Download - Neuromuscular Emergencies
Neuromuscular Emergencies
Hanni Bouma
Objectives
Discuss the approach to neuromuscular respiratory failure– Signs & Symptoms– Differential diagnosis– When to intubate
Brief overview of GBS & MG
Case 1
26 yo previously healthy F p/w tingling in feet since 1 week ago. Two days later noticed difficulty climbing stairs, followed by weakness of her arms. She is areflexic.
What percentage of patients with her disease develop resp failure?
Case 1
A) 5% B) 10 to 20% C) 25 to 50% D) 70 to 85%
Case 2
73 yo M adm w/ 3-month history of progressive proximal weakness
1 day after muscle biopsy, noticed on rounds to be breathless
What are we likely to see if this patient is in resp failure?
Case 2
A) Significant accessory muscle use B) Pt complaining that “I can’t breathe!” C) Shallow breaths; weak cough; neck flexor
weakness D) Able to count to 40 in a single breath
Case 3
63 yo M with limb-onset ALS since 2007 p/w choking sensation, difficulty “getting air in”
Not on home BiPAP No prior aspiration pneumonias; no fever, no
leukocytosis
Case 3
Case 3
Case 3
Which of the following are you LEAST likely to find in this patient?
A) FVC 1.1 L, MIP -20, MEP 30 B) MEP 40, MIP unable to complete C) pH 7.35, pCO2 60, bicarb 36 D) FVC 4 L, MIP -90, MEP 100
What can cause generalized weakness leading to resp failure?
Spinal cord lesion– Cervical cord compression, transverse myelitis
Motor neuron lesion– ALS
Peripheral nerve lesion– GBS, CIDP, critical illness polyneuropathy, Lyme disease, tick
paralysis, toxic NMJ disorder
– MG, LEMS, botulism, organophosphate poisoning Muscle lesion
– Polymyositis, dermatomyositis, critical illness myopathy, hyperthyroidism, congenital myopathy (muscular dystrophy), mitochrondrial myopathy
Mechanisms of NM resp failure
1) Upper airway obstruction:– Facial, oropharyngeal, laryngeal weakness mechanical
obstruction in supine position– impaired swallowing/secretion clearance aspiration
2) Inspiratory muscle weakness atelectasis V/Q mismatch hypoxia
3) Expiratory muscle weakness weak cough/poor secretion clearance aspiration & pneumonia
4) Acute complications PE, pneumonia, etc.
History
Time course?– progressive weakness over hours to days GBS– fluctuating weakness (on an hourly basis) present for
weeks/months MG Distribution of weakness?
– Proximal > distal (MG & GBS)– Ascending in GBS– Initally EOM/oropharyngeal muscle weakness, then
generalizes in botulism Sensory Sx.?
– Distal paresthesias common in GBS– No sensory inv’t in MG
History
Pain?– Low backache frequently in GBS; neck pain: C-spine
lesion? Antecedent illness?
– 60% of GBS triggered by viral URT illness or C.jejuni gastro– 40% of myasthenic crises triggered by infection
Medications Exposure to fertilizers & pesticides?
– Organophosphate poisoning Recent diet
– Botulism from home-canned goods
Exam: Signs of resp failure
Tachycardia Rapid, shallow breathing Stridor Weak cough, nasal voice, pooling of saliva (signs of bulbar
dysfunction) Orthopnea (abnormal if VC drops >10% supine) Staccato speech = the need to pause between words Abdominal paradox = diaphragm weakness weakness of neck & trapezius muscles (parallels diaphragm
weakness) Single-breath count: ask them to inhale fully & count from 1 to
50. If <25, sign of severe impairment of VC
Mehta, S. “Neuromuscular disease causing acute respiratory failure.” Respiratory Care, 2006. 51 (9): 1016-1023.
