1. Bell’s Palsy, Muscular Dystrophies -Erb’s Palsy Victor Politi, M.D., FACP Medical Director, SVCMC, School of Allied Health Professions, Physician Assistant Program

2. Facial Nerve 7th cranial nerve supplies all the muscles concerned with facial expression Small sensory component (the nervus intermedius of Wrisberg); conveys taste sensation from the anterior 2/3 of the tongue Motor nucleus of 7th nerve lies anterior and lateral to the abducens nucleus

3. Facial nerve A complete interruption of the facial nerve at the stylomastoid forearm paralyzes all muscles of facial expression –corner of mouth droops –crease and skin folds effaced –forehead un-furrowed –eyelids will not close

4. Facial Nerve Food collects between the teeth and lips, and saliva may dribble from the corner of the mouth The patient typically c/o a heaviness or numbness in the face

6. Facial palsy is usually unilateral and may be due to trauma, surgical intervention, tumor, stroke or infection of the 7th cranial nerve

7. The Most Common Form of Facial Paralysis is idiopathic -- Bell’s Palsy

8. Bell’s Palsy is a form with acute onset and unknown cause, possibly viral infection

9. Edema may play a part leading to compression of nerve fibers, with resulting acute unilateral paralysis of facial muscles

10. Bell’s palsy afflicts approximately 40,000 Americans each year

11. It disproportionately attacks pregnant women and people who have diabetes, influenza, a cold or some other upper respiratory ailment

12. The common cold sore virus, herpes simplex, and other herpes viruses are the likely cause of many cases of Bell’s palsy

13. In addition to one-sided facial paralysis with possible inability to close the eye, symptoms of Bell’s palsy may include pain, tearing, drooling, hypersensitivity to sound in the affected ear, and impairment of taste

15. The corner of the mouth droop, and there may be difficulty in speech, and eating

16. The symptoms usually disappear spontaneously but residual facial immobility and lip drooping may persist

17. Bell’s Palsy In general, the prognosis for Bell’s Palsy is very good With or without treatment most patients begin to get significantly better within 2 weeks

18. Bell’s Palsy For some, however, the symptoms may last longer In a few cases, the symptoms may never completely dissapear

19. Treatment Studies have shown that steroids are probably the most effective treatment Acyclovir combined with prednisone is possibly effective in improving facial function Other treatments are usually aimed at protection of the eye from drying during sleep

20. Treatment Analgesic for pain relief Corticosteroid drug to help reduce inflammation Massage of weakened muscles Splint to prevent drooping of lower part of face

21. Muscular dystrophy refers to a group of disorders that have little in common except for their name and the fact that they are inherited

22. Each type of muscular dystrophy has unique phenotypic and genetic features

23. Progressive Muscular Dystrophies Congenital Distal Scapuloperoneal

24. Progressive Muscular Dystrophies Duchenne Muscular Dystrophy (pseudohypertrophic) Becker’s (benign pseudohypertrophic) Myotonic Facioscapulohumeral Limb-Girdle (may include several disorders) Oculopharyngeal

25. Duchenne Muscular Dystrophy X-linked recessive Affects males almost exclusively Onset by age 5 Progressive weakness of girdle muscles Inability to walk after age 12 Kyphoscoliosis Respiratory failure - 2nd-3rd decade Other organ system involvement- cardiomyopathy/ mental impairment

27. Becker’s Muscular Dystrophy Less severe form of x-linked recessive dystrophy presentation similar to Duchenne’s except time course slower onset early to late childhood (not usually recognized until age 5) walking continues beyond age 15 - sometimes into 4th decade Calf muscle enlargement - prominent Death from complications similar to Duchenne’s may occur after age 40

28. Facioscapulohumeral Muscular Dystrophy Autosomal dominant disorder Slowly progressive disorder Affects males and females equally Extremely variable in severity Most common in 3rd or 4th decade, but may start at any age Cases starting earlier in life - worse prognosis

29. Facioscapulohumeral Muscular Dystrophy Some patients may remain asymptomatic throughout life Weakness of facial, shoulder girdle, proximal arm muscles and foot dorsiflexion weakness Other organ system involvement - HTN

30. Oculopharyngeal Muscular Dystrophy Autosomal dominant (French-Canadian or Hispanic background) Onset in 5th to 6th decade of life Slowly progressive weakness of extraocular, eyelid, face, and pharyngeal muscles Cricopharyngeal achalasia Other organ system involvement- cardiomyopathy

31. Scapuloperoneal Dystrophy Autosomal dominant Onset 3rd to 5th decade of life Progressive shoulder girdle and foot dorsiflex or weakness Other organ system involvement - cardiomyopathy

32. Limb-Girdle dystrophy Autosomal recessive inheritance Onset early childhood to adult slowly progressive weakness of shoulder and hip girdle muscles Other organ system involvement - cardiomyopathy

33. Limb-Girdle Dystrophy Proximal muscle weakness may begin in either the legs or the arms but usually progresses to all extremities Weakness may begin before age 5 or as late as the 3rd decade of life

34. Diagnosis Serum CK is elevated EMG pattern that of myopathy Muscle biopsy shows active myopathy but nonspecific

35. Physical therapy and orthopedic procedures may help to counteract deformities or contractures

36. Brachial Plexus Injuries A brachial plexus injury (Erb’s palsy) is a nerve injury The nerves that are damaged control muscles in the shoulder, arm, or hand and any or all of these muscles may be paralyzed

38. Brachial Plexus Injury The brachial plexus is a network of nerves, conducting signals from the spine to the arm and the hand Injury can occur at any time, most brachial plexus injuries happen during birth –about 1-2 babies in 1,000 suffer brachial plexus injury at birth, about 1 in 10 of these need treatment –treatment can consist of exercise, therapy, surgery or any combination thereof

40. Brachial Plexus Injuries Four types of nerve injury –Avulsion - the nerve is torn from the spine –Rupture- nerve torn, but not where it attaches to the spine –Neuroma- scar tissue forms around injury –Praxis-nerve damaged but not torn

41. Questions?????