Dystonias Peter McAllister, MD

Transcription

1 Dystonias Peter McAllister, MD Medical Director, New England Institute for Neurology and Headache Chief Medical Officer, New England Institute for Clinical Research Clinical Assistant Professor, Neurology, Yale University School of Medicine Assistant Professor, The Frank H. Netter School of Medicine at Quinnipiac University North Haven, CT

2 Disclosure Grant/research funding and served as a consultant for Allergan plc, Ipsen Biopharmaceuticals, Inc., and Merz. This lecture will discuss off label uses of the botulinum neurotoxins.

3 LIMB DYSTONIAS

4 Objectives Review case histories of patients with upper limb dystonia and spasticity treated successfully with Botulinum toxin injections Know strategies to evaluate the patient that requires Botulinum toxin Learn to decide which muscles to inject

5 Limb Dystonias Sustained or intermittent muscle contractions Cause twisting, often repetitive movements or both Abnormal postures, often patterned Often initiated by voluntary movements Worsened by voluntary movements Symptoms may overflow into adjacent muscles Albanesea A, et al. Mov Disord Jun 15;28(7):

6 Limb Dystonias Movement disorders can occur as primary (idiopathic) Genetic disease Neurodegenerative disorder Secondary to a number of neurological diseases Cerebrovascular disorders account for 22% of secondary movement disorders 1 Involuntary movements develop after 1-4% of strokes Albanesea A, et al. Mov Disord Jun 15;28(7):

7 Distribution of Dystonia Generalized (trunk, extremities, head/neck) Focal (single body part) Multifocal (2 or more non-contiguous body parts) Segmental (contiguous body parts) Hemidystonia (one side of body)

8 Writer s Cramp Insidious onset common range 20s to 50s mean age onset 38.4 years (Sheehy et al, 1988) Dystonic movements of fingers, wrist, forearm, or combination only with writing Finger flexion/extension, wrist deviation, arm pronation Sudden jerks of hand/arm unintended strokes tightening, tension, clasping the pen too tightly, twist away, pen flying away

9 Writer s Cramp Pain is uncommon Tension or discomfort in fingers, forearm, arm Tricks: pen between 2 nd -3 rd digits, thicker barrel, touching part of hand, writing with proximal muscles short hand writing on chalkboard May try to learn to write with non-dominant hand 1 in 4 develop similar dystonia in other hand

10 Writer s Cramp Majority are isolated idiopathic focal dystonia occasionally presenting feature in other widespread neurological disorders 1/3 have postural tremor Sheehy & Marsden: simple, progressive, dystonic types Gradual progression as one continues to write with the dominant hand of onset 5% with mirror movements

11 Writer s Cramp Spontaneous remissions are rare: more likely within first 5 years may last months to years recurrence is common Spread to other body regions is rare (if simple type)

12 Writer s Cramp - Flexion Steven Lo, MD

13 Typing Dystonia Steven Lo, MD

14 Limb Dystonias Movement disorders can occur as primary (idiopathic) Genetic disease Neurodegenerative disorder Secondary to a number of neurological diseases Cerebrovascular disorders account for 22% of secondary movement disorders 1 Involuntary movements develop after 1-4% of strokes Albanesea A, et al. Mov Disord Jun 15;28(7):

15 Case Presentation 31 y.o. male with H/O cerebrovascular accident as a teenager and resultant spastic right hemiplegia Has functioning (?) intrathecal baclofen (ITB) pump Dose-limited by side effects OnabotulinumtoxinA for right upper limb Gainfully employed as a janitor C/O toe excoriation of digit #3 and chronic painful walking therefrom

16 Day of Infection

17 Limb Dystonias Right Side: OnabotulinumtoxinA Extensor Digitorum Longus: 40 Flexor Digitorum Longus: 50 Flexor Digitorum Brevis: 50

18 12 Days Post Infection

19 Case Presentation 28 y.o. male presenting with post anoxic dystonia Severe in the left upper limb Dystonic tremor Chorea

20 Limb Dystonias Sentinel event was early in 2014 Apneic, unresponsive, cardiac arrest Resuscitated Developed involuntary movements several weeks later

21 Limb Dystonias Clonazepam (1.5 mg/day) Risperidone (worsened movements) Levodopa/Carbidopa, Cyclobenzaprine Baclofen (45 mg/day) Trihexyphenedyl (12 mg/day) OnabotulinumtoxinA 40 U superficial finger flexors and 40 U to the deep finger flexors NO RESPONSE

22 Prior to Injections

23 Prior to Injections VIDEOS: 1. Quiet sihng 2. Doff shirt

24 Prior to Injections

25 Limb Dystonias Left Side only: onabotulinumtoxina Pectoralis Major: 50 (clavicular portion) Biceps: ** Triceps: 50 (lateral) Flexor Digitorum Superficialis: 50** Extensor Carpi Radialis Brevis: 20 Extensor Carpi Radialis Longus: 30 Total injected (units): 400 units

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28 14 Days Post Injections VIDEOS: 1. Quiet sihng. 2. Doff + don shirt

29 Case Presentation 29 y.o. male sustained traumatic brain injury Blast injury from improvised explosive device Initially hemiparetic. Improved and was walking New onset symptoms on other side Viral encephalomyelopathy Tetraparetic Discharged to Hospice

30 Limb Dystonias Extended care facility x 4 years Has been getting neurotoxin injections in his upper limbs Presenting complaint: hands are tight Referred for baclofen pump implantation

31 Prior to Infections

32 Limb Dystonias Biceps: Levator Scapulae: 30 (cervical thoracic junction) Sternocleidomastoid: 40 (low) + 30 (high) + 30 (posterior, low) Trapezius Ridge: 40 (anterior) Cervical Trapezius: 30 Pectoralis Major: 50 Brachioradialis: 50 Total injected (units): 400 units.

