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Electrodiagnostic Evaluation Of Critical Illness Neuropathy

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Electrodiagnostic Evaluation Of Critical Illness Neuropathy

Article Author:
Tova Plaut
Article Editor:
Lyn Weiss
Updated:
9/27/2020 9:39:23 AM
For CME on this topic:
Electrodiagnostic Evaluation Of Critical Illness Neuropathy CME
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Electrodiagnostic Evaluation Of Critical Illness Neuropathy

Introduction

As medicine advances, more critically ill patients are surviving longer ICU admissions. Patients with sepsis, multi-organ failure, severe burns, prolonged mechanical ventilation, and polypharmacy often develop profound neuromuscular weakness during their hospitalization despite appropriate medical interventions and treatments. Often referred to as “intensive care unit acquired weakness” (ICUAW) this underdiagnosed condition most commonly results from a spectrum of critical illness polyneuropathy (CIP), critical illness myopathy (CIM), or a combination of both. While CIP and CIM often occur simultaneously, it is important to understand the key features of each condition to ensure prompt recognition, prevent further disability, and ensure better patient outcomes.[1][2] While the conditions are often underdiagnosed, studies show that the incidence of ICUAW in patients with greater than seven days of mechanical ventilation may range from 25% to 83%. [3]

Critical illness neuropathy may be evident as a patient fails to wean off the ventilator or if the patient has profound weakness or numbness in extremities upon awakening from sedation. A physical exam may also reveal diminished deep tendon reflexes, possible muscle atrophy, or balance impairment upon leaving the ICU. Bulbar and extraocular muscles will often be spared in CIP.[4] Laboratory studies such as cerebrospinal fluid will usually be normal; this may help differentiate the condition from other illnesses with similar presentations, such as Guillain-Barre Syndrome.[5] Creatine phosphokinase (CPK) will be within normal limits in CIP and can become elevated in CIM. Systemic inflammatory response syndrome (SIRS) or sepsis is the most frequent underlying factor in CIP (as opposed to the use of neuromuscular blocking agents and steroids, which predominate in CIM).[6]

Although not often performed, the gold standard for the diagnosis of critical illness neuropathy remains electrodiagnostic testing, which includes nerve conduction studies and needle electromyography. It should be noted that while EMG is the optimal way to make a diagnosis of CIP, there are many challenges to performing a complete study in ICU conditions, including electrical interference, anasarca, hypothermia, peripheral edema, or limited patient participation in the exam. Nevertheless, in the evaluation of profound weakness in the ICU setting, electrodiagnostic testing is an essential tool that can direct the clinical team in determining further management. 

Pathophysiology of CIP/CIM

Etiology is not entirely clear, but it is though to be complex and multi-factorial in nature. Ultimately it lead to axonal dysfunction, ischemia, and degeneration necessitating aggressive treatment of underlying medical causes and physical neurorehabilitation. It is thought to be related to abnormal vascular, metabolic, and electrical processes leading to motor and/or sensory neuron abnormalities:

  • Vascular autoregulation-leading to increase microvascular permeability (e.g. dilation of vessels) which can result in edema, hypoxemia, pro-inflammatory invasion of leukocytes and cytokines, and occlusion within the muscle-neuron units
  • Metabolic-generation of reactive oxygen and nitric oxide species, failure of mitochondrial repair, neuronal hyperglycemic damage, and hypoalbuminemia
  • Electrical-disruption of sodium and calcium channels and loss of cellular excitability 

Summary of clinical and histopathological features of CIP/CIM

1. Critical Illness Polyneuropathy 

  • Predisposing factors: sepsis with concurrent encephalopathy, recurrent exposure to non-depolarizing neuromuscular blocking agents, and multi-organ failure
  • Presence of diffuse paraparesis and/or paraplegia, more distal than proximal with preserved muscle bulk or mild atrophy
  • Early loss of deep tendon reflexes or areflexia
  • Long-standing difficulty weaning mechanical ventilation after exclusion of cardiopulmonary etiologies
  • Electrodiagnostic evidence of axonal sensorimotor polyneuropathy

2. Critical Illness Myopathy

  • Predisposing factors: recurrent exposure to neuromuscular blocking agents, severe asthma, pneumonia, prolonged acidemia, acute respiratory distress syndrome, prolonged high dose corticosteroid use, prolonged use of aminoglycosides, sepsis, multi-organ failure, and organ transplantation
  • Presence of diffuse paraparesis and/or paraplegia, more proximal than distal with severe atrophy
  • Delayed loss of deep tendon reflexes
  • Long-standing difficulty weaning mechanical ventilation after exclusion of cardiopulmonary etiologies
  • Electrodiagnostic evidence of motor unit and recruitment abnormalities as well as an absence of excitability with muscle stimulation, with possible preservation of sensory potentials
  • Biopsy: Type II atrophic myofibers on H&E and myofiber necrosis

