1. NOTE: To change the image on this slide, select the picture and delete it. Then click the Pictures icon in the placeholder to insert your own image. Amr Hasan, M.D. Associate Professor of Neurology Cairo University CairoNeuro Botulinum toxin in hemifacial spasm: A real “Face Off”

2. DR AMR HASAN AL HASANY 2

3. Agenda Hemifacial spasm 3 Anatomy of facial muscles Definition Aetiology Pathophysiology. Investigations Clinical features and differential diagnosis. Botulium toxin in hemifacial spasm Video presenations

4. DR AMR HASAN AL HASANY Agenda Hemifacial spasm 4 Anatomy of facial muscles Definition Aetiology Pathophysiology. Investigations Clinical features and differential diagnosis. Botulium toxin in hemifacial spasm Video presenations

5. DR AMR HASAN AL HASANY Anatomy of facial muscles 5

6. DR AMR HASAN AL HASANY Anatomy of facial muscles 6

7. DR AMR HASAN AL HASANY Anatomy of facial muscles 7

8. DR AMR HASAN AL HASANY Anatomy of facial muscles 8

9. DR AMR HASAN AL HASANY Anatomy of facial muscles 9

10. DR AMR HASAN AL HASANY Anatomy of facial muscles 10

11. DR AMR HASAN AL HASANY 11 Anatomy of facial muscles

12. DR AMR HASAN AL HASANY Anatomy of facial muscles 12

13. DR AMR HASAN AL HASANY Anatomy of facial muscles 13

14. DR AMR HASAN AL HASANY Anatomy of facial muscles (circumorbital and palpebral muscles) 14

15. DR AMR HASAN AL HASANY Anatomy of facial muscles (circumorbital and palpebral muscles) 15

16. DR AMR HASAN AL HASANY 16 Anatomy of facial muscles (circumorbital and palpebral muscles)

17. DR AMR HASAN AL HASANY 17 Anatomy of facial muscles (Nasal Muscles)

18. DR AMR HASAN AL HASANY 18 Anatomy of facial muscles (Nasal Muscles)

19. DR AMR HASAN AL HASANY 19 Anatomy of facial muscles (Nasal Muscles)

20. DR AMR HASAN AL HASANY 20 Anatomy of facial muscles (Buccolabial muscles)

21. DR AMR HASAN AL HASANY 21 Anatomy of facial muscles (Buccolabial muscles)

22. DR AMR HASAN AL HASANY 22 Anatomy of facial muscles (Buccolabial muscles)

23. DR AMR HASAN AL HASANY 23 Anatomy of facial muscles (Buccolabial muscles)

24. DR AMR HASAN AL HASANY 24 Anatomy of facial muscles (Buccolabial muscles)

25. DR AMR HASAN AL HASANY 25 Anatomy of facial muscles (Buccolabial muscles)

26. DR AMR HASAN AL HASANY 26 Anatomy of facial muscles

27. DR AMR HASAN AL HASANY QUIZ 27 14 15

28. DR AMR HASAN AL HASANY Agenda Hemifacial spasm 28 Anatomy of facial muscles Definition Aetiology Pathophysiology. Investigations Clinical features and differential diagnosis. Botulium toxin in hemifacial spasm Video presenations

29. DR AMR HASAN AL HASANY Agenda Hemifacial spasm 29 Anatomy of facial muscles Definition Aetiology Pathophysiology. Investigations Clinical features and differential diagnosis. Botulium toxin in hemifacial spasm Video presenations

30. DR AMR HASAN AL HASANY Hemifacial Spasm Characterized by: 30 Paroxysmal, involuntary clonic and tonic synchronous contraction of the muscles innervated by the facial nerve on one side. The spasms are due to brief burst of normal motor units firing at high frequency. Paroxysmal, involuntary clonic and tonic synchronous contraction of the muscles innervated by the facial nerve on one side. The spasms are due to brief burst of normal motor units firing at high frequency.

