Neuromuscular

Disorders and Spinal

Cord Injury in the ICU:
Diagnosis, Management
and Treatment
DIANA GREENE-CHANDOS MD, FNCS
A S S O C I AT E P R O F E S S O R O F N E U R O L O G Y
P R O G R A M D I R E C TO R - N E U R O S C I E N C E S
CRITICAL CARE FELLOWSHIP
B L O C K C H A I R - D O C TO R I N G 2 A ; C O R E
FA C U LT Y- N E U R O S C I E N C E S
L E A R N I N G C O M M U N I T I E S ’ M E N TO R
E D U C AT I O N D I R E C TO R - C R I T I C A L C A R E
CENTER
 Disclosures
vI have nothing to disclose
Outline
v Acute Demyelinating Polyneuropathy v AcuteTraumatic Spinal
v Diagnostic Features Cord Injury
v Key Management Points in the ICU
v Treatment options
v Myasthenia Gravis
v Diagnostic Features
v Key Management Point in the ICU
v Treatment Options
v Critical Illness Neuropathy and
Critical Illness Myopathy
How can NM diseases become an
emergency?
v Neurogenic Respiratory Failure
v Unreliable Typical Clinical
Indicators of Respiratory Failure
v Dysautonomia in certain
diseases
v Some of the first intensivists
were Neurologists due to the polio
epidemic and their management of
the “iron lungs”.
Acute Inflammatory
Demyelinating
Polyneuropathy
Signs and Symptoms of AIDP/GBS
Presentation At Diagnosis
Symmetrical Weakness Starting in Legs 90% (10% start in arms/face)
Need for Ventilatory Support 10-30%
Face and Oropharyngeal Weakness 50%
Oculomotor Weakness 15%
Areflexia at Presentation 90% (100% in progressed disease)
Pain (due to nerve root inflammation) 2/3 of patients
Dysautonomia 70%
 Diagnosis of
AIDP/GBS
Required features include:
• Progressive Weakness of the LE and UE
(sometimes only in LE), ranging from minimal
weakness of the LE to complete paralysis of
all four limbs, trunk, bulbar and facial muscles
and external ophthalmoplegia.
• Areflexia or decreased reflexes in weak limbs
Supportive features include:
• Progression of sx over days to 4 wks (80% reach nadir in
2 wks)
• Relative Symmetry
• Mild Sensory symptoms or signs
• Cranial nerve involvement, especially bilateral facial nerve
weakness
• Recovery starting 2 to 4 wks after progression halts
• Autonomic dysfunction
• Pain
• No fever at Onset
• Elevated protein in CSF with a cell count <50/mm3
(usually <5mm3)
• Electrodiagnostic abnormalities consistent with GBS
 More AIDP/GBS Diagnostic Diamonds
The following features make the diagnosis of GBS doubtful.
The following features make the diagnosis of GBS doubtful.
• Sensory level (decrement or loss of sensation below a spinal cord root level from neuro
exam)
• Marked, persistent asymmetry of weakness
• Bowel and Bladder Dysfunction at Onset
• Severe and Persistent Bowel and Bladder dysfunction
• Severe pulmonary dysfunction with little or no limb weakness at onset
• Severe sensory signs with little or no weakness at onset
• Fever at onset
• CSF pleocytosis with a white cell count >50mm3
 Management of Issues by System in AIDP/GBS