Indications

Both critical illness neuropathy and myopathy may clinically present with difficulty weaning off a ventilator or, if extubated, the patient may complain of profound weakness and flaccid limbs.[7] Additionally, patients with CIN may complain of sensory deficits, with numbness or paresthesias, especially in the distal extremities.[4] 

Weaning complications are due to the involvement of the phrenic nerve affecting the diaphragm, and other accessory respiratory muscles may also be affected.[1][10] The clinician must always keep in mind that Wallerian degeneration may take upwards of 10 to 14 days after the nerve injury to become apparent on an electrodiagnostic test.[8] Therefore, testing should be ordered judiciously only in the appropriate patients, and usually about two weeks after the onset of symptoms. 

Contraindications

Performing electrodiagnostic studies in critically ill patients have few absolute contraindications. Needle EMG is contraindicated in those with severe bleeding disorders. Needles should also never be inserted into areas of active soft tissue infection. Nerve conduction studies are contraindicated in patients with implanted cardiac defibrillators or if connected to external defibrillators. Patients should have screening for pacemakers, and electrical stimulation should not be performed directly on or near the device itself. 

Technique

Before performing any diagnostic study, a comprehensive review of the patient's history and clinical course, as well as a complete physical exam, must be performed. A thorough and compassionate diagnostician will inform the patient and family at the bedside of the indications and overview of the studies which will be performed. To accurately diagnose CIN with electrodiagnostic testing, one should ideally examine at least three extremities, performing both sensory and motor nerve conduction studies, as well as needle EMG testing in both proximal and distal muscles for comparison.

As with all nerve conduction studies, the temperature of the patient's limbs should be kept warm. Colder temperatures can surreptitiously increase amplitudes, prolong latencies, and slow conduction velocities.[9] To minimize the electrical interference which exists in the ICU, a notch filter should be used and, if possible, all unnecessary machines turned off, including unplugging the hospital bed.[5]

Complications

As with all electrodiagnostic studies in any setting and for any indication, the risk of complications is low. There is always a small risk of bleeding or introducing infection with needle studies.

Clinical Significance

Critical illness neuropathy is a primarily axonal sensorimotor peripheral neuropathy which will be apparent on electrodiagnostic studies. In both motor and nerve conduction studies, distal fibers will be more affected than proximal fibers. CIP and CIM are difficult to differentiate. Patients that are not comatose and can tolerate an EMG may be tested for MUAP recruitment or direct muscle stimulation. A purely sensory phenomenon, although rare, can help distinguish between CIP and CIM. On muscle biopsy, the myosin/actin ratio can be used to diagnose CIM in conjunction with histopathological findings such as myonecrosis, loss of thick myosin filaments, non-necrotizing myopathy, and atrophy.

A. Nerve conduction studies

  1. Sensory Nerve Conduction Studies

There is a reduction of conduction amplitude and absent sensory nerve conduction studies (SNAPs)[10]. Early in the course, conduction velocities and latency are often normal or slightly decreased due to the loss of the fastest fibers, Decreases in amplitude will be more profound than the slowing of conduction velocities and prolongation of latency. 

  2. Motor Nerve Conduction Studies

There is a reduction of conduction amplitude and absence of compound muscle action potentials (CMAPs)-[10][11]. Early in the course, as with the sensory studies, will have a decrease in amplitude more profound than any slight decrease in conduction velocity or prolongation of distal latency. The reduction is usually two standard deviations of the normal value.[12]

B. Needle Electromyography

Abnormal spontaneous potentials may be present in distal muscles, including positive sharp waves (PSWs) and fibrillation potentials (fibs). PSWs appear with an initial sharp downward deflection with a subsequent upward (negative) phase with a duration of 10 to 30 milliseconds. PSWs often sound like "dull thuds." Fibrillation potentials can have triphasic or biphasic spikes with a duration of 1 to 5 milliseconds and sound like "rain on a tin rooftop." These spontaneous potentials indicate that there is significant denervation of the muscles tested. Individual motor units may show decreased recruitment and an increase in the frequency of firing. In chronic conditions, motor unit action potentials (MUAPs) may be large and polyphasic.[10] Although it is technically challenging in the ICU, a needle EMG of the diaphragm muscle may reveal abnormal spontaneous potentials as well.[11] 

C. Summary of electrodiagnostic criteria

    1. Critical Illness Polyneuropathy 

  • Reduced amplitudes on CMAP
  • Reduced amplitude on SNAP, with rare exceptions (e.g. pure form of motor CIP has preserved SNAPs)
  • Normal velocity and normal latency.
  • Normal action potential excitability during direct muscle stimulation
  • Fibrillation potentials and positive sharp waves
  • Decreased motor unit recruitment upon voluntary muscle contraction
  • Large, polyphasic MUAP spontaneous potentials

