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Advances in the Treatment of Autoimmune Neuromuscular Disease

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Advances in the Treatment of Autoimmune Neuromuscular Disease From Diagnostic Challenges to Long-Term Management 16:00 17:40 SLIDE ADDED BY EDITOR ... – PowerPoint PPT presentation

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Title: Advances in the Treatment of Autoimmune Neuromuscular Disease


1
Advances in the Treatment of Autoimmune
Neuromuscular Disease From Diagnostic
Challenges to Long-Term Management
2
Faculty
Chairperson Gil I. Wolfe, MD Professor and Chief
of the Neuromuscular Section Department of
Neurology University of Texas Southwestern
Medical Center University of Texas Southwestern
Hospital Dallas, Texas
  • Erik R. Ensrud, MD
  • Board certified in PMR/EMG/Neurology/
  • Neuromuscular Disease
  • Director, Neuromuscular Center and EMG Laboratory
  • VA Boston Healthcare System
  • Associate Neurologist
  • Brigham and Womens Hospital
  • Boston, Massachusetts

Gregory T. Carter, MD, MS Clinical Professor of
Physical Medicine and Rehabilitation and of
Neuromuscular Medicine University of California
Davis Sacramento, California Medical
Director Regional Neuromuscular Center Providence
St. Peter Hospital Olympia, Washington
3
Topics
  • Diagnostic criteria for autoimmune neuromuscular
    diseases (NMD)
  • Making treatment decisionsusing experience,
    exploring the evidence
  • Role of rehabilitation in autoimmune NMD

4
Objectives
  • On completion of this activity, participants
    should be able to
  • Recognize the clinical presentations and key
    diagnostic features of autoimmune NMD
  • Select appropriate therapies and recognize
    potential treatment failures on the basis of
    patient history and available clinical evidence
  • Determine appropriate timing, dosage, and
    duration of therapies used in the treatment of
    autoimmune NMD
  • Recognize the long-term implications of NMD and
    become proficient in applying appropriate
    rehabilitative strategies

5
Barohn RJ, Saperstein DS. Semin
Neurol.19981849-61.
6
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  • Dyck at Mayo Clinic, 1975
  • Acquired neuropathy that shares a similar
    pathology and autoimmune etiology with GBS
  • Different from GBS
  • Usually, but not always, in the manner of
    presentation
  • Natural history of the disease
  • Rarely associated with antecedent infection
  • Responds to prednisone
  • Peak incidence at 4060 yrs old

Dyck PJ et al. Mayo Clin Proc. 197550621-637
Burns T, Ensrud E. (Producers). (17 Aug 2007).
Chronic inflammatory polyradiculoneuropathy with
Peter J. Dyck, MD Available at
http//beta.aanem.org/Education/Products/PhyPodcas
ts.aspx?page3fileid355.
8
  • Slow onset of progressive symmetric muscle
    weakness
  • Both proximal and distal weakness
  • Progresses for gt2 months may be
    relapsing-remitting
  • 60 slowly progressive course
  • 35 relapsing-remitting
  • Sensory loss less prominent than weakness
    paresthesias and numbness, but rarely significant
    pain
  • Large-fiber sensory modalities are more affected
  • Diffuse decreased or absent reflexes
  • 35 have evidence of CNS demyelination
  • Pathology
  • Multifocal demyelination
  • No vasculitic changes
  • Sural nerve biopsy is rarely necessary

9
Ensrud ER, Krivickas LS. Phys Med Rehabil Clin N
Am. 200112321-334.
10
Alberti MA et al. J Peripher Nerv Syst.
201116136-142.
11
AIDP
  • Prolonged distal motor and F-wave (early)
    latencies
  • Absent or impersistent F-waves
  • Slowing of CV and/or conduction block
  • Reduction of CMAPs /- temporal dispersion
  • Abnormal or absent median SNAP normal sural
    SNAP (AMNS) 39

Alberti MA et al. J Peripher Nerv Syst.
201116136-142.
12
Very Early EDX Findings in GBS (Within 4 Days of
Clinical Onset)
  • Abnormally late responses in 77
  • Prolonged distal motor latency in 55
  • Cranial nerve study abnormality in 44
  • Motor nerve conduction velocity slowing in 23

Alberti MA et al. J Peripher Nerv Syst.
201116136-142.
13
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14
Barohn RJ et al. Arch Neurol. 198946878-884.
15
  • Complex topic
  • Need to study multiple nerves, MS of PNS
  • Correct temperature (gt32.00C) is crucial
  • NCS shows electrophysiologic evidence of
    demyelination
  • Distal motor latency gt125 normal
  • Conduction velocity lt70 normal (lt80 if CMAP amp
    80 normal)
  • Conduction block
  • Temporal dispersion
  • Needle EMG tends to show more prominent
    reinnervation changes than acute denervation
    changes, consistent with slow temporal nature of
    pathology
  • EMG may look axonal because of secondary axonal
    damage

Barohn RJ, Saperstein DS. Semin Neurol
.19981849-61.
16
GBSEarly Recognition of Poor Prognosis
  • A recent paper looked at 397 GBS patients
  • Which patients were unable to walk at 4 weeks, 3
    months, and 6 months?
  • Three important factors
  • Older age (gt60)
  • Preceding diarrhea
  • MRC scores averaged 3 or less when the following
    were tested
  • Shoulder abd/elb flexion/wrist ext hip
    flexion/knee ext/ankle dorsiflexion
  • MRC scores were most predictive at 7 days after
    admission

