. |
|
| Sham rTMS | Sham Comparator | Placebo Transcranial Magnetic stimulation delivered to left somatosensory cortex during rest. Intervention is delivered during 20 minutes in one single session. |
|
| Name | Type | Description | Arm Group Labels | Other Names |
|---|---|---|---|---|
| Repetitive Transcranial Magnetic Stimulation (rTMS) | Device | Inhibitory 1 Hz rTMS, delivered to left somatosensory cortex during rest. Intervention is delivered during 20 minutes in one single session. |
| Measure | Description | Time Frame |
|---|---|---|
| immediate and significant improvement in writing | Improvement of handwriting performance immediately after one single 20 minutes session of inhibitory (1 Hz) repetitive transcranial magnetic stimulation over the left somatosensory cortex. quantitative test score of writing WCRS Active or sham stimulation will be randomly assigned to the patient and the other session will be performed 3 days later. | up to 1 week |
| Measure | Description | Time Frame |
|---|---|---|
| significant improvement in writing | improvement of other quantitative test scores of writing (VAS subjective discomfort writing, DPRE scales and parameters measured on touchpad) after a single session of 1 Hz rTMS inhibitory to the left CSS , compared to the scores achieved in the 24 hours before the session of rTMS. Active or sham stimulation will be randomly assigned to the patient and the other session will be performed 3 days later. |
Not provided
Inclusion Criteria:
Exclusion Criteria:
Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
| Name | Affiliation | Role |
|---|---|---|
| Nathalie KUBIS, MD, PhD | Physiology Department, Lariboisière Hospital | Study Director |
| Jean-Marc TROCELLO, MD | Neurology, Lariboisière Hospital | Principal Investigator |
| Facility | Status | City | State | ZIP | Country | Contacts |
|---|---|---|---|---|---|---|
| Hospital Lariboisiere | Paris | ÃŽle-de-France Region | 75010 | France |
Not provided
Not provided
Not provided
Not provided
Not provided
| pre and post-rTMS electroencephalogram | Other | to verify the absence of infraclinical seizures |
|
| WCRS | Other | Writer's cramp rating scale |
|
| handwriting scale DPRE | Other | handwriting in development and being evaluated by the NRC Wilson |
|
| visual analog scale of discomfort writing and parameters collected on touchpad | Other |
|
| up to 1 week |
| improvement of dystonia on the scale WDRS | Assessing the improvement of dystonia on the scale WDRS after a single session of 1 Hz rTMS inhibitory to the left CSS, compared to the scores achieved in the 24 hours before the session of rTMS. Active or sham stimulation will be randomly assigned to the patient and the other session will be performed 3 days later. | up to 1 week |
| correlation between the scores and the other parameters of disease severity | Assess whether age, disease duration and the presence of cortical atrophy are inversely correlated with the evolution of scores. | up to 1 week |
| ID | Term |
|---|---|
| D006527 | Hepatolenticular Degeneration |
| D009069 | Movement Disorders |
| D004421 | Dystonia |
| ID | Term |
|---|---|
| D008107 | Liver Diseases |
| D004066 | Digestive System Diseases |
| D001480 | Basal Ganglia Diseases |
| D001927 | Brain Diseases |
| D002493 | Central Nervous System Diseases |
| D009422 | Nervous System Diseases |
| D020739 | Brain Diseases, Metabolic, Inborn |
| D001928 | Brain Diseases, Metabolic |
| D020271 | Heredodegenerative Disorders, Nervous System |
| D019636 | Neurodegenerative Diseases |
| D030342 | Genetic Diseases, Inborn |
| D009358 | Congenital, Hereditary, and Neonatal Diseases and Abnormalities |
| D008661 | Metabolism, Inborn Errors |
| D008664 | Metal Metabolism, Inborn Errors |
| D008659 | Metabolic Diseases |
| D009750 | Nutritional and Metabolic Diseases |
| D020820 | Dyskinesias |
| D009461 | Neurologic Manifestations |
| D012816 | Signs and Symptoms |
| D013568 | Pathological Conditions, Signs and Symptoms |
Not provided
Not provided
| ID | Term |
|---|---|
| D050781 | Transcranial Magnetic Stimulation |
| D007854 | Lead |
| ID | Term |
|---|---|
| D055909 | Magnetic Field Therapy |
| D013812 | Therapeutics |
| D019216 | Metals, Heavy |
| D004602 | Elements |
| D007287 | Inorganic Chemicals |
| D008670 | Metals |
Not provided
Not provided