Robert Neel MD Associate Professor of Neurology University of Cincinnati Department of Neurology 6 levels of the PNS Motor Neuron Nerve Roots Plexus Nerve Neuromuscular Junction Muscle ID: 784775
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Slide1
Neuromuscular Emergencies
Robert Neel, M.D
.
Associate Professor of Neurology
University of Cincinnati
Department
of Neurology
Slide26 levels of the PNSMotor NeuronNerve Roots
Plexus
Nerve
Neuromuscular Junction
Muscle
Slide3Symptoms
Positive
Negative
Motor
Sensory
Slide4OrgAnizing symptoms by level of PNSPain, Motor, Sensory
Root
Plexus
Nerve
Motor, no pain, no sensory
Anterior Horn Cell
Neuromuscular Junction
Muscle
Slide5Danger Concepts in Neuromuscular DiseasePulmonaryAirwayCardiac Issues
Autonomic Dysfunction
Falls and Weakness
Other Systemic Considerations
Slide6Airway/pulmonary manifestations of neuromuscular diseasesVentilation vs OxygenationAirway Obstruction
Aspiration, Aspiration Pneumonia
Ventilatory
Failure
Early and terminal
respiratory
insufficiency
Restrictive defects
Interstitial Lung Disease
Pulmonary Embolism
Slide7Cardiovascular manifestations of neuromuscular diseasesCardiacArrhythmia: Bradycardias, Tachycardias, Blocks, Atrial
Systolic dysfunction
Diastolic
dysfunction
Cardiovascular
Blood Pressure
Slide8CaseA 23 year old woman has had intermittent diplopia and
ptosis
for the last
5-6 weeks
. In the last week, she has noted trouble chewing food and going up stairs.
She normally likes to run but has been unable in the last few weeks due to shortness of breath. Her dyspnea has been blamed on allergies. Her
general health is excellent.
Her general exam is normal. She has
moderate left
eye ptosis and
disconjugate
gaze in several directions. Her voice has a nasal quality. She has 4/5 strength in her proximal
muscles in arms and legs.
Otherwise her neurological exam is normal.
What do you do?
Slide9What is Myasthenia Gravis?
Acquired autoimmune disorder of the junction between nerve and muscle (NMJ)
Rare: Annual incidence is 2 per 1,000,000, but prevalence 1 per 10,000
Antibodies to nicotinic acetylcholine
receptors, EMG with RNS
Symptoms
1) Fluctuating weakness, fatigability
2) Ocular, bulbar, limb/neck
weakness
3)
Pulmonary and airway emergencies: Crisis
Course
1) Response to cholinergic drugs
2)
Immunosuppression: Corticosteroids, Steroid sparing agents, IVIG
3) Relapsing, remission, only 5% mortality
4) Thymus abnormalities-- CT Chest,
thymectomy
5)
Crisis: Need for Hospitalization, ICU
Slide10What is Myasthenic Crisis?Severe exacerbation of
myasthenic
symptoms with life-threatening bulbar symptoms
Respiratory
muscle weakness
Weakness of the
intercostal muscles
and the
diaphragm
may result in CO2 retention due to hypoventilation
Oxygenation vs. Ventilation
May cause a neuromuscular emergency
Weakness of
pharyngeal muscles
may collapse the upper airway
Airway closure: OSA like
process
Swallowing
Usually merits hospitalization
ICU
stay,
Intubation and Ventilation, Plasmapheresis or IVIG
Slide11Myasthenia: Pulmonary CarePulmonary
Frequent
respiratory therapist visits (q2-4 hours sometimes
), FVC and
Nif
Do
NOT rely on pulse oximetry or ABG
Arterial blood oxygenation may be normal while CO2 is
retained
Bedside counting test
Normal >
50-70
Impaired < 40
FVC criteria:
>20 cc/kg: Acceptable
15-20 cc/kg: ICU
10-15 cc/kg: Voluntary Intubation
<10 cc/kg: Intubation
Pulmonary
Secretions
SuctionAnticholinergics ?!?P & PD (Percussion and Postural Drainage)AspirationSwallowing evaluationNPO, NG tube
Head of bed with meals
Slide12How To Treat Myasthenic Crisis
Supportive: ABCs first
ICU vs
StepDown
vs Floor
Intubation and Ventilation
NPO, speech and swallow
Look for Offending Medications or Triggers
Quinolones, Aminoglycosides,
Chloroquines
, Statins
Hold
Mestinon
(or at least decrease)
Aggressive Rescue Therapy
Plasmapheresis
or IVIG
Slide13Treatment for Myasthenia
Prednisone
1
-2 mg/kg/ day
Decrease
WBCs that produce antibody
Caution: 30 hour rule and respiratory
Side effects:
Glaucoma, cataracts,
Cushingoid
, Weight gain
Hypertension, diabetes
Osteoporosis,
Avascular
necrosis
Depression, mania, insomnia
GERD,
PUD
Etc, etc, etc.
