Critical illness polyneuropathy

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Critical illness polyneuropathy
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Critical illness polyneuropathy (CIP) and critical illness myopathy (CIM) are overlapping syndromes of diffuse, symmetric, flaccid muscle weakness occurring in critically ill patients and involving all extremities and the diaphragm with relative sparing of the cranial nerves. CIP and CIM have similar symptoms and presentations and are often distinguished largely on the basis of specialized electrophysiologic testing or muscle and nerve biopsy. [1] [2] The causes of CIP and CIM are unknown, though they are thought to be a possible neurological manifestation of systemic inflammatory response syndrome. [3] Corticosteroids and neuromuscular blocking agents, which are widely used in intensive care, may contribute to the development of CIP and CIM, [4] as may elevations in blood sugar, which frequently occur in critically ill patients. [5]

Contents

CIP was first described by Charles F. Bolton in a series of five patients. [6]

Combined CIP and CIM was first described by Nicola Latronico in a series of 24 patients. [7]

Signs and symptoms

People with CIP/CIM have diffuse, symmetric, flaccid muscle weakness. CIP/CIM typically develops in the setting of a critical illness and immobilization, so patients with CIP/CIM are often receiving treatment in the intensive care unit (ICU).[ citation needed ]

Weakness (motor deficits) occurs in generalized fashion, rather than beginning in one region of the body and spreading. Limb and respiratory (diaphragm) muscles are especially affected. The muscles of the face are usually spared, but in rare cases, the eye muscles may be weakened, leading to ophthalmoplegia. [8]

Respiratory difficulties can be caused by atrophy of the muscles between the ribs (intercostals), atrophy of the diaphragm muscle, and degeneration of the nerve that stimulates the diaphragm (phrenic nerve). [8] This can prolong the time it takes to wean a person off of a breathing machine (mechanical ventilation) by as much as 7 – 13 days. [9]

Deep tendon reflexes may be lost or diminished, and there may be bilateral symmetric flaccid paralysis of the arms and legs. The nervous system manifestations are typically limited to peripheral nerves, as the central nervous system is usually unaffected.[ citation needed ]

Cause

The causes of CIP and CIM are unknown, though they are thought to be a possible neurological manifestation of systemic inflammatory response syndrome. [3] [10]

Pathology

Nerve biopsy would show axonal neuropathy, [7] but it is no longer indicated. A muscle biopsy of critical illness myopathy would show selective loss of thick filaments in muscle, demonstrating the loss of myosin and the presence of muscle cell death (necrosis). [1] [11] When muscles lose stimulation from neurons, they can undergo degeneration. However, when critical illness myopathy occurs, it is not solely due to loss of innervation of the muscle. [11] With critical illness myopathy, no other cause of the muscle degeneration can be found.[ citation needed ]

Unlike Guillain–Barre syndrome, another neurological disorder that causes weakness, patients with critical illness polyneuropathy do not have loss of the myelin sheath that normally surrounds neurons (demyelination). [11]

Diagnosis

CIP and CIM are a major cause of ICU-acquired weakness (ICUAW). Current guidelines recommend a clinical diagnosis of ICUAW, made by manually testing the muscle strength with the use of the Medical Research Council (MRC) sum score or handgrip dynamometry. [12]

CIP/CIM is often not identified until a patient is unable to be successfully weaned from a mechanical ventilator. Early detection of the condition is difficult, because these patients are often sedated and intubated, and thus unable to cooperate with a thorough neuromuscular physical examination. [11] The use of conventional nerve conduction studies is time-consuming and requires specialized personnel; however, simplified electrophysiologic tests can be used as screening tools in the critically ill to confirm or exclude CIP/CIM. [13] [14] The peroneal nerve test is a validated, high-sensitivity, minimally invasive, non-volitional and quick diagnostic test which can accurately exclude CIP/CIM if the result is normal. [13] Moreover, patients with disuse atrophy and muscle deconditioning have normal electrophysiological tests even if muscle strength is severely reduced [14] Hence, these tests are important to define the cause of muscle weakness and can be helpful to refine the prognosis. [15]

Laboratory values

The serum creatine phosphokinase (CPK) can be mildly elevated. [11] While the CPK is often a good marker for damage to muscle tissue, it is not a helpful marker in CIP/CIM, because CIP/CIM is a gradual process and does not usually involve significant muscle cell death (necrosis). Also, even if necrosis is present, it may be brief and is therefore easily missed. If a lumbar puncture (spinal tap) is performed, the protein level in the cerebral spinal fluid would be normal.[ citation needed ]

