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 Florida @ Gainesville
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.
Common Indications for Botox Dystonia (ST, blepharospams,, focal and segmental) Hemi-facial spasms Spasticity Headache Wrinkles Drooling Gait freezing
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.
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
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)
Overflow Dystonia
Task Specific Dystonia
Sensory Trick
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)
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.)
Cervical Dystonia: idiopathic
Cervical dystonia: tardive
Cervical Dystonia: tardive
Blepharospasms
Apraxia of eyelid opening
Laryngeal Dystonia: adductor
Laryngeal Dystonia: abductor
Focal Limb Dystonia
Hemi-dystonia
Generalized Dystonia
Secondary Dystonia: Lubag
Secondary Dystonia: perinatal injury
Secondary Dystonia: Post Traumatic
Dystonia in CBGD
Dystonia in PD
Dystonia in PSP
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
Botox for the face: Blepharospasms x x x x x x x x x x x x
Botox for headache Frontalis Temporalis Insertion of the SCM Occipitalis Upper Trapezius
Botox for Hemifacial Spasms x x x x x x
Jaw dystonia Jaw closing dystonia Masseter Temporalis Jaw opening dystonia Lateral pterygoids Digastric
Cervical Dystonia: : Which muscles to inject? Torticollis (chin deviation) Laterocollis (tilt) Anterocollis (neck flexion) Retrocollis (neck extension) Shoulder elevation Head tremor
Torticollis Contralateral SCM Ipsilateral splenius capitis Ipsilateral semispinalis capitis
Laterocollis Ipsilateral scalenes Ipsilateral levator scapulae Ipsilateral splenius capitis Ipsilateral SCM
Shoulder elevation Ipsilateral levator scapulae Ipsilateral upper trapezius
Anterocollis Bilateral SCM Bilateral anterior scalene Bilateral submentalis
Retrocollis Bilateral splenius capitis Bilateral trapezius Bilateral semispinalis capitis
Head tremor Main vs compensatory muscles 3 to 4: 1 dosing ratio
Structures to avoid Brachial plexus Carotid sheath Greater occipital nerve Larynx and trachea Pharynx and esophagus Thyroid gland Pleura and apex of the lung
Post-injection expectations 70% get about 60-80% improvement 3-44 months duration Side effects Dysphagia Pain Dry mouth Fatigue Secondary non- reponsiveness
Let s s do some exercises!
Example 1
Example 2
Example 3
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
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
Thank you!