2. • Acute neuromuscular Weakness - rapid onset ; maximum severity 4 wk
New onset acquired neuromuscular disease
Acute complications of preexisting chronic neuromuscular disease
Neuromuscular disease arising in ICU
3. Indications
for PICU
Admission:
Respiratory weakness and pulmonary infection;
Bulbar weakness and aspiration;
Cardiomyopathy, or cardiac dysrhythmia due to conductio
defects;
Dysautonomia
Acute rhabdomyolysis and renal failure
17. • Antibody mediated- 50%
• Antibodies associated: LM1, GM1, GM1b, GM2, GD1a,GalNAc-GD1a, GD1b, GD2,
GD3, GT1a, and GQ1b.
• Associated with specific type of disease
Subtype Antibodiee
AIDP GM1
AMAN, AMSAN GM1b, GD1a, GaINAc-GD1a
MFS, GBS and overlapping GQ1b, GT1a,GD3
18. Molecular mimicry and cross-reactivity
• C jejuni isolates lipo-oligosaccharides (LOS) that mimic
carbohydrates of gangliosides
• Antibodies cross-react, and recognize LOS as well as gangliosides or
ganglioside complexes
31. • Waveform amplitude usually correlates best with axonal integrity
• Loss of amplitude suggests axonal loss or dysfunction
• Conduction velocity depends highly on the degree of myelination
• Slowing of conduction velocity or latency prolongation usually
implies demyelination
• F waves allow testing of proximal segments of nerves
34. • The earliest findings in AIDP F-wave latencies or poor F-wave
repeatability due to demyelination of the nerve roots.
• This is followed by prolonged distal latencies (due to distal
demyelination) and temporal dispersion or conduction block.
• Slowing of nerve conduction velocities is less helpful as it tends to
appear late after 2-3 weeks.
35. Ambler Z. Guilain-Barré syndrome: an overview of current concepts. In Supplements to
Clinical neurophysiology 2000 Jan 1 (Vol. 53, pp. 388-395). Elsevier.
38. Indication of immunotherapy
• Severely affected patients, inability to walk unaided/disability scale ≥3
• Preferably within first 2 weeks from onset
39. Plasma Exchange
• North American PE study
• Confirmed by French PE trial
The Guillain-Barré Syndrome Study Group. Plasmapheresis and acute Guillain-Barré syndrome. Neurology 1985; 35: 1096–104
French Cooperative Group on Plasma Exchange in Guillain-Barré Syndrome. Efficiency of plasma exchange in Guillain-Barré syndrome:
role of replacement fluids. Ann Neurol 1987; 22: 753–61.
40. • 2 PE were significantly superior to none in mild GBS and 4 were superior to 2 in
moderate GBS; however 6 were not superior to 4 in severe GBS
• Continuous flow plasma exchange machines may be superior to intermittent flow
machines and albumin to fresh frozen plasma as the exchange fluid in GBS
requiring ventilation.
• Full muscle strength recovery at 1 year was greater with PE
Raphael JC, Chevret S, Hughes RAC, et al. Plasma exchange for Guillain-Barre syndrome. Cochrane
Database Syst Rev 2002;2:CD001798.
41. • PE after IVIg not advised
• PE wash out the IVIg previously administered.
42. IvIg
• PE compared with IVIG and combined
treatment of plasma exchange followed
by IVIG in 379 patients with severe GBS
PE and IVIG had equivalent efficacy
Combination of the two treatments was
not of significant advantage.
Randomised trial of plasma exchange, intravenous immunoglobulin, and combined treatments in
Guillain-Barré syndrome. Plasma Exchange/Sandoglobulin Guillain-Barré Syndrome Trial Group.
Lancet 1997;349:225-30
43. • IVIG -
Modulation of complement activation products
Neutralization of idiotypic antibodies,
Saturation of Fc receptors on macrophages
Suppression of inflammatory mediators such as cytokines,
chemokines and matrix metalloproteinase
44.
45. • January 2008 to April 2017
• 63 children (45.7%) received IVIg and 75 did not receive IVIg
• Fifty age and peak disability matched children with GBS did not receive IVIg controls.
46. • Primary outcome patients with complete recovery at 3
and 6 months
• Secondary outcome in-hospital death, duration of MV, and
hospital stay.
47.
48. • There were three in-hospital deaths; one in the IVIg and two in the control group (p = 0.56).