Screening

Initial screening for CIP/CIM may be performed using an objective scoring system for muscle strength. The Medical Research Council (MRC) score is one such tool, and sometimes used to help identify CIP/CIM patients in research studies. The MRC score involves assessing strength in 3 muscle groups in the right and left sides of both the upper and lower extremities. Each muscle tested is given a score of 0–5, giving a total possible score of 60. An MRC score less than 48 is suggestive of CIP/CIM. However, the tool requires that patients be awake and cooperative, which is often not the case. Also, the screening tool is non-specific, because it does not identify the cause a person's muscle weakness.[ citation needed ]

Once weakness is detected, the evaluation of muscle strength should be repeated several times. If the weakness persists, then a muscle biopsy, a nerve conduction study (electrophysiological studies), or both should be performed. [11]

Prognosis

CIP/CIM can lead to difficulty weaning a person from a mechanical ventilator, and is associated with increased length of stay in the ICU and increased mortality (death). [9] It can lead to impaired rehabilitation. Since CIP/CIM can lead to decreased mobility (movement), it increases the risk of pneumonia, deep vein thrombosis, and pulmonary embolism.

Critically ill people that are in a coma can become completely paralyzed from CIP/CIM. [8] Improvement usually occurs in weeks to months, as the innervation to the muscles are restored. About half of patients recover fully. [8]

Epidemiology

While the exact incidence is unknown, estimates range from 33 - 57 percent of patients staying in the ICU for longer than 7 days. [9] More exact data is difficult to obtain, since variation exists in defining the condition.

The three main risk factors for CIP and CIM are sepsis and systemic inflammatory response syndrome (SIRS), and multi-organ failure. Reported rates of CIP/CIM in people with sepsis and SIRS range from 68 to 100 percent. [9] Additional risk factors for developing CIP/CIM include: female gender, high blood sugar (hyperglycemia), low serum albumin, and immobility. A greater severity of illness increases the risk of CIP/CIM. Such risk factors include: multi-organ dysfunction, kidney failure, renal replacement therapy, duration of organ dysfunction, duration of ICU stay, and central neurologic failure.[ citation needed ]

Certain medications are associated with CIP/CIM, such as corticosteroids, neuromuscular blocking agents, vasopressors, catecholamines, and intravenous nutrition (parenteral nutrition). Research has produced inconsistent results for the impact of hypoxia, hypotension, hyperpyrexia, and increased age on the risk of CIP/CIM. The use of aminoglycosides is not an independent risk for the development of CIP/CIM.[ citation needed ]

History

CIP was first described in 1984 by Charles F. Bolton in a series of five patients. [16] The condition used to be described as "Bolton's neuropathy.". [9] In 1996, Latronico and colleagues first described that CIP and CIM often coexist in the same patient. [7]

Terminology

A number of terms are used to describe critical illness polyneuropathy, partially because there is often neuropathy and myopathy in the same person, and nerve and muscle degeneration are difficult to distinguish from each other in this condition. Terms used for the condition include: critical illness polyneuromyopathy, critical illness neuromyopathy, and critical illness myopathy and neuropathy (CRIMYNE). [17] [18] [13] Bolton's neuropathy is an older term, which is no longer used.

See also

Related Research Articles

Diabetic neuropathy is various types of nerve damage associated with diabetes mellitus. Symptoms depend on the site of nerve damage and can include motor changes such as weakness; sensory symptoms such as numbness, tingling, or pain; or autonomic changes such as urinary symptoms. These changes are thought to result from a microvascular injury involving small blood vessels that supply nerves. Relatively common conditions which may be associated with diabetic neuropathy include distal symmetric polyneuropathy; third, fourth, or sixth cranial nerve palsy; mononeuropathy; mononeuropathy multiplex; diabetic amyotrophy; and autonomic neuropathy.

<span class="mw-page-title-main">Peripheral neuropathy</span> Nervous system disease affecting nerves beyond the brain and spinal cord

Peripheral neuropathy, often shortened to neuropathy, refers to damage or disease affecting the nerves. Damage to nerves may impair sensation, movement, gland function, and/or organ function depending on which nerve fibers are affected. Neuropathies affecting motor, sensory, or autonomic nerve fibers result in different symptoms. More than one type of fiber may be affected simultaneously. Peripheral neuropathy may be acute or chronic, and may be reversible or permanent.

