Blepharospasm

"When your eyes can't stop blinking"

1. Blepharospasm “When your eyes can’t stop blinking” Ade Wijaya, MD August 2017

2. Outline Introduction Epidemiology and Genetics Risk factors Etiology Patophysiology Clinical manifestation Complication Rating scare Management - Botulinum toxin injection

3. Introduction Focal dystonia Excessive eyes blinking Benign essential blepharospasm vs secondary blepharospasm

4. Epidemiology & Genetics 12 / 1.000.000 in Japan – 133 / 1.000.000 in South Italy Female : Male = 2,3 : 1 Autosomal dominant; familial ?

5. Risk Factors predisposing Family history of dystonia and postural tremor History of head injury with loss of consciousness History of blepharitis or conjunctivitis Periorbital and dental procedure trauma Dry eyes Post-menopausal protective Smoking Coffee

6. Etiology Basal ganglia abnormality Increase grey matter at right somatosensory cortex Decrease grey matter at left primary motot cortex and right cingulate cortex

7. Pathophysiology  30 % dopamine at substansia nigra pars compacta  Trigeminal nerve circuit inhibition  Orbicularis oculi muscles weakness  Imbalance and compensation to increase blinking Eyes dryness  increase blinking

8. Clinical Manifestation Involuntary eyelids spasm Excessive blinking Usually bilateral Dry eyes Uncomfortable periorbital sensation Symptoms get better just after waking up Presipitating factors: bright light, chewing, driving, emotional stress, anxiety Sensory trick: yawning, whistling, singing, neck extention, using sunglasses Could be part of Meige Syndrome symptoms

9. Complication Impaired daily functional activities such as reading, writing, driving Decrease quality of life Functional blindness in 32 from 1.000.000 Anxiety & depression

10. Blepharospasm Jankovic Rating Scale

11. Management Botulinum Toxin For refractory cases: Deep Brain Stimulation Surgery In trial: Transcranial Magnetic Stimulation Not satisfactory: Medications: anticholinergics, benzodiazepines, bromocriptin, dopamine

12. Management Botulinum Toxin  blocking presynaptic cholinergic receptors thus, inhibiting acethylcholine release Type A Botulinum Toxin: Introduced by Scott at 1980, FDA approved for blepharospasm at 1989 Only short-term temporary effect (12 weeks – 1 year), long term use can reduce efficacy, probably due to antibody formation Dose: 30-200 unit / eyes, higher dose  higher efficacy but also higher side effects/complications

13. Management ( Botulinum toxin side effects/complications) local bruise ecchymosis ptosis keratopathy diplopia lagophtalmus dry eyes midfacial weakness nasopharyngitis respiratory tract infection visual disturbance dyspnea

14. Management

15. Summary Blepharospasm is a focal dystonia Main symptoms: excessive eye blinking, involuntary eyelids spasm Decrease QOL, and functional ability leading to depression and anxiety Drug of choice: botulinum toxin injection

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