Deep Brain Stimulation Therapy in Movement Disorders
Background: \- In deep brain stimulation (DBS), a device called a neurostimulator is placed in the chest. It is attached to wires in parts of the brain that affect movement. DBS might help people with movement disorders like Parkinson s disease (PD), dystonia, and essential tremor (ET).
Objective: \- To provide DBS treatment to people with some movement disorders.
Eligibility: \- Adults 18 years and older with PD, ET, or certain forms of dystonia.
Design: * Participants will be screened with medical history and physical exam. They will have blood and urine tests and: * MRI brain scan. The participant will lie on a table that slides in and out of a metal cylinder with a magnetic field. They will be in the scanner about 60 minutes. They will get earplugs for the loud noises. During part of the MRI, a needle will guide a thin plastic tube into an arm vein and a dye will be injected. * Electrocardiogram. Metal disks or sticky pads will be placed on the chest, arms, and legs. They record heart activity. * Chest X-ray. * Tests of memory, attention, concentration, thinking, and movement. * Eligible participants will have DBS surgery. The surgery and hospital care afterward are NOT part of this protocol. * Study doctors will see participants 3 4 weeks after surgery to turn on the neurostimulator. * Participants will return every month for 3 months, then every 3 months during the first year, and every 6 months during the second year. Each time, participants will be examined and answer questions. DBS placement will be evaluated with MRI. The neurostimulator will be programmed. At two visits, participants will have tests of movements, thinking, and memory.
• To be eligible for entry into the study, candidates must meet all the following criteria:
• Be 18 years of age or older.
• Able to comply with study procedures and provide informed consent.
• Have a clinical diagnosis of idiopathic PD, primary dystonia, or ET:
∙ The diagnosis of idiopathic PD will be based on the UK Brain Bank Criteria, and confirmed by the Movement Disorders Neurologists in the NIH Parkinson Clinic.
‣ The diagnosis of primary (generalized or segmental), hemidystonia, or cervical dystonia will be confirmed on clinical examination in the NIH Movement Disorders Clinic.
‣ The diagnosis of ET will be confirmed on clinical examination in the NIH Movement Disorders Clinic (the diagnosis of ET will be based on bilateral, largely symmetric postural or kinetic tremor involving hands and forearms that is visible and persistent. Additional or isolated tremor in head may be present but there should be the absence of abnormal posturing).
• a. History of appropriate response to dopaminergic medication, with at least a 30% improvement in motor UPDRS with L-DOPA by history or in-clinic testing, for the PD patients. OR
• b.Patients with tremor-dominant PD that do not respond to dopaminergic therapy and that exhibit a tremor score of at least 2 for tremor severity on at least one side of the body on the motor UPDRS examination.
• Unsatisfactory clinical response to maximal medical management (with trials of both higher and lower doses of drugs), including:
• For PD patients:
⁃ good benefit from dopaminergic medication but associated with insufficient duration of action or unacceptable side-effects OR
⁃ intractable disabling motor fluctuations (severe off periods, dyskinesias, or freezing spells) OR
⁃ For ET and dystonia:
⁃ intractable symptoms of ET or dystonia impacting at least 2 activities of daily living.
∙ Interested in being evaluated to undergo DBS, if indicated, to treat medically refractory movement disorder or
‣ Patients already implanted with DBS for continued management
• (Note: Inclusion criteria 4 and 5 can be met by historical report in patients who had DBS implanted outside the NIH)