    2. Critical Illness Myopathy

  • Preserved sensory response (>80% of LLN)
  • Reduced motor responses (compound muscle action potential <80% LLN)
  • Normal repetitive nerve stimulation
  • Small, short-duration, polyphasic MUAP with early full or normal recruitment or interference pattern; in later stages, recruitment may be affected
  • Reduced or absent action potential excitability during direct muscle stimulation
  • Fibrillation potentials and positive sharp waves
  • Decreased motor unit recruitment upon voluntary muscle contraction

 

Enhancing Healthcare Team Outcomes

Often patients with a prolonged ICU admission will develop profound weakness or failure to wean off a ventilator. Intensivists, physiatrists, and neurologists must recognize that further evaluation with electrodiagnostic testing is warranted in these circumstances. As healthcare professionals, appropriate disease recognition and diagnosis of critical illness neuropathy will help expedite patient recovery and can improve long term quality of life.

While there is a vast differential in the patient with weakness which develops in the ICU, NCS and EMG studies may be able to identify the etiology and further guide the management. Once the diagnosis of CIN is made, the interprofessional team must include an interprofessional team of physicians, physical therapists, occupational therapists, speech therapists, nutritionists, social workers, and case managers. These professionals can work together to coordinate early mobilization and aggressive multifaceted rehabilitation.

Typically, there is a long and challenging recovery ahead for patients with CIN upon leaving the critical care setting. Only with a coordinated effort between the various medical disciplines and departments will the best outcomes for patients be achieved.[2][13]


References

[1] Symeonidou Z,Theodoraki K,Chalkias A,Argyra E,Casale R, Critical Illness Polyneuropathy (CIP): a multicenter study on functional outcome. Giornale italiano di medicina del lavoro ed ergonomia. 2019 Mar;     [PubMed PMID: 30946550]
[2] Lorin S,Nierman DM, Critical illness neuromuscular abnormalities. Critical care clinics. 2002 Jul;     [PubMed PMID: 12140913]
[3] De Jonghe B,Sharshar T,Lefaucheur JP,Authier FJ,Durand-Zaleski I,Boussarsar M,Cerf C,Renaud E,Mesrati F,Carlet J,Raphaël JC,Outin H,Bastuji-Garin S, Paresis acquired in the intensive care unit: a prospective multicenter study. JAMA. 2002 Dec 11;     [PubMed PMID: 12472328]
[4] Friedrich O,Reid MB,Van den Berghe G,Vanhorebeek I,Hermans G,Rich MM,Larsson L, The Sick and the Weak: Neuropathies/Myopathies in the Critically Ill. Physiological reviews. 2015 Jul;     [PubMed PMID: 26133937]
[5] Shepherd S,Batra A,Lerner DP, Review of Critical Illness Myopathy and Neuropathy. The Neurohospitalist. 2017 Jan;     [PubMed PMID: 28042370]
[6] Bolton CF, Neuromuscular manifestations of critical illness. Muscle     [PubMed PMID: 15825186]
[7] Hermans G,De Jonghe B,Bruyninckx F,Van den Berghe G, Clinical review: Critical illness polyneuropathy and myopathy. Critical care (London, England). 2008;     [PubMed PMID: 19040777]
[8] Kamble N,Shukla D,Bhat D, Peripheral Nerve Injuries: Electrophysiology for the Neurosurgeon. Neurology India. 2019 Nov-Dec;     [PubMed PMID: 31857526]
[9] Weiss LD,Weiss JM,Johns JS,Strommen JA,Kim CT,Williams FH,Rashbaum IG, Neuromuscular rehabilitation and electrodiagnosis. 2. Peripheral neuropathy. Archives of physical medicine and rehabilitation. 2005 Mar;     [PubMed PMID: 15761795]
[10] Jang MH,Shin MJ,Shin YB, Pulmonary and Physical Rehabilitation in Critically Ill Patients. Acute and critical care. 2019 Feb;     [PubMed PMID: 31723900]
[11] Zifko UA,Zipko HT,Bolton CF, Clinical and electrophysiological findings in critical illness polyneuropathy. Journal of the neurological sciences. 1998 Aug 14;     [PubMed PMID: 9741406]
[12] Latronico N,Bertolini G,Guarneri B,Botteri M,Peli E,Andreoletti S,Bera P,Luciani D,Nardella A,Vittorielli E,Simini B,Candiani A, Simplified electrophysiological evaluation of peripheral nerves in critically ill patients: the Italian multi-centre CRIMYNE study. Critical care (London, England). 2007     [PubMed PMID: 17254336]
[13] Fan E, Critical illness neuromyopathy and the role of physical therapy and rehabilitation in critically ill patients. Respiratory care. 2012 Jun;     [PubMed PMID: 22663968]