31. DR AMR HASAN AL HASANY Agenda Hemifacial spasm 31 Anatomy of facial muscles Definition Aetiology Pathophysiology. Investigations Clinical features and differential diagnosis. Botulium toxin in hemifacial spasm Video presenations

32. DR AMR HASAN AL HASANY Agenda Hemifacial spasm 32 Anatomy of facial muscles Definition Aetiology Pathophysiology. Investigations Clinical features and differential diagnosis. Botulium toxin in hemifacial spasm Video presenations

33. DR AMR HASAN AL HASANY Hemifacial Spasm Causes: 33 Vascular compression of the facial nerve by a dolichoectatic (a distorted, dilated, and elongated) vertebral artery or tortuous AICA. Facial nerve compression by a mass, brainstem lesions such as stroke or multiple sclerosis plaques. Vascular compression of the facial nerve by a dolichoectatic (a distorted, dilated, and elongated) vertebral artery or tortuous AICA. Facial nerve compression by a mass, brainstem lesions such as stroke or multiple sclerosis plaques.

34. DR AMR HASAN AL HASANY Hemifacial Spasm Causes: 34 Secondary causes such as trauma or Bell palsy. Familial Idiopathic Secondary causes such as trauma or Bell palsy. Familial Idiopathic

35. DR AMR HASAN AL HASANY 35 Hemifacial Spasm Causes:

36. DR AMR HASAN AL HASANY Agenda Hemifacial spasm 36 Anatomy of facial muscles Definition Aetiology Pathophysiology. Investigations Clinical features and differential diagnosis. Botulium toxin in hemifacial spasm Video presenations

37. DR AMR HASAN AL HASANY Agenda Hemifacial spasm 37 Anatomy of facial muscles Definition Aetiology Pathophysiology. Investigations Clinical features and differential diagnosis. Botulium toxin in hemifacial spasm Video presenations

38. DR AMR HASAN AL HASANY Hemifacial spasm 38

39. DR AMR HASAN AL HASANY Agenda Hemifacial spasm 39 Anatomy of facial muscles Definition Aetiology Pathophysiology. Investigations Clinical features and differential diagnosis. Botulium toxin in hemifacial spasm Video presenations

40. DR AMR HASAN AL HASANY Agenda Hemifacial spasm 40 Anatomy of facial muscles Definition Aetiology Pathophysiology. Investigations Clinical features and differential diagnosis. Botulium toxin in hemifacial spasm Video presenations

41. DR AMR HASAN AL HASANY Hemifacial spasm 41 Unilateral (rarely bilateral) spasm involving half of the facial muscles, typically lasting several minutes at a time. Affects women more than men. Onset fifth and sixth decade. Persists during sleep.

42. DR AMR HASAN AL HASANY Hemifacial spasm 42 Painless, no sensory loss. Involves entire side of face. HS most often begins insidiously in the orbicularis oculi muscle.

43. DR AMR HASAN AL HASANY Hemifacial spasm 43 Contractions gradually spread to other muscles of the face including the platysma

44. DR AMR HASAN AL HASANY Hemifacial spasm 44

45. DR AMR HASAN AL HASANY Hemifacial spasm Differential Dx: 45 Blepharospasm Meige’s syndrome (not to be confused with Meigs' syndrome,the triad of ascites,pleural effusion and benign ovarian tumor) Tourette’s syndrome (multiple compulsive muscle spasms associated with utterances of bizarre sounds or vile words). Trigeminal Neuralgia (acute episodes of pain in the distribution of the V CN) Eyelid Myokymia (eyelid twitches; caffeine & stress) Tardive Dyskinesia (Orofacial dyskinesia, associated with dystonic movements of the trunk and limbs, from long-term antipsychotic drug use) Eyelid apraxia

46. DR AMR HASAN AL HASANY Hemifacial spasm Blepharospasm: 46 Onset: usually in adult life (sixth & seventh decade) 3:1 female predominance Always Bilateral, episodic, involuntary contractions of the orbicularis oculi (therefore, may not involve other facial muscles). Disappears during sleep. Possible association with involuntary spasm of the lower facial musculature (orofacial dyskinesia or Meige’s Syndrome).