<span class="mw-page-title-main">Polyneuropathy</span> Medical condition

Polyneuropathy is damage or disease affecting peripheral nerves in roughly the same areas on both sides of the body, featuring weakness, numbness, and burning pain. It usually begins in the hands and feet and may progress to the arms and legs and sometimes to other parts of the body where it may affect the autonomic nervous system. It may be acute or chronic. A number of different disorders may cause polyneuropathy, including diabetes and some types of Guillain–Barré syndrome.

In medicine, myopathy is a disease of the muscle in which the muscle fibers do not function properly. Myopathy means muscle disease. This meaning implies that the primary defect is within the muscle, as opposed to the nerves or elsewhere.

<span class="mw-page-title-main">Neuritis</span> Inflammation of a nerve or generally any part of the nervous system

Neuritis, from the Greek νεῦρον), is inflammation of a nerve or the general inflammation of the peripheral nervous system. Inflammation, and frequently concomitant demyelination, cause impaired transmission of neural signals and leads to aberrant nerve function. Neuritis is often conflated with neuropathy, a broad term describing any disease process which affects the peripheral nervous system. However, neuropathies may be due to either inflammatory or non-inflammatory causes, and the term encompasses any form of damage, degeneration, or dysfunction, while neuritis refers specifically to the inflammatory process.

Polyneuropathy in dogs and cats is a collection of peripheral nerve disorders that often are breed-related in these animals. Polyneuropathy indicates that multiple nerves are involved, unlike mononeuropathy. Polyneuropathy usually involves motor nerve dysfunction, also known as lower motor neuron disease. Symptoms include decreased or absent reflexes and muscle tone, weakness, or paralysis. It often occurs in the rear legs and is bilateral. Most are chronic problems with a slow onset of symptoms, but some occur suddenly.

Proximal diabetic neuropathy, also known as diabetic amyotrophy, is a complication of diabetes mellitus that affects the nerves that supply the thighs, hips, buttocks and/or lower legs. Proximal diabetic neuropathy is a type of diabetic neuropathy characterized by muscle wasting, weakness, pain, or changes in sensation/numbness of the leg. It is caused by damage to the nerves of the lumbosacral plexus.

<span class="mw-page-title-main">Chronic inflammatory demyelinating polyneuropathy</span> Medical condition

Chronic inflammatory demyelinating polyneuropathy (CIDP) is an acquired autoimmune disease of the peripheral nervous system characterized by progressive weakness and impaired sensory function in the legs and arms. The disorder is sometimes called chronic relapsing polyneuropathy (CRP) or chronic inflammatory demyelinating polyradiculoneuropathy. CIDP is closely related to Guillain–Barré syndrome and it is considered the chronic counterpart of that acute disease. Its symptoms are also similar to progressive inflammatory neuropathy. It is one of several types of neuropathy.

<span class="mw-page-title-main">Centronuclear myopathy</span> Medical condition

Centronuclear myopathies (CNM) are a group of congenital myopathies where cell nuclei are abnormally located in the center of muscle cells instead of their normal location at the periphery.

<span class="mw-page-title-main">Neurointensive care</span> Branch of medicine that deals with life-threatening diseases of the nervous system

Neurocritical care is a medical field that treats life-threatening diseases of the nervous system and identifies, prevents, and treats secondary brain injury.

<span class="mw-page-title-main">Hereditary motor and sensory neuropathy</span> Medical condition

Hereditary motor and sensory neuropathies (HMSN) is a name sometimes given to a group of different neuropathies which are all characterized by their impact upon both afferent and efferent neural communication. HMSN are characterised by atypical neural development and degradation of neural tissue. The two common forms of HMSN are either hypertrophic demyelinated nerves or complete atrophy of neural tissue. Hypertrophic condition causes neural stiffness and a demyelination of nerves in the peripheral nervous system, and atrophy causes the breakdown of axons and neural cell bodies. In these disorders, a patient experiences progressive muscle atrophy and sensory neuropathy of the extremities.

Critical illness–related corticosteroid insufficiency is a form of adrenal insufficiency in critically ill patients who have blood corticosteroid levels which are inadequate for the severe stress response they experience. Combined with decreased glucocorticoid receptor sensitivity and tissue response to corticosteroids, this adrenal insufficiency constitutes a negative prognostic factor for intensive care patients.