33 4 Weeks After Infections

34 4 Weeks A(er Infec.ons

35 Persistent Right Upper Limb Tremor Reapplication to R. upper limb p 10 months Flexor carpi radialis 10 units Long head of biceps brachii 10 units

36 5 Months Later

37 Multiple Sclerosis

38 Multiple Sclerosis 71 yr. old woman w 20 yr. H/O MS Right hand dominant Severe ROM restriction at right elbow Lost ROM right elbow one year ago Frozen elbow after 3-week hospitalization R. hand works but is limited by elbow issue wheelchair user

39 Partial List of Problems Cannot cook or do bimanual activities Cannot perform ADLs Donning a blouse is difficult Restricted types of dress wear Emotional distress secondary to above

40 Physical Examination Right elbow fixed in fully flexed position Passive ROM < 20º from flexed position ¾ Modified Ashworth Scale at the elbow No forearm pronation or supination Full finger function and good wrist flex/ext Shoulder ROM to 100º of abduction

41 Major Goals Active function of right upper limb Improve ROM right elbow Improve all aspects of ADLs Cook breakfast

42 Treatment Botulinum Toxin Type A 200 Units Biceps brachii 50 U x 2 Brachioradialis 50 U, 30 U, and 20 U Only 2 MUAPs recorded in biceps w maximum voluntary effort or strong pull on flexed elbow (disuse/suppressed axons)

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44 6 WEEKS AFTER BOTULINUM TOXIN INJECTION

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47 12 Weeks After Injection Wears any style of clothing that she pleases Full interference pattern on EMG of right biceps

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49 Suggested Botulinum Toxin Dosing (Dose in Units; Typical Range) Flexor digitorum superficialis Flexor digitorum profundus Ona (Botox) Abo (Dysport) Inco (Xeomin) Rima (Myobloc) 20 (15-25) 100 (50-150) 20 (15-25) 1000 ( ) 30 (20-40) 100 (75-150) 30 (20-40) 1000 ( ) Flexor pollicis longus 20 (15-25) 50 (25-50) 20 (15-25) 1000 ( ) Opponens pollicis 5 (2.5-10) 12.5 ( ) 5 (2.5-10) 500 ( ) Adductor pollicis 10 (5-15) 25 ( ) 10 (5-15) 1000 ( ) Lumbricals 5 each (5-10) 12.5 each ( ) 5 each (5-10) per hand Dorsal Interosseus 5 each (5-10) 12.5 each ( ) 5 each (5-10) 500 each ( ) Extensor indicis proprius Extensor digitorum communis 5 ( ) 12.5 ( ) 5 ( ) 500 ( ) 15 (10-20) 50 (50-75) 15 (10-20) 1000 ( )

50 Ona (Botox) Abo (Dysport) Inco (Xeomin) Rima (Myobloc) Flexor carpi radialis 25 (20-40) 100 (50-150) 25 (20-40) 1000 ( ) Flexor carpi ulnaris 25 (20-40) 100 (75-150) 25 (20-40) 1000 ( ) Pronator teres 25 (20-40) 100 (50-100) 25 (20-40) 1000 ( ) Pronator quadratus 20 (15-25) 75 (50-100) 20 (15-25) 1000 ( ) Extensor carpi radialis Extensor carpi ulnaris Suggested Botulinum Toxin Dosing (Dose in Units; Typical Range) 20 (10-30) 75 (50-100) 20 (10-30) 1000 ( ) 15 (5-25) 50 (25-100) 15 (5-25) 1000 ( ) Biceps 100 (50-150) 250 ( ) 100 (50-150) 5000 ( ) Brachialis 25 (20-30) 250 ( ) 25 (20-30) 2000 ( ) Brachioradialis 50 (40-75) 100 (75-150) 50 (40-75) 2000 ( )

51 Suggested Botulinum Toxin Dosing (Dose in Units; Typical Range) Ona (Botox) Abo (Dysport) Inco (Xeomin) Rima (Myobloc) Quadriceps 100 (75-200) 1000 ( ) 100 (75-200) 5000 ( ) Adductor magnus 125 ( ) 1000 ( ) 125 ( ) 7500 ( ) Adductor longus 125 ( ) 1000 ( ) 125 ( ( ) Adductor brevis 125 ( ) 1000 ( ) 125 ( ) 7500 ( ) Hamstrings 100 (75-200) 1000 ( ) 100 (75-200) 7500 ( ) Gastrocnemius 75 (50-150) 500 ( ) 75 (50-150) 5000 ( ) Soleus 50 (25-75) 500 ( ) 50 (25-75) 5000 ( ) Tibialis posterior 150 ( ) 250 ( ) 150 ( ) 5000 ( ) Flexor digitorum brevis 75 (50-100) 200 ( ) 75 (50-100) 5000 ( ) Flexor hallucis longus 90 (50-100) 200 ( ) 90 (50-100) 2500 ( ) Extensor hallucis longus 40 (30-50) 150 (75-200) 40 (30-50) 2500 ( )

52 Recommended Maximum Dosing Per Session Recommended Maximum Dosing (Upper Extremity) Recommended Maximum Dosing (Lower Extremity) Recommended Maximum Dosing per session OnabotulinumtoxinA 400 U 400 U 600 U AbobotulinumtoxinA 1500 U 2000 U 2000 U IncobotulinumtoxinA 400 U 400 U 600 U RimabotulinumtoxinB U U U

53 Thank you