Focused exam
HEENT: – look for pooled secretions– Swallow test – Dysphonia (nasal voice from palatal paralysis)– Dysarthria
Lungs Diaphragm: observe/palpate for normal, outward
abdominal movement with inspiration Cough strength Count test (1 to 50)
Neuro exam
CNs– Pupils:
Reactivity may be lost in botulism or Miller-Fisher variant of GBS– EOM:
weakness + ptosis characteristic of MG, but also seen in MF, botulism, etc.– Face, palate, tongue, & neck strength
Motor exam: – Fasciculations? (ALS, organophosphate poisoning); tone; power (distribution of
weakness? Fatigable weakness?) Sensory:
– Distal sensory loss in GBS– Sensory level at C-spine level w/ quadriparesis = C-spine lesion
Coordination:– Ataxia in MF variant of GBS
Reflexes:– Areflexia in GBS; usually preserved reflexes in MG
Investigations
Bedside PFTs: “20/30/40 rule”– Vital capacity (max exhaled volume after full inspiration).
Normal = 60 ml/kg (4 L in 70 kg person). VC < 20 ml/kg (or 1 L) means intubation
– Max inspiratory pressure. Index of ability to avoid atelectasis. Normal = <-50 cm H2O. MIP >-30 means intubation
– Max expiratory pressure. Index of ability to cough/clear secretions. Normal >60 cm H2O. MEP <40 means intubation
– PFTs may be low if inadequate mouth closure from facial palsy, and may fluctuate in MG
Investigations
ABG: – Hypercarbia (PCO2 > 45 mmHg) =
hypoventilation
* PCO2 often normal or low until late in NM resp failure
– Hypoxia (PO2 < 75 mmHg) = V/Q mismatch usually atelectasis or pneumonia in this setting
Investigations
Basic labs (CBC, SMA-10, LFTs, CK) CXR EKG (electrolyte D/O; GBS ass’d w/
dysautonomia arrhythmias)
General care
Electrolytes: low potassium, high magnesium & low phosphate exacerbate muscle weakness
Serial PFTs (MIP/MEP/FVC) bid to qid Chest physio, suctioning & incentive spirometry DVT prophylaxis HOB elevation NPO if bulbar weakness; NG or Dobhoff feeding Bowel/bladder: paralysis predisposes to
constipation; GBS pts may have urinary retention
Determine:
– If resp failure is imminent– If ICU should be involved– What is the localization?
Predictors of need for MV
20/30/40 rule or a reduction in VC, MIP, MEP by >30% PO2 <70 mmHg on RA or PCO2 >50 mmHg w/ acidosis Dysarthria, dysphagia, impaired gag reflex In GBS:
– Time from onset to admission < 7 days– Inability to cough– Inability to stand– Inability to lift elbows or head– LFT increases– Presence of autonomic dysfxn
Sharshar T, Chevret S, Bourdain F, Raphael JC. Early predictors of mechanical ventilation in Guillain-Barre´ syndrome. Crit Care Med 2003;31(1):278–283.
Intubation: things to think about
Code status? Identify imminent resp failure early to avoid
emergency intubation– Minimizes atelectasis/pneumonia– Minimizes complications of intubation specific to GBS &
MG: Dysautonomia: can cause severe bradycardia, BP shifts,
profound hypotension w/ sedatives Denervated muscle: can cause fatal hyperkalemia with use of
succinylcholine Avoid depolarizing NM blockers Small doses of benzos
NPPV?
Few studies on its use in GBS & MG Inappropriate if upper airway function
severely impaired or hypercapnic resp failure
GBS
Most common cause of acute or subacute gen’d paralysis
Monophasic AIDP: autoimmune attack against surface antigens on peripheral nerves
Develops 5 days to 3 weeks after resp/GI infection in 60%
– Campylobacter jejuni (26%)– Viral URTI, influenza– EBV, CMV, VZV, HIV, hep A & B, coxsackie
Other precipitants: immunization, pregnancy, surgery, Hodgkin’s disease
Presentation
Sensory: – distal paresthesias/numbness (earliest Sx.)– Reduced vibration/proprioception
Motor:– Symmetric; evolves over days to 1-2 wks– Ascending: LE before UE; proximal> distal– May progress to involve trunk, intercostals, neck, bulbar, B/L FNs– Median duration from onset to max weakness 12 days
Reflexes: reduced, then absent Autonomic instability:
– Sinus tachy/brady, arrhythmias, labile BP (esp hypertension), urinary retention, anhydrosis
Other: low backache very common, myalgias
Investigations
EMG:– Reduced conduction velocities– Loss of F waves– Conduction block in motor nerves– Reduced motor amplitudes: 2° axonal damage worse Px.