IVIG
Two regimens
0.4 grams/kg/day x 5 days1 gram/kg/day x 2 daysAdverse side effects:
Anaphylaxis, rashAseptic meningitis, HA
DVT, PE
Pulmonary edema
Contraindications:
IgA def
.
Relative: renal failure
Plasmapheresis
Typical protocol:
200-250 mL/kg for each of 4 or 5 QOD exchanges
1-2 volume exchanges
Nl
plasma volume is 3 L
Requires 2 large bore IVs or a dialysis catheter (preferred
)
Relative Contraindications
Cardiac:
CAD, arrhythmias
Autonomic instability
Hypoalbuminemia
Hypocalcemia
Slide14Case A 31 year old male physician developed progressive weakness of his legs over the last week. He went to see his primary care doctor because he had just been to China and had a nasty bout of diarrhea upon his return. In the last two days, however, he has noted that his arms are becoming increasingly “heavy”. He has no major medical problems and takes no medicines.
His general exam was normal as were his mental status and cranial nerve findings. Strength was
4/
5 in the legs both proximally
and distally and 5/5
in the arms. He was
1/1
at the ankles,
knees
were
2/
2
,
biceps 2/2. His toes were
downgoing
. There was a mild decrease in pin sensation to the ankles.
Slide15What is Guillain Barre Syndrome?
Acute Inflammatory Demyelinating
Polyradiculoneuropathy
(Guillain-Barre Syndrome)
Immune mediated segmental demyelination of peripheral nerve and root
Maximum deficit in 4-6
weeks
66% with Antecedent infection (URI, GI),
injection
Symptoms
1) Ascending paralysis.
Motor predominant symptoms, mild sensory
2)
Mild sensory,
back pain 50%
, autonomic instability
3) Cardiac, cardiovascular, pulmonary and airway emergencies (
respiratory
failure
30%)
Signs and
Diagnsotic Testing
1)
Hyporeflexia
and
areflexia
(decreased then absent)
2) CSF:
albuminocytologic
dissociation (
10% normal at 10 days
)
3) EMG with demyelinating
features
Treatment
IVIG or
Plasmapheresis
, respiratory support, cardiac monitoring
Floor vs Step Down vs ICU
Slide16Treatment for Guillain Barre
Plasmapheresis
Typical protocol:
200-250 mL/kg for each of 4 or 5 QOD exchanges
1-2 volume exchanges
Nl
plasma volume is 3 L
Requires 2 large bore IVs or a dialysis catheter (preferred)
IVIG
Two regimens
0.4 grams/kg/day
x
5 days
1 gram/kg/day
x
2 days
Adverse side effects:
Anaphylaxis, rash
Aseptic meningitis, HA
DVT, PE
Pulmonary edema
Contraindications: IgA def.Relative: renal failure
No steroids!Experimental studies on monoclonal antibodies
Slide17How To die with GBS?Cardiovascular Instability (Dysautonomia)
Cardiac Arrhythmia
Hypotension
Pulmonary Embolism
Respiratory Failure
Aspiration Pneumonia
Slide18GBS: Pulmonary
33
% develop
respiratory failure
muscle weakness (aka
ventilatory
failure)
some 2
aspiration
Usually within first 7 days
50% vent < 3
weeks
Respiratory
Therapy
Frequent respiratory therapist visits (q2-4 hours sometimes)
FVC and
Nif
q2-8 hours (bedside)
Indications for Intubation:
Vital capacity < 12 to 15 ml/kg: Esp. with rapid decline
Negative inspiratory force (NIF) < 25 cm H2O Hypoxemia: PaO2 < 80 mm Hg
Difficulty with secretions
Slide19GBS: CardiovascularTelemetry
Cardiac arrhythmias: Brady,
Tachy
, SVT
Beta Blockers
Propanolol
preferred
Labetalol
,
Esmolol
Calcium Channel Blockers
Amiodarone
Atropine and Transcutaneous Pacemaker
Blood pressure monitoring
Shorter acting agents are better
Hypotension
Neo-
synephrine
and
Levophed
Avoid
Dobutamine, Dopamine Hypertension:Preferred: labetalol, enalapril, nicardipine, esmololClonidine has REBOUND
Gabapentin???
