1. Blepharospasm is a focal dystonia characterized by excessive eye blinking and involuntary eyelid spasms. 2. Botulinum toxin injection is the primary treatment and provides temporary relief of symptoms for up to a year by blocking acetylcholine release at the neuromuscular junction. 3. Untreated blepharospasm can decrease quality of life by impairing daily activities and potentially leading to functional blindness, anxiety, and depression.

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

16. References:
1. Hallett, M. (2002). Blepharospasm recent advances. Neurology, 59(9), 1306-1312.
2. Hallett, M., Evinger, C., Jankovic, J., & Stacy, M. (2008). Update on blepharospasm Report from the BEBRF International Workshop. Neurology,71(16), 1275-1282.
3. Horovitz, S. G., Ford, A., Najee-Ullah, M. A., Ostuni, J. L., & Hallett, M. (2012). Anatomical correlates of blepharospasm. Transl Neurodegener, 1(1), 12.
4. Defazio, G., Martino, D., Aniello, M. S., Masi, G., Abbruzzese, G., Lamberti, S., ... & Berardelli, A. (2006). A family study on primary blepharospasm.Journal of Neurology, Neurosurgery & Psychiatry, 77(2), 252-254.
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and spastic entropion: a multicentre study using two drug-dose escalation indexes. eye,24(4), 600-607.
9. Tsai, C. P., Chiu, M. C., Yen, D. J., Guo, Y. C., Yuan, C. L., & Lee, T. C. (2005). Quantitative assessment of efficacy of dysport (botulinum toxin type A) in the treatment of idiopathic blepharospasm and hemifacial
spasm. Acta Neurol Taiwan, 14(2), 61-68.
10. Truong, D. D., Gollomp, S. M., Jankovic, J., LeWitt, P. A., Marx, M., Hanschmann, A., ... & Xeomin US Blepharospasm Study Group. (2013). Sustained efficacy and safety of repeated incobotulinumtoxinA (Xeomin®)
injections in blepharospasm. Journal of Neural Transmission, 120(9), 1345-1353.
11. Kranz, G., Shamim, E. A., Lin, P. T., Kranz, G. S., & Hallett, M. (2010). Transcranial magnetic brain stimulation modulates blepharospasm A randomized controlled study. Neurology, 75(16), 1465-1471.
12. Kranz, G., Shamim, E. A., Lin, P. T., Kranz, G. S., Voller, B., & Hallett, M. (2009). Blepharospasm and the modulation of cortical excitability in primary and secondary motor areas. Neurology, 73(23), 2031-2036.

17. THANK YOU