Management •
System
Neurologic/Psychiatric
Complications
Neuropathic Pain
Prevention/Treatment
• Pain meds w/ antidepressant action: gabapentin/pregabalin,
of Issues by •• Depression
Anxiety •
SNRIs, TCAs
Avoid opioids (can worsen ileus)
System in •
•
Insomnia
Delirium
•
•
Sleep aids
Delirium precautions, including avoiding sedative drugs (ie
AIDP/GBS benzos)
Cardiac • Labile Blood Pressure • Continuous Cardiac Monitoring
• Cardiac Arrhythmias • Avoid Beta-blockers
• Tachy/ Brady • Atropine at bedside
• AV-blocks • Low-dose short acting drugs preferred (ie, nicardipine,
• Asystole clevidipine)
• Fluids for hypotensive episodes
• Low dose vasopressors, if needed
• Avoidance of vagal maneuvers
Pulmonary • Respiratory Failure • Avoid emergency intubations if possible; do elective
• Aspiration intubation using the 20/30/40 rule from bedside spirometry
• Pneumonia (minimum of once a day measurements).
• Ventilator-associated • Use of Non-Invasive Ventilation is not recommended
complications (PTX or VAP) • Avoid succinylcholine for induction for intubation
• Mucous plugging • HOB 30 degrees or more
• Need for tracheostomy • Aggressive pulmonary hygiene and chest physiotherapy
• Pulmonary Embolism • Adequate mobilization when possible
• DVT prophylaxis.
Management of Issues by System in AIDP/GBS
(cont).
System Complications
Gastrointestinal • Adynamic Ileus
• Gastroparesis
• Need for NG tube or
ultimately a PEG tube
• Stress Ulcers
Genitourinary • Urinary retention
• Urinary Incontinence
Endocrinology • Syndrome of inappropriate
secretion of antidiuretic
hormone (hyponatremia)
Musculoskeletal • Pressure Ulcers
• DVT
• Critical Illness
neuropathy/myopathy
Prevention/Treatment
• Aggressive bowel regiments
including use of daily
suppositories
• Avoid metoclopramide and
neostigmine if dysautonomia
• Minimize opioids
• Stress Ulcer Prophylaxis
• Intermittent or Continuous
Catheterization
• Fluid restriction
• Hypertonic Saline
• DVT prophylaxis
• Mobilization
• Early involvement of
PMR/PT/OT
IGOS GBS Prognosis Tool
vRisk of Respiratory Failure in the First Week
vDays between onset of weakness and hospital admission
vFacial or Bulbar Weakness at Admission
vMRC at hospital admission for proximal and distal musculature
groups in UE and LE bilaterally
 Who may need a tracheostomy?
v Age (inconsistent in studies)
v Pre-existing Pulmonary Disease
vInability to dorsiflex after completing iv ig or plasma exchange
vSignificant Deltoid Weakness
vAMAN or AMSAN types (if known)
vPF score which is the addition of:
vVital Capacity # + MEP # + MIP #
vCalculate ratio of day of intubation PF score/ day #12 PF score
v If PF ratio<1- pt will require vent > 3wks (70% sensitivity)
therefore consider trach
v If PF ratio>1- pt will likely be extubated before 3 weeks
Myasthenia
Gravis
85% Anti-Ach Ab
40% of Ach Ab
seronegative have
anti- Musk Ab
9% who have
neither above
antibodies will have
Lipoprotein Related
Protein-4 (LRP4) Abs
 Clinical Features of MG
• Hallmark- fatiguable muscle
weakness

• Head drop and bulbar

weaknessà MuSK Ab MG

• 50-80% w/ Ocular MG
develop systemic MG in 2
years

• 20% have a crisis in their first

year after dx
Myasthenic Crisis
v Bulbar Weakness and Respiratory distress
v Non-Invasive Ventilation (particularly BiPAP) decreases need for
intubation and length-of-stay in the ICU
v Consider Intubating those with:
v Increasing pCO2 despite BiPAP
v Significant bulbar weakness
v Decreasing paO2 despite maximum oxygen and/or BiPAP support
v Contraindications to BiPAP
v Failure of Extubation in Crisis is 25%
v Extubation to BiPAP is an option
v Pyridostigmine: continue with NIV but discontinue when intubated
to reduce secretions
Critical Illness Polyneuropathy (CIP) and
Myopathy (CIM)
 Mechanisms behind Critical Illness Polyneuropathy
Sepsis à impairment of
nerve microcirculation.
Cytokines released à
endoneural edema (worsened
by hyperglycemia and
hypoalbuminemia).