Anti-MAG peripheral neuropathy is a specific type of peripheral neuropathy in which the person's own immune system attacks cells that are specific in maintaining a healthy nervous system. As these cells are destroyed by antibodies, the nerve cells in the surrounding region begin to lose function and create many problems in both sensory and motor function. Specifically, antibodies against myelin-associated glycoprotein (MAG) damage Schwann cells. While the disorder occurs in only 10% of those afflicted with peripheral neuropathy, people afflicted have symptoms such as muscle weakness, sensory problems, and other motor deficits usually starting in the form of a tremor of the hands or trouble walking. There are, however, multiple treatments that range from simple exercises in order to build strength to targeted drug treatments that have been shown to improve function in people with this type of peripheral neuropathy.

The compound muscle action potential (CMAP) or compound motor action potential is an electrodiagnostic medicine investigation . The CMAP idealizes the summation of a group of almost simultaneous action potentials from several muscle fibers in the same area. These are usually evoked by stimulation of the motor nerve. Patients that suffer from critical illness myopathy, which is a frequent cause of weakness seen in patients in hospital intensive care units, have prolonged compound muscle action potential.

Electromyoneurography (EMNG) is the combined use of electromyography and electroneurography This technique allows for the measurement of a peripheral nerve's conduction velocity upon stimulation (electroneurography) alongside electrical recording of muscular activity (electromyography). Their combined use proves to be clinically relevant by allowing for both the source and location of a particular neuromuscular disease to be known, and for more accurate diagnoses.

Post-intensive care syndrome (PICS) describes a collection of health disorders that are common among patients who survive critical illness and intensive care. Generally, PICS is considered distinct from the impairments experienced by those who survive critical illness and intensive care following traumatic brain injury and stroke. The range of symptoms that PICS describes falls under three broad categories: physical impairment, cognitive impairment, and psychiatric impairment. A person with PICS may have symptoms from one or multiple of these categories.

Charles Francis Bolton, MD, CM, MS, FRCP(C), is a Canadian professor of neurology at Queen's University in Ontario, Canada. He was first to describe critical illness polyneuropathy in a series of patients.

Electrodiagnosis (EDX) is a method of medical diagnosis that obtains information about diseases by passively recording the electrical activity of body parts or by measuring their response to external electrical stimuli. The most widely used methods of recording spontaneous electrical activity are various forms of electrodiagnostic testing (electrography) such as electrocardiography (ECG), electroencephalography (EEG), and electromyography (EMG). Electrodiagnostic medicine is a medical subspecialty of neurology, clinical neurophysiology, cardiology, and physical medicine and rehabilitation. Electrodiagnostic physicians apply electrophysiologic techniques, including needle electromyography and nerve conduction studies to diagnose, evaluate, and treat people with impairments of the neurologic, neuromuscular, and/or muscular systems. The provision of a quality electrodiagnostic medical evaluation requires extensive scientific knowledge that includes anatomy and physiology of the peripheral nerves and muscles, the physics and biology of the electrical signals generated by muscle and nerve, the instrumentation used to process these signals, and techniques for clinical evaluation of diseases of the peripheral nerves and sensory pathways.

Mary M. Reilly FRCP is an Irish neurologist who works at National Hospital for Neurology and Neurosurgery. She studies peripheral neuropathy. She is the President of the Association of British Neurologists.

<span class="mw-page-title-main">Shin Joong Oh</span>

Shin Joong Oh is a Korean physician who is Distinguished Professor of Neurology Emeritus at The University of Alabama at Birmingham in the United States. Oh is a clinician, researcher, and educator known for his contributions to the fields of neurology and electrodiagnostic medicine, particularly electromyography. He retired in 2014.