CSF: – High protein (may be normal in first 2 days)– No cells or few lymphs
10% have 10-50 lymphs
Management
Admit for observation (potential for deterioration) Determine if resp failure imminent Dysautonomia: most frequently sustained HTN &
tachycardia– Esp in older pts w/ CAD, consider Labetolol– Hypotension in 10% fluids, pressors
PLEX (4-6 Rx. q1-2d) & IVIG (0.4g/kg/d x 5 d):– Equally effective– PLEX useful in first 2 weeks; benefit less clear after that– Steroids no proven benefit
Course
Progression over 1-4 weeks Plateau: 2-4 months Recovery: few wks to months Mortality 3-5% Poor prognosis:
– Resp failure requiring intubation – Advanced age– Very low distal motor amplitudes (axonal damage)– Rapidly progressive weakness over 1 week
MG
Ab-mediated attack on nicotinic Ach rec defective transmission across NMJ
Bimodal: F 20-30 yo; M 50-60 yo 2 autoimmune forms
– Ach receptor Ab+: 80% with generalized MG & 50% with ocular MG
– Anti-MuSK Ab+: 50% of patients who are Ach rec Ab negative; typically female with prominent bulbar weakness
Presentation
Motor:– Fluctuating, fatigable weakness involving eyes (90%),
face/neck/oropharynx (80%), limbs (60%)– Limbs rarely affected in isolation– Rest restores strength (at least partially)– Usually insidious onset
Sensory: normal Reflexes: preserved Thymic abnormalities:
– Malignant thymoma in 10-15% (more severe disease)– Thymic hyperplasia in 50-70%
Myasthenic Crisis
Defined by resp failure requiring ventilatory assistance
Occurs in 20-30%; mortality 5% Common precipitants:
– Infection in 40% (esp respiratory)– Pregnancy– Medications– Aspiration– Surgery– Emotional upset, hot environment
Drugs that exacerbate MG
Antibiotics:– Aminoglycosides (genta, tobra)– Fluroquinolones (cipro)– Macrolides (erythromycin, azithro, tetracycline, doxycycline)
Cardiac:– All beta-blockers– Calcium channel blockers– Class I anti-arrhythmics (quinidine, procainamide)
Anticonvulsants:– Phenytoin, CBZ
Antipsychotics, lithium Thyroid hormones Magnesium toxicity Iodinated contrast agents Muscle relaxants
– Baclofen– Long-acting benzos
**Too much anticholinesterase
Investigations
Ach receptor Abs Anti-MuSK Abs EMG:
– Repetitive nerve stimulation: >10% decrement in amplitude betw 1st & 5th CMAP
Sens/spec 90% when weak, proximal muscles stimulated; <50% sens in pts w/o limb weakness
– Single-fiber: “jitter” (variation in time interval betw firing of muscle fibers in same motor unit)
Sens >95% for MG but not specific Edrophonium (Tensilon) test:
– Not recommended in suspected crisis
Management
Determine if resp failure imminent Stop exacerbating meds Treat infection Symptomatic therapy (mild-moderate weakness):
– Cholinesterase inhibitors (Mestinon) Short-term disease suppression:
– To hasten clinical improvement in hospitalized pts w/ crisis or impending crisis; pre-operatively; chronic refractory disease
– PLEX: improvement w/i days, but lasts only 2-4 wks– IVIG: benefit may last up to 30 days– Comparable benefits
Long-term immunosuppression:– When weakness is inadequately controlled by Mestinon– Prednisone – Azathioprine (if steroid failure or excessive SE)
Key points
20/30/40 rule Identify pts at risk for resp failure EARLY to
avoid emergency intubation Don’t wait for pts to complain of SOB before
doing bedside PFTs