Protocol Number: 03-N-0134

Title:

Neurophysiological Studies in Patients With Paroxysmal Hyperkinetic Movement Disorders

Number:

03-N-0134

Summary:

This study will use three neurophysiological tests (see below) to determine what areas of the brain are responsible for paroxysmal hyperkinetic movement disorders. Patients with these disorders have sudden, brief attacks of movement, similar to epileptic seizures, but without loss of consciousness.

Normal volunteers and patients with two subtypes of paroxysmal hyperkinetic movement disorder, paroxysmal dyskinesia and psychogenic variant, that can be induced by a specific trigger, such as a sudden movement or prolonged exercise, will be included in this study. Candidates must be 12 years of age or older. Women of childbearing potential will be screened with a pregnancy test.

Participants will undergo one or more of the procedures detailed below. Patients' test results will be compared with those of normal volunteers. Before each test, participants will provide a medical history and undergo a brief physical examination. During each procedure, the subject will have surface electromyography (EMG) to measure the electrical activity of muscles. For EMG, electrodes (metal discs) filled with a conductive gel are taped to the skin over the muscle to be evaluated.

Functional Magnetic Resonance Imaging (fMRI)

MRI uses a strong magnetic field, radio waves, and computer technology to provide detailed images of the brain. For this test, the subject lies in a narrow cylinder (the scanner), while pictures of the brain are taken. Earplugs are worn to muffle loud noises caused by electrical switching of radio frequency circuits used in the scanning process. For functional MRI (fMRI), the subject is asked to mimic a movement that occurs during an attack, such as stiffening the hand to make a fist or flexing and rotating the arm inward, to detect changes in the brain regions involved in the movement. During the procedure, involuntary movements and voluntary movements will be monitored by surface EMG and by video camera. The test will last about 1-1/2 hours.

Electroencephalography (EEG)

EEG measures the electrical activity of the brain (brain waves) with electrodes placed on the scalp. During the procedure, muscle activity will be recorded with EMG. The subject will first relax and then will be asked to mimic a movement attack. The test will last from 1-1/2 to 2 hours.

Startle Reflex

The subject will put on a headphone and hear loud noises in a random fashion. During the test, muscle activity will be recorded with EMG and with a video camera. The test will last about 1 hour.

Sponsoring Institute:

National Institute of Neurological Disorders and Stroke (NINDS)

Recruitment Detail

Type: Active Accrual Of New Subjects

Gender: Male & Female

Referral Letter Required: No

Population Exclusion(s): None

Eligibility Criteria:

INCLUSION CRITERIA:

Paroxysmal Dyskinesia patients:

A. Established diagnosis of paroxysmal hyperkinetic movement disorders. The diagnosis will be established by the preliminary screening in the NINDS Movement Disorders Outpatient Clinic, based on review of medical record, history, clinical evaluation, and videotapes of an attack.

B. Age 8 or older.

C. A reproducible trigger of paroxysmal hyperkinetic attacks, such as sudden movement, or prolonged exercise, which will produce attacks at least with 50% consistency.

D. Patients only with paroxysmal attacks of hyperkinetic movements.

E. Patients whose attacks can be precipitated easily.

F. Patients with typical attack involving unilateral extremities.

G. Patients taking medication that may influence the central nervous system, such as phenytoin, phenobarbital, carbamazepine, clonazepam, and antidepressants (but not limited to these) will be asked to hold the medication prior to the study. A sufficient drug washout period will be established dependant upon the individual drug. Subjects may be admitted to the NIH if necessary. They will be asked to abstain from alcohol and caffeine 24 hours prior to the study as well.

Psychogenic paroxysmal hyperkinetic movement attack patients:

A. Established diagnosis of psychogenic hyperkinetic movement disorders. The diagnosis will be established by the preliminary screening in the NINDS Movement Disorders Outpatient Clinic, based on review of medical record, history, clinical evaluation, and videotapes of an attack.

B. Patients with clear onset stereotyped and defined hyperkinetic attacks.

C. Patients only with paroxysmal attacks of hyperkinetic movements.

D. Age 8 or older.

E. A reproducible trigger of paroxysmal hyperkinetic attacks, such as sudden movement, startle or prolonged exercise, which will produce attacks at least with 50% consistency.

F. Patients whose attacks can be precipitated easily.

G. Patients with typical attack involving unilateral extremities.

H. Patients taking medication that may influence the central nervous system, such as phenytoin, phenobarbital, carbamazepine, clonazepam, and antidepressants (but not limited to these) will be asked to hold the medication prior to the study. A sufficient drug washout period will be established dependant upon the individual drug. Subjects may be admitted to the NIH if necessary. They will be asked to abstain from alcohol and caffeine 24 hours prior to the study as well.

EXCLUSION CRITERIA:

A. Age younger than 8 years old.

B. Patients with attacks that involve head and neck movements, axial movements, bilateral limb involvement, or violent attacks that typically make patients fall to the ground.

C. Previous history of or MRI findings consistent with brain tumors, strokes, trauma or arterial venous malformations.

D. Contraindication to MRI such as having devices not compatible with MRI (pacemaker, an implanted medical pump, brain stimulators, etc.), metallic prostheses in their body (metal pins and rods, heart valves, cochlear implants, etc.), and history of working with metals in the past, since such persons may potentially have small metal fragments in the eye without being aware of it.

E. Any diagnosis of progressive neurological disorders other than paroxysmal hyperkinetic movement disorder.

F. Any history of significant medical disorders requiring chronic treatment with other drugs that affects the CNS, which cannot be stopped.

G. Women who are pregnant or nursing.

H. Any subject who is not capable of giving an informed consent. This will be determined at the initial evaluation at NINDS clinic. Patients with Mini Mental Score less than 25 or significant psychiatric history will be further evaluated by detailed neuropsychiatric testing, or consultation with a psychiatrist.

Special Instructions: Currently Not Provided

Keywords:

Electroencephalogram

Functional (BOLD) MRI

Startle Reflex

Electromyogram

Psychogenic

Paroxysmal

Dyskinesia

Kinesiogenic

EEG

Movement-Related Potential

Recruitment Keywords:

Movement Disorder

Paroxysmal Hyperkinetic Movement

Disorder

Paroxysmal Dyskinesia

Conditions:

Movement Disorders

Investigational Drug(s):

None

Investigational Device(s):

None

Contacts:

Patient Recruitment and Public Liaison Office
Building 61
10 Cloister Court
Bethesda, Maryland 20892-4754
Toll Free: 1-800-411-1222
TTY: 301-594-9774 (local),1-866-411-1010 (toll free)
Fax: 301-480-9793

Electronic Mail:prpl@mail.cc.nih.gov

Citations:

Amjad AM, et al., An extended difference of coherence test for comparing and combining several independent coherence estimates: theory and application to the study of motor units and physiological tremor. J Neurosci Methods. 1997 Apr 25;73(1):69-79. PMID: 9130680

Andres FG, et al., Functional coupling of human cortical sensorimotor areas during bimanual skill acquisition.

Brain. 1999 May;122 ( Pt 5):855-70.

PMID: 10355671

Berardelli A, et al., The pathophysiology of primary dystonia.

Brain. 1998 Jul;121 ( Pt 7):1195-212. Review. PMID: 9679773

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