Blepharospasm, Dystonia, Other Eye and Face Disorders
Conditions
Brief summary
The aim of this study is to develop new rating scales to help diagnose and measure the severity of blepharospasm. This is a condition involving a lot of blinking and spasms of eye closure that people can't control. This study will also test some video software to see if it can help diagnose people or tell the severity of disease using only a video recording of an exam. There is an additional plan to create an educational video to teach others the proper use of the scale and video software.
Detailed description
Blepharospasm is a chronic disorder characterized by too many contractions in the muscles around the eye and nearby facial muscles, leading to involuntary eye closure. This study involves a comprehensive evaluation for patients with blepharospasm, other eye and face disorders, and people without any neurologic or eye or face disorders. It addresses abnormal movements of the muscles around the eye, pain in those muscles, psychological accompaniments, and impact on regular daily life. The evaluation for each participant will be done on a single visit, and it may take up to 1.5 hours to complete. Each participant in this study will be asked to do the following: * Provide a copy of medical records and provide a medical history relating to the diagnosis of blepharospasm. * Have an examination by a neurologist or ophthalmologist to reveal the features and extent of dystonia or other eye and face disorders. The examination will be video recorded so it can be reviewed later by different experts. * Complete some questionnaires about quality of life and psychiatric well being The doctors will also complete various scales and questionnaires based on the participant's study visit.
Interventions
OTHERNo intervention.
Participants will complete questionnaires and interviews as well as have a neurological exam that will be video recorded. For this exam they will do simple movements (like looking at the camera, squeezing your eyes closed, moving your head, and writing).
Sponsors
Benign Essential Blepharospasm Research Foundation
Emory University
Study design
Observational model
CASE_CONTROL
Time perspective
PROSPECTIVE
Eligibility
Sex/Gender
ALL
Age
18 Years to No maximum
Healthy volunteers
Yes
Inclusion criteria
BLEPHAROSPASM (BL) GROUP Inclusion Criteria: * Diagnosed with Focal, Multifocal or Segmental Isolated Dystonia with Onset in Adulthood, must include blepharospasm. * Sufficiently symptomatic at recruitment to ensure that (eye) problems are evident to evaluators. * Has ability to provide informed consent and follow study directions.
Exclusion criteria
* Evidence of a secondary cause for dystonia as defined as blepharospasm caused by drug exposure, parkinsonism, or stroke. * Suspected psychogenic movement or eye disorders. * Has had surgical intervention for blepharospasm or eye problems that may confound interpretations, such as orbital myectomy, blepharoplasty, or deep brain stimulation surgery. * Is being treated with dopamine receptor antagonists. * Received BTX injection/botulinum toxin less than 10 weeks prior to study visit. * Taking any concomitant medications that haven't been at stable doses for at least 1 month prior to study enrollment. * Has significant medical or neurological conditions that preclude completing the video protocol and questionnaires. * Has significant physical or other condition that would confound diagnosis or evaluation. DISEASE CONTROL GROUP: Inclusion Criteria: * Diagnosed with a facial or eye disorder that can be confused with blepharospasm, including, but not limited to, hemifacial spasm, facial tics, psychogenic facial disorders, apraxia, and ptosis due to weakness. It is permissible for these problems to be part of Bell's palsy, myasthenia gravis, or Progressive Supranuclear Palsy (PSP). * Has no significant dystonia in any body part. * Sufficiently symptomatic at recruitment to ensure that (eye) problems are evident to evaluators. * Has ability to provide informed consent and follow study directions.
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| Blepharospasm Diagnostic Rating Scale (BDRS) | Day 1 | The ratings for the BDRS will be examined for internal consistency by pair-wise correlations among all items in the scale in all combinations of possible pairs. |
| Blepharospasm Severity Rating Scale (BSRS) | Day 1 | The ratings for the BSRS will be examined for internal consistency by pair-wise correlations among all items in the scale in all combinations of possible pairs. |
| Blepharospasm Screening Questions - Motor (BSQ-M) | Day 1 | — |
| Blepharospasm Screening Questions - Psych (BSQ-P) | Day 1 | Concurrent validity for the BSQ-P will be assessed using correlations between the responses to the questionnaire and data from the corresponding scales included in the concurrent validity packet (BDI-II, HADS, LSAS and PHQ-9). |
| Blepharospasm Disability Index (BSDI) | Day 1 | — |
Secondary
| Measure | Time frame | Description |
|---|---|---|
| Global Dystonia Rating Scale (GDRS) | Day 1 | — |
| Fahn-Marsden Dystonia Scale (FM) | Day 1 | — |
| Beck Depression Inventory II (BDI-II) | Day 1 | Concurrent validity for the BSQ-P will be assessed using correlations between the responses to the questionnaire and data from the corresponding scales included in the concurrent validity packet (BDI-II, HADS, LSAS and PHQ-9). |
| Craniocervical Dystonia Questionnaire (CDQ-24) | Day 1 | — |
| Hospital Anxiety and Depression Scale (HADS) | Day 1 | Concurrent validity for the BSQ-P will be assessed using correlations between the responses to the questionnaire and data from the corresponding scales included in the concurrent validity packet (BDI-II, HADS, LSAS and PHQ-9). |
| Liebowitz Social Anxiety Scale (LSAS) | Day 1 | Concurrent validity for the BSQ-P will be assessed using correlations between the responses to the questionnaire and data from the corresponding scales included in the concurrent validity packet (BDI-II, HADS, LSAS and PHQ-9). |
| Short Form Health Survey-36 Quality of Life Scale (SF-36) | Day 1 | — |
| Patient Health Questionnaire 9 (PHQ-9) | Day 1 | Concurrent validity for the BSQ-P will be assessed using correlations between the responses to the questionnaire and data from the corresponding scales included in the concurrent validity packet (BDI-II, HADS, LSAS and PHQ-9). |
| Eye Symptoms in Blepharospasm | Day 1 | — |
| Jankovic Rating Scale (JRS) | Day 1 | — |
| Oromandibular Dystonia Questionnaire (OMDQ-25) | Day 1 | — |
| Obsessive Compulsive Inventory-Revised Edition (OCI-R) | Day 1 | — |
Countries
Canada, Germany, Italy, United States
Outcome results
None listed