Slide20Diseases to know that have high associations with emergenciesMotor Neuron DiseasesAmyotrophic Lateral SclerosisMuscle DiseasesMyotonic Dystrophy
Muscular Dystrophies and Limb Girdles
X Linked Dilated Cardiomyopathy and
Dystrophinopathies
Inflammatory Myositis (
Dermatomyositis
and
Polymyositis
)
Slide21Case Presentation20 year old African American male with 4 weeks of symptoms.Started with fatigue and worsening dyspnea. Still lifting weights in the basement, but developing more nausea.
3
weeks prior to symptom onset, patient had been
shovelling
snow from the blizzard
12/22/04.
Within 3 weeks of symptom onset, found in congestive heart failure with liver and kidney involvement.
Found to have EF 10%, BUN 77/Cr 2.8.
Physical Exam:
General: No rashes, no calf hypertrophy,
Tachycardic
with laterally displaced PMI
Neuro:
Normal except:
Neck flex 4/extend 4+, Deltoid 4/4,
Tricep
5/5, Bicep 4+/4+, Wrist Flex/extend 5/5, Iliopsoas 4/4, Hamstrings 4/4, Quadriceps 4/4, Plantarflexion 5/5, Dorsiflexion
4/4
Slide22Case PresentationLabs: CK 5000 HD#1, CK 392 HD#8, ESR 12, ANA (-)EMG: Normal NCSs, Needle exam with small amplitude polyphasic motor units without fibrillation in
vastus
, bicep, deltoid, and anterior
tibialis
Muscle Biopsy:
Slide23Cardiomyopathies in Muscular DystrophyHypertrophic (Diastolic)
Arrhythmogenic
Dilated (Systolic)
Restrictive**
Pulmonary HTN
from chest wall muscle weakness:
Cor
pulmonale
Scoliosis
Hypomobility
leads to low cardiac output
Primary:
Secondary:
Slide24Arrhythmogenic CardiomyopathyMyotonic DystrophyEmery-Dreifuss Muscular DystrophyBeckers/Duchenne Muscular Dystrophy
X linked Dilated Cardiomyopathy
Mitochondrial Disease
Slide25Treatment: PreventionEKG
Classic tall right
precordial
R wave
R/S ratio > 1
Brief PQ segment
Holter
Monitor
Cardiology Referral for
Electrophysiologic
Studies
Echocardiogram
Long term assisted
ventilation, NIV, Trach
Beta Blockers***
Coreg (carvedilol)
ACE inhibitor***
Aspirin
Calcium Channel
Blockers
Steroids
Animal modelsPrednisone in humansMilrinoneTransplant
Slide26CaseA 43 year old African American male with known elevated CPKs, under evaluation by rheumatology, presents to the ED with shortness of breath.
He has been a healthy manual laborer for the last 21 years. Notes that he has been having increased shortness of breath with exertion at work, worsening in the last 6 weeks. He has also had some
dysphagia
for pills and particulates.
You are consulted to help.
Slide27CaseTTE was normal. EF 55%, mild mild concentric LVH.
CT scan chest showed interstitial fibrosis.
Muscle biopsy showed
perifascicular
infiltrates and atrophy, c/w DM.
Pt was
intubated
within the hospitalization for
hypoxygenation
.
The patient died 2 weeks into the course of his hospitalization, despite starting
Cytoxan
.
Slide28SummaryDyspnea may be related to cardiac and pulmonary disease in neuromuscular processes
Restrictive
defects, CSA and OSA are the most common pulmonary conditions associated w/ neuromuscular
disease
Restrictive defects are caused by muscle
weakness
Treat with NIV, aggressive pulmonary toilet, cough assist techniques
Invasive ventilation available but needs to be a thoughtful
decision
Cardiac arrhythmia and diastolic/systolic dysfunction may be related to muscle disease
Slide29SourcesKissel, JT
Cornblath
, DR
Mendell
, JR. Guillain-Barre Syndrome. Diagnosis and Management of Peripheral Nerve Disorders. 2001: 145-172.
Asbury, AK. Diagnostic considerations in
Guillain
Barre
Syndrome. Annals of Neurology 9(
suppl
): 1-5, 1981.
Asbury, AK and
Cornblath
DR. Assessment of Current Diagnostic Criteria for
Guillain
Barre
Syndrome. Annals of Neurology 27(
suppl
): S21-24, 1990.
Barth, D. et al. Comparison of IVIg and PLEX in patients with myasthenia gravis. Neurology 2011;76;2017Van derMeche, FGA, Schmitz, PIM, The Dutch Guillain-Barre Study Group. A randomized trial comparing intravenous immunoglobulin and plasma exchange in Guillain Barre
syndrome. Muscle and Nerve 1992;16: 1267-1268.Plasma Exchange /
Sandoglobin
Guillain-Barre Syndrome Trial Group. Randomized trial of plasma exchange, intravenous immunoglobulin, and combined treatments in Guillain-Barre syndrome. Lancet 1997;349:225-230.