Edema increase O2 diffusing

distance SO….

Nutrients cannot be
transported into axon easily

The result is a dying back

axonopathy
Mechanisms behind
Critical Illness Myopathy
Inactivity leads to protein
degradation causing
* Decreased muscle
mass
* Muscle Atrophy

Decreased Muscle Force is

due to:
* Disruption of
Myofibrillar
Contraction
*Excitation-Contraction
Coupling
 Critical Illness Myopathy
Presentation Typically exposed to NM blocking
agent or corticosteroids in the
setting of critical illness
Physical Examination Flaccid symmetric atrophy and
weakness
Proximal > Distal
Cranial Nerves Spared
Normal Sensation
Reflexes reduced, but may be
preserved
Respiratory Muscle Weakness
Laboratory Investigations Creatine kinase level may be
increased
EMG Low motor amplitudes (<80%
lower limit of normal)
Preserved sensory response
Biopsy Myosin Filament Loss
Muscle Necrosis
Critical Illness Polyneuropathy
Critically ill (sepsis and multi-organ failure)
Flaccid symmetric atrophy and
weakness of limbs
Distal > Proximal
Lower > Upper limbs
Cranial Nerves Spared
Reduced Sensation
Reflexes reduced or absent
Respiratory muscle weakness
Creatine kinase level normal
Reduced or absent motor potential
Reduced or absent sensory
Neurogenic Motor Unit Potentials
Active Denervation
Normal Repetitive Nerve Stimulation
Axonal Neuropathy
Muscle Denervation
Risk Factors for Development of CIN/CIM
Prevention and Management Strategies
for Critical Illness Associated Weakness