• The secondary outcome variables which included duration of MV (p = 0.47) and hospital stay (p =
0.09) were not different between IVIg and non-IVIg group
• AMAN had better recovery at 6 months on IV immunoglobulin (58.3% vs 11.1%; p = 0.03), but
not those with AIDP (58.3% vs 72.2%; p = 0.22).
49. • Theoretically, corticosteroids expected to reduce inflammation; lessen nerve damage in
inflammatory neuropathy
• No significant difference between the groups in mortality, intensive care unit stay, or improvement
in disability by the time of hospital discharge
• In both the large trials, hypertension was significantly less frequent in the steroid-treated than the
control group.
50.
51. Treatment
Related
Fluctuation
• 5–10% of patients with GBS deteriorate after
initial improvement or stabilization
• Common practice to give a second IVIg
course (2 g/kg in 2–5 days)
• Likely to improve after reinitiating treatment
• Prolonged immune response causing persistent nerve
damage
• Weaning IvIg Levels
• CIDP / acute onset A-CIDP
53. CSF Filtration-
Spinal catheter is placed, 30-50 ml of CSF withdrawn from subarachnoid space via filter
bypass into a 50-mL syringe installed on a specifically designed pump.
Bi-directional syringe pump (FLOFORS, Infors AG, Bottmingen, Switzerland) allowed
automatic flow- and pressure-controlled withdrawal and re-infusion of the CSF.
During re-infusion, the CSF was passed through a sterile filter system (CSF 1E, Pall
Medical, Dreieich, Germany) elimination of cells, bacteria, endotoxins,
immuneglobulins, and inflammatory mediators.
A filtration session comprised several cycles (usually five to six times 30 to 50 mL) and such
sessions were repeated on 5 to 15 consecutive days.
Average flow velocity - 2 to 3 mL/min.
56. • Other pharmacological treatments or combinations of treatments compared with no treatment, placebo
or another treatment.
• All trials randomised participants aged 16 years and older
57.
58. • All six RCTs were too small to exclude clinically important benefit or harm from the assessed
interventions. The certainty of the evidence
59.
60. • Significantly lower mean pain scores were found at the endpoint (day 7) in
the gabapentin phase
• Gabapentin group significantly lower median pain scores on all
treatment days in comparison to the placebo and carbamazepine groups
(P < 0.05)
• There were no adverse effects of gabapentin or carbamazepine reported,
other than sedation.
61. Autonomic
Dysfunction
Occurs in two-thirds of patients
• Cardiac Arrythmias
• Hypotension/ Hypertension
• Perspiration
• Abnormal response to drugs
Recovery of autonomic dysfunction parallels the
improvement of motor function
Lyu RK, Tang LM, Hsu WC, Chen ST, Chang HS, Wu YR (2002) A
longitudinal cardiovascular autonomic function study in mild Guillain-
Barré syndrome. Eur Neurol 47:79–84
62. • Potentially serious bradyarrhythmias - bradycardia to asystole found in 7–34 % of
patients.
• May also develop in less severely affected patients
• May require Cardiac pacing
63.
64. • Holter monitoring
• Half of patients showed AD
• HRV was significantly reduced in some time domains in those with AD
• AD was much more common in the AIDP subtype than the AMAN
subtype
• No association between AD and severity of disease
65. Management
of AD
• Hypertension- often no treatment
• Short acting – hydralazine, labetalol,
Nitruprusside
• Hypotension- Fluid, Norepinephrine,
phenylephrine
• Bradyrythmias- Pacing
• Adynamic ileus- Aviod opoids, can use
neostigmine, erythromycin
66. Respiratory Management
• When to consider Ventilation?
• When is ready for extubation?
• Long term ventilation/ Tracheostomy?
67. • Weakness of the diaphragm phrenic nerve demyelination.
• The respiratory pattern is restrictive,
• Vital capacity (VC) and Total lung capacity (TLC) are diminished
• Residual volume (RV) usually is normal or increased
• RV/TLC ratio is often high
68. • The decrease in inspiratory capacity caused by paralysis of the
abdominal and intercostal muscles
• Impaired ability to clear airway secretions by coughing atelectasis.
• Impaired swallowing caused by facial and oropharyngeal weakness
Aspiration pneumonia
69.
70. • 20/30/40 rule
• Vital capacity of ˜20 mL/kg,
• Maximum inspiratory pressure <-30 cm H2O,
• Maximum expiratory pressure of <40 cm H2O
• Values that fall by 50% from baseline or >30% in a 24-hour
period are of concern.