Qureshi, A.I. et al. Plasma exchange versus intravenous immunoglobulin treatment in
myasthenic
crisis. Neurology 52:629-632, 1999
.
Guillain-Barre Syndrome Steroid Trial Group. Double-blind trial of intravenous methylprednisolone in Guillain-Barre syndrome. Lancet 1993; 341:586-590.
The Guillain-Barre Syndrome Study Group.
Plasmapheresis
and acute Guillain-Barre syndrome. Neurology 1985; 35:1096-1104.
Slide30SourcesFrench Cooperative Group on Plasma Exchange in Guillain-Barre Syndrome: One year follow-up. Annals Neurology 1992;32:94-97.
Odaka
M,
Tatsumoto
M,
Hoshiyama
E, et al. Side effects of combined therapy of methylprednisolone and intravenous immunoglobulin in Guillain-Barre syndrome.
Eur
Neurol
. 2005;53(4):194-6.
eMedicine.com : Acute Inflammatory Demyelinating
Polyradiculoneuropathy
Hughes RA, Swan AV, van
Doorn
PA. Corticosteroids for
Guillain-Barré
syndrome.
Cochrane Database
Syst
Rev
. Feb 2010;2:CD001446.
Lawn, ND, Flecher, D, Henderson R, Wolter T, Wijdicks, E. Anticipating Mechanical Ventilation in Guillain-Barre Syndrome. Archives of Neurology, 2001. 58: 893-898Mehta, S. Neuromuscular Disease Causing Respiratory Failure. Respiratory Care, 2006. 51: 1016-1023Rabinstein
, A; and Wijdicks, E. Warning signs of imminent respiratory failure in neurological patients. Seminars in neurology, 23 (1), 97-103
Rieder
, P; Louis, M; Jolliet, P; Chevrolet, J. The repeated measurement of vital capacity is a poor predictor of the need for mechanical ventilation in myasthenia gravis
Slide31SourcesSharshar, T,
Chevret
, S,
Bourdain
, F,
Raphae
, J. Early predictors of mechanical ventilation in
Guillian-Barre
Syndrome. For the French Cooperative Group on Plasma Exchange in
Guilliain-Barre
Syndrome. Clinical Care Medicine, 2003. 31: 278-283
Roppolo
, LP et al. Airway
Management in Neurological
Emergencies.
Neurocritical
Care 1:405-414 (2004)
Pierson DJ. Indications for mechanical ventilation in adults
with acute
respiratory failure.
Respir Care 2002;47:249–262, discussion 262–265.Bourke, S. Respiratory involvement in neuromuscular disease. Clinical Medicine 2014 Vol 14, No 1: 72–5Morgan RK, McNally S, Alexander M, et al. Use of sniff nasal-inspiratory force to predict survival in amyotrophic lateral sclerosis. Am J
Respir Crit
Care
Med
2005;171:269–74
.
Lo Coco D, Marchese S, Pesco MC,
et al.
Noninvasive
positive-pressure
ventilation
in ALS: predictors of tolerance and survival.
Neurology
2006;67:761–5.
Bourke SC, Tomlinson M, Williams TL,
et al.
Effects of
non-invasive ventilation on survival and quality of life in patients with amyotrophic lateral sclerosis: a randomised controlled trial. Lancet Neurol 2006; 5:140–7.Eagle M, Baudouin SV, Chandler C, et al. Survival in Duchenne muscular dystrophy: improvements in life expectancy since 1967 and the impact of home nocturnal
ventilation.
Neuromuscul
Disord
2002;12:926–9
.
Slide32SourcesKimura, F et al. Wheelchair
economy class syndrome in amyotrophic lateral sclerosis. Kimura, F et al.
Neuromuscul
Disord
. 2006 Mar;16(3):204-7
.
Qureshi et al. Increased incidence of deep venous thrombosis in ALS.
Neurology
2007;68:76 –77
.
Hutchinson D, Whyte K. Neuromuscular disease
and respiratory
failure.
Pract
Neurol. 2008;8(4):
229–37.
Mehta
S. Neuromuscular disease causing acute
respiratory failure
.
Respir Care. 2006;51(9):1016–21. discussion 1021–3.Markham, L.W. et al. Pediatric Cardiology 2005.Silversides, CK. et al. American Journal of Cardiology 2003.Ernset, FC & Reed, AM. Idiopathic Inflammatory Myopathies: Current Trends
in Pathogenesis, Clinical Features, and Up-to-Date Treatment
Recommendations.
Mayo
Clin
Proc
. 2013;88(1):
83-105
Emergency
Neurology
.
Roos
, Karen L. (Ed
.) 2012: 211-234, 295-