AIDP/GBS
Factors associated with poor outcome:
• Age greater than 60 years
• Rapid progression to severe weakness (less
than 7 days)
• Need for Ventilator Support
• Mean Distal CMAP amplitude of less than
20%
• Preceding diarrheal illness
20% not able to walk w/o assistance after 6
months.
Most patients still have some pain and fatigue
after 6 months.
Overall mortality 2.4- 6.4%
Mechanically ventilated patients mortality 15-
30%
MORTALITY
Myasthenic Crisis
Mortality Improvement in past 50
yrs. from 30% to less than 5%
50% of MG related deaths occurs in
the first 3 years when the sx are
most severe
Thymectomy, Immunosuppressants
and improved specialty ICU care
improve prognosis
CIM/CIP
Development of either is assoc. w/:
• Increased ICU LOS
• Prolonged Mechanical Ventilation
• Increased Mortality
CIM is associated with better
outcomes than CIP
CIM has full recovery between 3-6
months
CIP or CIPNM has recovery between
6-12 months, some do not fully
recover
Overall 70.4% of patients have a
good recovery where they are able to
walk without assistance.
 Switching Gears:
Acute Traumatic Spinal Cord Injury
 Pathophysiology of SCI phases
v Primary phase of SCI (blunt trauma)
v shearing and laceration of spinal cord fibers
v contusion of spinal cord
v rarely full transection
v Axonal injury, disruption of blood vessels, and disruption of cellular membranes in this
phase
v Secondary phase of SCI
v Immediate phase - first 2 hours resulting in death of neurons and glia- assoc with
“spinal shock”, reversible reduction in sensory, motor and reflexes
v Acute phase- 2 hrs and 2 wks- increased inflammation, edema and hemorrhage,
release of free-radicals, excitotoxicity, then phagocytic response, then astrocytic
proliferation and scarring
v Intermediate/Chronic phase- after 2 wks and up to 6 months- continued maturation of
the astrocytic scar and beginning of astrocytic spouting. Chronic phase- myelomalacia
and cystic cavitations
 Acute Support and Imaging
vInjury rostral to C5 likely to require intubation and ventilator
support
v Multiple types of shock can be seen in SCI (given polytrauma) but
if hypotensive and bradycardic neurogenic shock is likely the cause
(d/t sympathetic disruption and unopposed parasympathetic tone
v Fluids followed by vasopressors for immediate circulatory
stabilization
v CT scan is the initial imaging- shows bony anatomy for defining
fractures, bony destruction
v MRI gives further understanding of injury- shows disc spaces,
ligaments, and if epidural or subdural blood is present.
AO spine guidelines
recommend early
spinal stabilization
when possible.
Although there is
potential for bias in
prior studies, early
(< 24 hrs from injury)
decompression may
result in improved
motor and functional
status of patients.
 Medical Management Pearls
vCorticosteroids still controversial-
vAANS/CNS guidelines 2013 do not recommend use for SCI
vAO spine guidelines 2017 state that high-dose methylprednisolone iv for 24 hours if started
before 8 hours from injury time (30mg/kg bolus followed by 5.4mg/kg/hr)
vBP management
v Hypotension (SBP <90 should be avoided)
v AANS/CNS guidelines (low level evidence but standard practice)- MAP greater than 85
for 7 days.
vConsortium for Spinal Cord Medicine recommends the use of norepinephrine and
dopamine for cervical and upper thoracic SCI
vCan also utilize invasive spinal cord monitoring for Spinal Perfusion Pressure (SPP)
with an intrathecal catheter- and concomitant drainage of CSF may help
vDVT prophylaxis
vProphylactic IVC filters do not reduce PE or death and not recommended
v NCS recommends initiation of DVT prophylaxis within 72 hours of injury
v Consortium of SCI recommends Enoxaparin
A Midwestern Story- Case
v You are a neurologist doing locums for a semi-rural town in the Midwest.
v You are called to the emergency room around 11pm to see a 61 y/o woman who came in a
few hours ago with abdominal pain, nausea and vomiting. She was given fluids and
ondasetron but started complaining of dizziness, blurry vision and is “so tired she can’t keep
her eyes open”. EM doc thinks she has bilateral ptosis.
v You find the patient moaning. She doesn’t want to talk much, but you hear that she started
having stomach pains and nausea around 7pm. She felt good all day, went to church, then to
a church picnic, then did some shopping. She feels dizzy lying on the bed and feels like her
vision is blurry, no diplopia. She feels weak all over.
v PMHx: HTN, Type II DM, hypercholesterolemia Meds: ASA, carvedilol, enalapril,
atorvastatin Soc Hx: married, no tobacco, rare EtOH, ROS: no fever, no diarrhea,
v T 36.9 C P 95 BP 167/93 . A and O times 4; speech dysarthric; language intact. CN-
Pupils 8mm reactive to 6mm; bilateral ptosis; EOMI; facial sensation normal, weak facial
muscles bilaterally in upper and lower face, decreased palatal movement. Motor exam-
decreased tone with 3/5 strength throughout. Reflexes 1+ bilaterally, absent ankle jerks,
Sensation intact, Coordination limited by strength- refused to walk.
A Midwestern Story- Case (cont)
v Labs, EKG, head and abdominal CT and normal
v Patient becomes more weak and begins drooling, also
appears to be using accessory muscles.
v The ED doc comes to you and says there is a 38 year man
with the same story but not as severe. Came in with
uncontrolled nausea and vomiting.
v What are you thinking???????
Outbreak….
 Food-Borne Botulism
vCaused by the toxin produced by anaerobic bacterium
Clostridium botulinum.
vUsually a history of an ingested food from improper canning
or purulent wound (especially in intravenous drug abusers).
vWeakness and anticholinergic symptoms start 6-24 hours
later
vRespiratory failure can develop rapidly
vToxin interferes with pre-synaptic release of Ach into the
NMJ
vAntitoxin can be given, but must be given within 24 hours of
the onset of symptoms
vDiaminopyridine which facilitates Ach release helps
symptomatically
vTreatment is supportive otherwise
Thank you!!!!