References

  1. 1 2 Latronico N, Bolton CF (2011). "Critical illness polyneuropathy and myopathy: a major cause of muscle weakness and paralysis". Lancet Neurol. 10 (10): 931–4. doi:10.1016/S1474-4422(11)70178-8. PMID   21939902. S2CID   45670414.
  2. ↑ Maramattom BV, Wijdicks EF (2006). "Acute neuromuscular weakness in the intensive care unit". Crit. Care Med. 34 (11): 2835–41. doi:10.1097/01.CCM.0000239436.63452.81. PMID   16932235. S2CID   10556731.
  3. 1 2 Visser LH (2006). "Critical illness polyneuropathy and myopathy: clinical features, risk factors and prognosis". Eur. J. Neurol. 13 (11): 1203–12. doi:10.1111/j.1468-1331.2006.01498.x. PMID   17038033. S2CID   7557453.
  4. ↑ Schweickert WD, Hall J (2007). "ICU-acquired weakness". Chest. 131 (5): 1541–9. doi:10.1378/chest.06-2065. PMID   17494803.
  5. ↑ Hermans G; Wilmer A; Meersseman W; et al. (2007). "Impact of intensive insulin therapy on neuromuscular complications and ventilator dependency in the medical intensive care unit". Am. J. Respir. Crit. Care Med. 175 (5): 480–9. doi:10.1164/rccm.200605-665OC. PMID   17138955.
  6. ↑ Bolton CF; Gilbert JJ; Hahn AF; Sibbald, W.J. (November 1984). "Polyneuropathy in critically ill patients". J Neurol Neurosurg Psychiatry. 47 (11): 1223–31. doi:10.1136/jnnp.47.11.1223. PMC   1028091 . PMID   6094735.
  7. 1 2 3 Latronico N; Fenzi F; Recupero D; et al. (1996). "Critical illness myopathy and neuropathy". Lancet. 347 (9015): 1579–82. doi:10.1016/S0140-6736(96)91074-0. PMID   8667865.
  8. 1 2 3 4 Hermans, Greet; De Jonghe, Bernard; Bruyninckx, Frans; Berghe, Greet (2008). "Clinical review: Critical illness polyneuropathy and myopathy". Critical Care. 12 (6): 238. doi: 10.1186/cc7100 . PMC   2646339 . PMID   19040777.
  9. 1 2 3 4 5 Johnson, KL (Apr–Jun 2007). "Neuromuscular complications in the intensive care unit: critical illness polyneuromyopathy". AACN Advanced Critical Care. 18 (2): 167–80, quiz 181–2. doi:10.1097/01.AACN.0000269260.99169.70. PMID   17473545.
  10. ↑ Friedrich O; Reid MB; Van den Berghe G; et al. (2015). "The sick and the weak: neuropathies/myopathies in the critically ill". Physiol Rev. 95 (3): 1025–1109. doi:10.1152/physrev.00028.2014. PMC   4491544 . PMID   26133937.
  11. 1 2 3 4 5 6 Kress, John P.; Hall, Jesse B. (24 April 2014). "ICU-Acquired Weakness and Recovery from Critical Illness". New England Journal of Medicine. 370 (17): 1626–1635. doi:10.1056/NEJMra1209390. PMID   24758618.
  12. ↑ Fan E; Cheek F; Chlan L; et al. (2014). "An official American Thoracic Society Clinical Practice guideline: the diagnosis of intensive care unit-acquired weakness in adults". Am. J. Respir. Crit. Care Med. 190 (12): 1437–46. doi:10.1164/rccm.201411-2011ST. PMID   25496103.
  13. 1 2 3 Latronico N; Nattino G; Guarneri B; et al. (2014). "Validation of the peroneal nerve test to diagnose critical illness polyneuropathy and myopathy in the intensive care unit: the multicentre Italian CRIMYNE-2 diagnostic accuracy study". F1000Res. 3: 127. doi: 10.12688/f1000research.3933.3 . PMC   4184363 . PMID   25309729.
  14. 1 2 Moss M; Yang M; Macht M; et al. (2014). "Screening for critical illness polyneuromyopathy with single nerve conduction studies". Intensive Care Med. 40 (5): 683–90. doi:10.1007/s00134-014-3251-6. PMID   24623137. S2CID   23033336.
  15. ↑ Latronico N; Smith M. (2014). "Introducing simplified electrophysiological test of peripheral nerves and muscles in the ICU: choosing wisely". Intensive Care Med. 40 (5): 746–8. doi: 10.1007/s00134-014-3282-z . PMID   24711089.
  16. ↑ Bolton; Gilbert; Hahn; Sibbald (November 1984). "Polyneuropathy in critically ill patients". J Neurol Neurosurg Psychiatry. 47 (11): 1223–31. doi:10.1136/jnnp.47.11.1223. PMC   1028091 . PMID   6094735.
  17. ↑ Latronico N; Bertolini G; Guarneri B; et al. (2007). "Simplified electrophysiological evaluation of peripheral nerves in critically ill patients: the Italian multi-centre CRIMYNE study". Crit Care. 11 (1): R11. doi: 10.1186/cc5671 . PMC   2151880 . PMID   17254336.
  18. ↑ Guarneri B; Bertolini G; Latronico N (2008). "Long-term outcome in patients with critical illness myopathy or neuropathy: the Italian multicentre CRIMYNE study". J Neurol Neurosurg Psychiatry. 79 (7): 838–41. doi: 10.1136/jnnp.2007.142430 . PMID   18339730.
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