47. DR AMR HASAN AL HASANY Hemifacial spasm Blepharospasm: 47 Etiology: Adults: usually idiopathic (“essential blepharospasm”) related to dysfunction of the basal ganglia and limbic system. May occur in patients with: Parkinson’s disease Progressive supranuclear palsy Huntington’s disease Multiple Sclerosis Brainstem stroke May occur in patients with: Parkinson’s disease Progressive supranuclear palsy Huntington’s disease Multiple Sclerosis Brainstem stroke

48. DR AMR HASAN AL HASANY Agenda Hemifacial spasm 48 Anatomy of facial muscles Definition Aetiology Pathophysiology. Investigations Clinical features and differential diagnosis. Botulium toxin in hemifacial spasm Video presenations

49. DR AMR HASAN AL HASANY Agenda Hemifacial spasm 49 Anatomy of facial muscles Definition Aetiology Pathophysiology. Investigations Clinical features and differential diagnosis. Botulium toxin in hemifacial spasm Video presenations

50. DR AMR HASAN AL HASANY Hemifacial spasm 50 Work Up –MRI of cerebellopontine angle to R/O tumor Work Up –MRI of cerebellopontine angle to R/O tumor

51. DR AMR HASAN AL HASANY 51

52. DR AMR HASAN AL HASANY Agenda Hemifacial spasm 52 Anatomy of facial muscles Definition Aetiology Pathophysiology. Investigations Clinical features and differential diagnosis. Botulium toxin in hemifacial spasm Video presenations

53. DR AMR HASAN AL HASANY Agenda Hemifacial spasm 53 Anatomy of facial muscles Definition Aetiology Pathophysiology. Investigations Clinical features and differential diagnosis. Botulium toxin in hemifacial spasm Video presenations

54. DR AMR HASAN AL HASANY Hemifacial spasm Treatment 54 Pharmachologic: Carbomazepine, Baclofen, Clonazepam, Neurontin Botulinum toxin Surgery: Janetta procedure=posterior fossa craniotomy with insertion of inert material b/w vascular loop and VII nerve (to decrompress the nerve).

55. DR AMR HASAN AL HASANY Sites of injection 55

56. DR AMR HASAN AL HASANY Sites of injection 56

57. DR AMR HASAN AL HASANY 57 Sites of injection

58. DR AMR HASAN AL HASANY Sites of injection 58

59. DR AMR HASAN AL HASANY Sites of injection 59

60. DR AMR HASAN AL HASANY Sites of injection 60

61. DR AMR HASAN AL HASANY Sites of injection for BEB 61

62. DR AMR HASAN AL HASANY Sites of injection 62

63. DR AMR HASAN AL HASANY Ptosis (7-11%) Lagophthalmos (5-12%) Symptomatic dry eyes Diplopia (<1%) Ecchymosis Lower facial weakness Potential adverse effect 63 Facial assymetry.

64. DR AMR HASAN AL HASANY Agenda Hemifacial spasm 64 Anatomy of facial muscles Definition Aetiology Pathophysiology. Investigations Clinical features and differential diagnosis. Botulium toxin in hemifacial spasm Video presenations

65. DR AMR HASAN AL HASANY Agenda Hemifacial spasm 65 Anatomy of facial muscles Definition Aetiology Pathophysiology. Investigations Clinical features and differential diagnosis. Botulium toxin in hemifacial spasm Video presenations

66. DR AMR HASAN AL HASANY 66

67. DR AMR HASAN AL HASANY Case 1 32 ys old male. Unremarkable medical history. 3 years ago started to develop Rt HFS Increasing in frequency up to being continous all over the day MRI Brain : Normal 67

68. DR AMR HASAN AL HASANY 68 Case 1

69. DR AMR HASAN AL HASANY 69 Case 1

70. DR AMR HASAN AL HASANY Case 2 16 ys old girl. Student 4 years ago, she had Bell’s palsy Afterwhich, she started to develop Rt HFS Embarrasing and disfiguring 70

71. DR AMR HASAN AL HASANY 71 Case 2

72. DR AMR HASAN AL HASANY 72 Case 2

73. DR AMR HASAN AL HASANY Case 3 42 ys old Yemenese man. Employee 2 years ago, he started to develop bilateral facial dyskinsia (ocular, facial, orobuccal) Khat 73

74. DR AMR HASAN AL HASANY 74 Case 3

75. DR AMR HASAN AL HASANY 75 Case 3

76. DR AMR HASAN AL HASANY Pearls 76 Up to 50 units at one treatment. Injection should not be made inferior to the nasolabial fold! Injections here cause lip droop that in turns leads to very annoying lip biting by the patient. Avoid injecting the mid portion of the upper lid so as to avoid paralyzing the levator palpebri and causing ptosis. Avoid orbicularis oris Smaller dose for zygomaticus major Lower dose if secondary to facial palsy

77. DR AMR HASAN AL HASANY 77

78. DR AMR HASAN AL HASANY 78