Levator muscle − main muscle in the upper Ptosis – (pronounced \to−ses\, drooping or falling eyelid responsible for raising the eyelid of eyelid(s) that limits the ability to fully open the eye which can result in decreased vision Meige syndrome – a form of facial dystonia named after Henri Meige, in which Ptosis crutches − elevate the eyelid and allow blepharospasm is associated with involuntary the eye to be opened; mounted on the top inside movements of the mid and lower facial muscles; of eyeglasses to help hold the upper lid open symptoms include forceful contractions and thrusts in the mouth, jaw or tongue causing Procerus muscle − a small pyramidal slip of difficulty in opening and closing the mouth and muscle deep to the superior orbital nerve, artery can affect chewing and speech. It is now often and vein; its Latin meaning − tall or extended Medical called cranial dystonia. Punctal Plugs – also known as tear duct plugs or Movement disorder − neurological condition lacrimal plugs, these are small medical devices Glossary: that affects the ability to control muscle inserted into the tear duct (puncta) to treat dry movement; common disorders are Dystonia, eye. They block the duct and prevent drainage of Essential Tremor, and Parkinson's disease liquid from the eye; used to treat dry eye. Artificial A Reference for BEBRF Patients tears are usually still required Myectomy (limited or radical) – surgical procedure to remove some or most of the Residual functional capacity − the most you muscles that close the eyelids; sometimes can do despite physical or mental limitations that performed on patients for whom BoNT is not affect what you can do in a work setting effective; often BoNT injections are still needed Spasm – involuntary contraction of muscles that Orbicularis oculi muscle – the main muscle can be sudden that closes the eyelid; it is shaped like an ellipse encircling the whole eye Synapse – a junction between two nerve cells or between a nerve and muscle, consisting of a Oromandibular (OMD) − pertaining to the minute gap across which chemical signals mouth, jaw or tongue. (neurotransmitters) pass

Pathophysiology – the functional changes that Thalamus – a deep midline brain region that produce a particular syndrome or disease relays sensory and other information to higher Patho – Greek word meaning relating to levels of the brain (cerebrum) helping to control disease voluntary movements and higher mental Physiology branch of biology that deals functions with the normal functions of living organisms

Phenotype − the set of observable Benign Essential Blepharospasm Benign Essential Blepharospasm characteristics of an individual resulting from the Research Foundation Research Foundation interaction of its genotype with the environment For further information, contact:

Photophobia – extreme sensitivity to light; not Phone: 409-832-0788 the fear of light Fax: 409-832-0890 [email protected] Plasticity – the quality of being shaped or http://www.blepharospasm.org molded; the adaptability of an organism to changes Revised August 2019

Apraxia of eyelid opening − a neurologic Botulinum neurotoxin (BoNT) – a biologic EMLA cream – An anesthetic cream that can be condition characterized by difficulty keeping the nerve toxin derived from bacteria which when used to reduce the discomfort of BoNT eyes open because the muscles that open the injected directly into the muscles, temporarily injections; applied to the injection sites 45 eyes don’t work, not because of spasms of the weakens the muscle fibers so they remain minutes before the injections are to be muscles that close the eyes relaxed and no longer contract, thus preventing performed uncontrollable muscle spasms. Note: This is the Artane® (generic term, trihexyphenidyl) − An BEB “treatment of choice.” It is known to wear off Epiphora – Another word for “tearing” anti-movement drug taken by mouth that is used in about 3 months so injections need to be to treat some patients with Blepharospasm or repeated. Individual patients will spasm Essential − the isolated critical feature Meige syndrome; said to help about 30% of differently and therefore require different patients dosages at different intervals. Etiology – the cause, set of causes, or manner of causation of a disease or condition Artificial tears − lubricant eye drops used to Brow pin – a micro−screw device surgically treat the dryness and irritation associated with implanted to help hold open the eyelids Focal dystonia – a subtype of dystonia in which deficient tear production (dry eyes) a single body part is affected with contractions; Chemodenervation – used to describe BoNT with BEB it is in the eyelid muscles Basal ganglia − areas deep inside the brain that injected into overactive muscles to paralyze or are believed to play a major role in the weaken them FL−41 glasses − a special type of rose coordination of voluntary muscle movement colored lenses that filter certain wavelengths of Deep Brain Stimulation (DBS) − continuous, light and which might reduce symptoms of Benign − does not kill; is not fatal or malignant high−frequency electrical stimulation in the brain photophobia by means of an implanted electrode controlled Benign Essential Blepharospasm (BEB) – see by a battery just below the clavicle; these signals Functional blindness – although the term “Blepharospasm” block those from the brain that cause spasms "functional" has many meanings, in this context and tremors; most successful on larger muscles it means blindness that limits or prevents daily Blepharo − Greek word meaning eyelid functions or activities (example: driving) Dopamine − neurotransmitter chemical found in Blepharitis – an infection of the eyelid the brain and believed to play a role in many Genotype – the complete set of genes or dystonias genetic material present in a cell or organism Blepharospasm − involuntary forcible closure of the eye by the eyelid muscles Dry eye syndrome – an ocular surface Idiopathic − of unknown cause Primary blepharospasm – usually condition in which there is a decrease in the occurs quality or quantity of tears, resulting in drying out Hemifacial spasm – a neuromuscular disorder without the symptoms of any other neurological of the ocular surface causing discomfort, visual characterized by spasming seen on only one or metabolic disease; considered to be caused disturbance, secondary tearing, or a foreign side of the face; this is not a dystonia but rather by changes in the brain that have not yet been body sensation thought to be caused by compression of the identified; most common type of blepharospasm facial nerve; treatment sometimes similar to Secondary blepharospasm − Dystonia − a neurological movement disorder in BEB; surgery might relieve symptoms for some attributed to an outside factor such as physical which involuntary, sustained and repetitive patients trauma, exposure. to certain medications, or muscular contractions result in abnormal additional neurological or metabolic diseases; movements. Some frequent types: Klonopin® (generic, clonazepam) − An sometimes associated with brain lesions or blepharospasm (eyes), cranial (mouth & jaw), anti−movement drug taken by mouth that is used drugs cervical dystonia or torticollis (neck), spasmodic to treat some patients with blepharospasm or dysphonia (larynx), writer’s cramp (hand), and Meige syndrome. generalized (throughout the body)