Hubert H. Fernandez, MD

Transcription

1 Hubert H. Fernandez, MD Associate Professor Co-Director, Movement Disorders Center Director, Clinical Trials for Movement Disorders Program Director, Neurology Residency and Movement Disorders Fellowship Training Program University of Gainesville

2 Disclosures Hubert H. Fernandez, MD has, over the past 5 years, been a paid consultant, paid speaker or performed clinical research under contract with: Amarin, Allergan, AstraZeneca, Aventis, Boehringer Ingelheim, Boston Life Sciences, Biogen Idec, Cephalon, Easai, Elan, Forest Laboratories, GlaxoSmithKlein, Huntington Study Group, Ipsen, Kyowa, Merck KgaA, Merz, MylanBertek, National Parkinson Foundation, Neurotrax, NIH/NINDS, Novartis, Parkinson Study Group, Solstice, Solvay, Teva, United Biosource Corporation, Valeant, and Vernalis; but has no owner interest in any pharmaceutical company.

3 Common Indications for Botox Dystonia (ST, blepharospams,, focal and segmental) Hemi-facial spasms Spasticity Headache Wrinkles Drooling Gait freezing

4 Dystonia.is a neurologic syndrome characterized by involuntary, sustained, patterned, and often repetitive muscle contractions of opposing muscles causing twisting movements or abnormal postures. Partly because of its rich expression and a variable course, dystonia is frequently not recognized or misdiagnosed Fahn 1984.

5 Main features of dystonia relatively long duration (unlike chorea or myoclonus) simultaneous contractions of agonist and antagonist muscles results in twisting of a body part same muscle groups are generally involved

6 Other Features Primary dystonia almost always begins by affecting a single part of the body (focal dystonia); most often the spread is to contiguous body parts The younger the age at onset, the more likely for dystonia to spread Action dystonia: dystonic movement aggravated during voluntary movements Task specific dystonia: abnormal movement appear during special action Overflow: as dystonia progresses, non-specific voluntary action can bring out dystonia, also actions in other parts of the body can induce dystonic movements of the primarily affected body part Worsen with fatigue, stress; suppressed with sleep, hypnosis, relaxation Sensory tricks (geste( antagoniste) Pain is not very common except in cervical dystonia Can present with tremor (dystonic tremor) or myoclonus (dystonia-myoclonus)

7 Overflow Dystonia

8 Task Specific Dystonia

9 Sensory Trick

10 Classification By Age at Onset Early onset (<26 years) Late onset (>26 years) By Distribution Focal - single body region (e.g., writer s s cramp, torticollis, blepharospasm) Segmental - contiguous body regions (e.g., face+jaw [Meige s], neck+arm) Multifocal - non-contiguous body regions (e.g., arm+leg, bleph+writer s cramp) Generalized - both legs + at least one other body region (one or both arms) By Cause Primary (idiopathic) - dystonia is only sign in the absence of identifiable exogenous cause or other inherited or degenerative disease Secondary (symptomatic) Dystonia-plus (accompanied by parkinsonism or myoclonus) Heredodegenerative dystonia (X-linked, autosomal dominant or recessive) Acquired/exogenous (drugs, trauma, stroke) Parkinsonian syndromes (PD, CBGD, PSP)

11 Focal Dystonias Cervical dystonia (CD) or spasmodic torticollis Blepharospasm Laryngeal dystonia or spasmodic dysphonia (SD) Limb dystonia (writer s s or musician s s cramp, etc.)

12 Cervical Dystonia: idiopathic

13 Cervical dystonia: tardive

14 Cervical Dystonia: tardive

15 Blepharospasms

16 Apraxia of eyelid opening

17 Laryngeal Dystonia: adductor

18 Laryngeal Dystonia: abductor

19 Focal Limb Dystonia

20 Hemi-dystonia

21 Generalized Dystonia

22 Secondary Dystonia: Lubag

23 Secondary Dystonia: perinatal injury

24 Secondary Dystonia: Post Traumatic

25 Dystonia in CBGD

26 Dystonia in PD

27 Dystonia in PSP

28 Therapy Pharmacotherapy CNS active drugs Anticholinergics GABAergics (benzodiazepines, baclofen, tizanidine) Dopaminergics (DA agonists, DA antagonists, DA depletors) Other (carbamazepine, alcohol, lithium, cannabinoids) Local injections (Botulinum toxin) Physical and supportive therapy PT and orthopedic devices (bracing) Repetitive transcranial magnetic stimulation Patient and caregiver education and support Surgical therapy Peripheral denervation Central Ablative procedures (thalamotomy, pallidotomy) DBS

29 Botox for the face: Blepharospasms x x x x x x x x x x x x

30 Botox for headache Frontalis Temporalis Insertion of the SCM Occipitalis Upper Trapezius

31 Botox for Hemifacial Spasms x x x x x x

32 Jaw dystonia Jaw closing dystonia Masseter Temporalis Jaw opening dystonia Lateral pterygoids Digastric

33 Cervical Dystonia: : Which muscles to inject? Torticollis (chin deviation) Laterocollis (tilt) Anterocollis (neck flexion) Retrocollis (neck extension) Shoulder elevation Head tremor

34 Torticollis Contralateral SCM Ipsilateral splenius capitis Ipsilateral semispinalis capitis

35 Laterocollis Ipsilateral scalenes Ipsilateral levator scapulae Ipsilateral splenius capitis Ipsilateral SCM

36 Shoulder elevation Ipsilateral levator scapulae Ipsilateral upper trapezius

37 Anterocollis Bilateral SCM Bilateral anterior scalene Bilateral submentalis

38 Retrocollis Bilateral splenius capitis Bilateral trapezius Bilateral semispinalis capitis

39 Head tremor Main vs compensatory muscles 3 to 4: 1 dosing ratio

40 Structures to avoid Brachial plexus Carotid sheath Greater occipital nerve Larynx and trachea Pharynx and esophagus Thyroid gland Pleura and apex of the lung

41 Post-injection expectations 70% get about 60-80% improvement 3-44 months duration Side effects Dysphagia Pain Dry mouth Fatigue Secondary non- reponsiveness

42 Let s s do some exercises!

43 Example 1

44 Example 2

45 Example 3

46 Dosing Increase Low weight Small muscle bulk Mild disease High probability of weakness Too much weakness from previous injection Decrease Heavy weight Large muscle bulk Severe disease Low concern for weakness

47 Materials Needed 30G x ½ needle 27G x 1¼ 1 needle 21G x 2 2 needle 1 cc syringe Lidocaine 4% topical cream Preservative free normal saline

48 Thank you!