Walgaard C et al. Neurology. 201176968-975.
17
CIDP With Acute Onset (A-CIDP)
  • 170 patients with GBS
  • 1-year follow-up
  • Treatmentrelated fluctuation (TRF) in GBS always
    occurred within 8 weeks of symptom onset
  • Always 1 or at the most 2 TRFs
  • Consider A-CIDP when a patient thought to have
    AIDP/GBS deteriorates 8 weeks from onset or has 3
    or more TRFs
  • Start maintenance therapy for CIDP when A-CIDP is
    recognized

Ruts L et al. Neurology. 2010741680-1696
Ensrud E. (Producer). (25 May 2010).
Distinguishing acute-onset CIDP from fluctuating
Guillain-Barré syndrome a prospective study
with Dr. Pieter van Doorn. Available at
http//www.aan.com/rss/?eventfeedchannel1.
18
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19
GBSRehabilitative Strategies
  • ICU setting
  • Passive ROM, prevent joint contracture
  • Active assisted ROM as tolerated
  • Close monitoring
  • Medical-surgical setting
  • Increase intervention
  • Isometric exercises while the patient is
    nonambulatory (Delorme technique)
  • Physical therapy

20
CIDP
  • Evidence-based treatments
  • Prednisone 60100 mg per day, followed by taper
  • IVIG 2 g/kg as induction therapy
  • Plasma exchange 56 treatments

Hughes RA et al. Cochrane Database Syst Rev.
2004(4)CD003280. Mehndiratta MM et al. Cochrane
Database Syst Rev. 2004(3)CD003906. van Schaik
IN et al. Cochrane Database Syst Rev.
2002(2)CD001797.
21
MGRoutine Diagnostic Work-up
  • Repetitive nerve testing (3 Hz)
  • U-shaped decrement
  • Reproducible
  • Partially repairable
  • Anti-acetylcholine receptor antibodies (IgG)
  • Antimuscle-specific tyrosine kinase (anti-MUSK)
    antibodies
  • Chest CT to look for thymoma

22
Autoimmune NMD Rehabilitative Strategies
  • MG patients who are adequately medicated may have
    minimal rehabilitative needs
  • During an acute crisis, passive limb exercises
    lower the risk of deep vein thrombosis (DVT)
  • DVT prophylaxis is recommended
  • Bulbar dysfunction increases the risk of
    aspiration

23
  • Anticholinesterase agents (pyridostigmine) for
    symptomatic relief
  • Corticosteroids
  • Immunosuppressants for steroid-sparing effect
    (azathioprine, mycophenolate mofetil,
    cyclosporine, tacrolimus, rituximab)
  • Plasma exchange or IVIG for a crisis before
    thymectomy for severe exacerbations or in
    refractory patients
  • IVIG is more accessible
  • Plasma exchange may work faster
  • Thymectomy
  • Removal of thymoma
  • Thymectomy is a treatment option in
    nonthymomatous MG1

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29
Adapted from Grob D et al. Muscle Nerve.
200837141.
30
MGSelection of SteroidSparing Agents
  • Azathioprine (may be used in conjunction with
    prednisone)
  • At least 1 year to see a full effect
  • Often seenan increase of at least 10 from
    baseline MCV in CBC in responders
  • Mycophenolate
  • Tacrolimus

31
GBSTreatment of Pain
  • Almost 90 of GBS patients experience pain
  • 66 have very severe pain
  • Gabapentin or pregabalin
  • Tricyclic antidepressants
  • Opioid analgesics

32
GBSBiPAP for Respiratory Support
  • Respiratory failure is a significant cause of
    morbidity and mortality in GBS
  • 33 of GBS patients require intubation
  • Average time to intubation is 7 days after
    symptom onset
  • Little clinical experience with BiPAP in GBS
    (used more commonly in MG)

33
GBSRehabilitative Approach to Pain Management
  • Topical agents (capsaicin, lidocaine gel)
  • Desensitization techniques (pressure garment)
  • Transcutaneous electrical nerve stimulation (TENS)

34
NMDRehabilitative Strategies
  • Isotonic exercise
  • Isokinetic exercise
  • Occupational therapyaddress activities of daily
    living (ADLs), functional concerns
  • Speech therapy if necessary
  • Assistive devices (walker, canes, etc)

35
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37
NMDExercise as a Precautionary Step
  • Watch for weakness as a sign of overwork
  • Exercise level should be submaximal
  • Higher repetition, lower weight
  • Endurance trainingwater exercise for uniform
    resistance
  • Monitor serum creatine kinase monitor for
    myoglobinurea watch for delayed-onset muscle
    soreness or pain

38
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40
Key PointsSummary
  • Autoimmune NMD
  • Diagnostic criteria
  • Treatment decisions
  • Role of rehabilitation
  • Diagnostic approachtypical presentations of GBS
    and CIDP
  • Acute presentation of CIDP with treatmentrelated
    fluctuations timing could lead to diagnosis of
    acute-onset CIDP
  • Diagnostic elements of MG long-term management
    and outcomes
  • Role of physical medicine and rehabilitative
    strategies in autoimmune NMD in the acute and
    chronic setting

41
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