71. • Single-breath count - older children and adolescents to
assess severity and follow progression of weakness due to
acute NMD.
Can count up to 10 FVC 15-20 mL/kg.
Can count up to 25 FVC 30-40 mL/kg.
72. • Till SBC 7, all the ABG parameters are maintained.
• Admission SBC < 13 along with relative delta SBC of more than 20% can be used to monitor GBS
patients with respiratory involvement
73.
74.
75.
76.
77.
78.
79. time from onset to admission less than 7
days;
inability to lift head;
inability to lift elbows;
inability to stand;
ineffective cough;
elevated liver enzyme levels
80. • 552 patients, 150 requiring MV
• Predictors of prolonged MV -Muscle weakness
-Axonal degeneration or unexcitable
nerves on NCV
• Inability to lift the arms from the bed (bilateral Medical Research
Council [MRC] of deltoid muscles of 0–2) at 1 week after intubation have
an 87 % chance to require prolonged MV
81. On the day of extubation, lower negative inspiratory force (NIF) (−50.3
± 12.7 versus −28.6 ± 16.5 cm H2O, p = 0.0005) and higher VC (21.9 ±
8.4 versus 13.0 ± 5.9 mL/kg, p = 0.003) correlated with successful
extubation.
Change in VC preintubation to preextubation by greater than 4 mL/kg
correlated with 82% sensitivity and 90% positive predictive value for
successful extubation.
Failed extubations were associated with the presence of pulmonary
comorbidities (79 versus 36%, p = 0.008) and autonomic dysfunction
(73 versus 27%, p = 0.008).
82. ICU-AW
• Includes critical illness myopathy (CIM), critical illness
polyneuropathy (CIP), or a mixture
• Cause of failure of weaning from MV
• C/F- Symmetric weakness
Facial Sparing
Decreased DTR
83. MRC score
Upper limb Lower limb
Shoulder antepulsion Hip flexion
Elbow flexion Knee flexion and
Wrist extension) Ankle flexion
Total score – 60
Weakness < 48
85. Pathophysiology
• Inactivity loss of mechanical loading
• catabolic breakdown of muscle proteins
• The septic ICU patient increased proteosome proteolytic activity
• There is also evidence of a vasculopathy with marked endothelial
activation in both nerve and muscle
• Diaphragmatic atrophy- MV
87. • Reduced CMAP with normal MCV
• Spontaneous electrical activity on muscle
needle recording.
• SAP can be reduced axonal involvement.
• RNST normal
ENMG
88. • Muscle biopsy –
Myopathy with loss of thick myosin filaments with denervation
atrophy.
Necrosis
89. Conclusion
• Acute NM weakness- on admission or acquired later
• GBS- Characteristic History, physical examination, CSF
• Monitor patients for respiratory insufficiency
• IvIg - PE
• No role of corticosteroids
• In case of TRF with underlying sepsis, always consider ICU AW
The syndrome was first described
by French neurologists Guillain-Barre and Strohl in 1916
in two soldiers with acute areflexic paralysis followed by
recovery
Respiratory muscle involvement in GBS was possibly first observed by Landry in 1859, which was
recorded as acute ascending paralysis and risk of asphyxia [1
Symptoms generally have resolved by the time the patient presents with the neurological condition.
It was previously suggested
that enhancement solely of the anterior CE roots was indica
tive of GBS, but it is now known that both the anterior and
posterior roots may be simultaneously involved.
Hughes Motor Disability scale
Used to evaluate disability and functional end point
ikelihood of severe motor sequelae was less
Three potentially
interesting future treatments are in trial phase. They are
cerebrospinal fluid filtration, interferon‑β and two new
cyclooxygenase-two inhibitors.[33-35]
CIPNM manifests as early as the first 3 days of illness in some cases Examination shows weakness, muscle atrophy, and reduced or absent reflexes with sensory deficit being less prominent
has shown that strict glycemic control may significantly reduce the incidence of CIPNM in an adult, surgical intensive care unit population
he most frequent pattern on standard ENMG investigation is reduced compound muscle action potential with normal conduction velocity on motor nerve stimulation, and spontaneous electrical activity on muscle needle recording. This is observed in 70 to 100% ICU patients with sepsis or multiple organ failure after 5 to 7 of mechanical ventilation [9]. This pattern reflects either axonal or muscle involvement or both. Sensory action potential can be reduced, suggesting that at least part of the ENMG pattern is explained by an axonal involvemen
