Chronic Illness Polyneuromyopathy/ Myopathy

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Chronic Illness Polyneuromyopathy/ Myopathy

  1. 1. CHRONIC ILLNESS POLYNEUROMYOPATHY “A series of Unfortunate Events”.... Edel Joey Leonar Reyes, MD, FPCP 1st Year Pulmonology Fellow
  2. 2. OBJECTIVES To present a local case of chronically ventilated patient due to CINPM To review key points in the Scope of Disease, presentation, Risk Factors , Diagnostics and treatment options in CIPNM
  3. 3. MS, 90 yo, M, RPV  On chronic Hospice Care since 2004  Senile Dementia  COPD  HCVD, AF-CVR  Previous Chronic Smoker Admitted at VMMC due to Dyspnea and productive Cough Case Salient Features June 20, 2011
  4. 4.  PHYSICAL EXAM Lethargic, cachiectic, In Resp Distress  BP-110/70 CR – 68 RR – 28 cpm  Adventitious sounds by chest auscultation  DIAGNOSES:  Acute Respiratory Failure Secondary to Retained Secretions  HCAP  ECOPD
  5. 5. Patient on Ventilatory Support Antibiotic Therapy IV Steroids Nebulizations Diuretics Anti-Anginals Nutritional Support General Management Plans
  6. 6. Course of Admission 1st month • ARF • Medical/Rehab Management 2nd month • Retained Secretions • Weaning not Tolerated 3rd month • CRF • Intermittent Failed Weaning
  7. 7. 4th month • CRF • Tracheostomy 5th month • Retained Secretions • Weaning not Tolerated 6th month • Chronic Ventilator Dependence Course of Admission
  8. 8. Chronic Ventilator Dependence
  9. 9. Trial Weaning Weaning Failure What POSSIBLE Causes for this? LOOKING BACK…..
  10. 10. Potentially reversible reasons for difficult weaning:  Inadequate respiratory drive  Poor gas exchange  Psychological dependency  Ventilatory pump failure (usually due to inspiratory muscle weakness or fatigue) What POSSIBLE Causes for this?
  11. 11. Respiratory Muscle Weakness… Murray and Nadel’s Textbook of Respiratory Medicine, 5th Ed, 2010
  12. 12. Causes for inspiratory muscle weakness or fatigue:  Nutritional or metabolic deficiencies  Steroids  Chronic renal failure  Decreased protein synthesis and increased degradation  Decreased glycogen stores  Anemia  Persistently increased work of breathing  Cardiovascular failure  Neuromuscular blockers  Polyneuropathy of critical illness
  13. 13. PROLONGED VENTILATION
  14. 14. A REVIEW CHRONIC ILLNESS POLYMYONEUROPATHY
  15. 15. Nomenclature POLYNEUROPATHY MYOPATHY CIP/CIPN AQM CINMAs ICUAP NMDs
  16. 16. - CIPNM CHRONIC ILNESS POLYNEUROMYOPATHY Nomenclature CIPN MYOPATHY
  17. 17. CIPNM NEUROMUSCULAR WEAKNESS is a very common finding in patients who are in the ICU.  Possible COMMON causes:  Guillain-Barrý syndrome  rhabdomyolysis  cachecticmyopathy and  critical illness neuropathy and myopathy De Jonghe B, Lacherade JC, Durand MC, et al: Critical illness neuromuscular syndromes. NeurolClin 2008; 26:507-520.ix
  18. 18. “Spontaneous weaknesses indicate disease.” Hippocrates, 460-377 B.C. first detailed report described by Bolton and colleagues in 1984 In 1995 Maher et al demonstrated acute neuromuscular disorders to be a common cause of difficult weaning. CIPNM (Background)
  19. 19. initially described in sepsis and multisystem organ failure Most common acquired neuromuscular condition in adult ICU recognized as a serious complication contributes significantly to morbidity and mortality.Fishman’s Pulmonary Diseases and Disorders, 4th Ed. 2008 CIPNM
  20. 20. Scope of the Problem 7-90% of Reported cases Rudis MI, Guslits BJ, Peterson EL, et al. _Economic impact of prolonged motor weakness complicating neuromuscular blockade in the intensive care unit. Crit 33. Care Med 199624:1749-56
  21. 21. Scope of the Problem J Pak Med Assoc Vol. 60, No. 11, November 2010 AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE VOL 168 2003
  22. 22. Critical Care 2008, 12:238 (doi:10.1186/cc7100 Scope of the Problem
  23. 23. Short Term/Long Term Implications  Typically cause muscle weakness and paralysis and impair rehabilitation  prolong the need for ventilatory support as the phrenic nerve and diaphragmatic muscle can be involved  duration of weaning is increased 2 to 7 times  Improvement occurs within weeks in mild cases and within months in severe cases
  24. 24.  increased hospital costs, and increased mortality  In patients recovering from ARDS, persistent functional limitation due to muscle wasting and weakness 1 year after discharge was noted in all patients  Clinical and neurophysiological signs may remain present for up to 5 years after ICU discharge Short Term/Long Term Implications
  25. 25. most severe cases, 32% remain severely disabled with tetraparesis, tetraplegia, or paraplegia Critical Care 2008, 12:238 (doi:10.1186/cc7100
  26. 26. Clinical Signs and Symptoms  CIP and CIM share the major clinical sign of flaccid and usually symmetrical weakness  reduction in or absence of deep tendon reflexes  distal loss of sensitivity to pain, temperature, and vibration  involvement of the phrenic nerves and the diaphragm, and intercostal and other accessory respiratory muscles
  27. 27. Bolton CF. Neuromuscular manifestations of critical illness. Muscle Nerve 2005;32:140–63.
  28. 28. Journal review
  29. 29. Multi-center study 92, ICU patients Daily measurements of the action potential amplitude and nerve conduction velocity 30% developed either CIMP or CINP Onset time of critical illness myopathyand/or neuropathy during intensive care unit (ICU) stay Critical Care 2007, 11:R11 (doi:10.1186/cc5671)
  30. 30.  development of muscle weakness over days to weeks.  weakness may be variable and ranges from mild weakness to severe quadriplegia  follows a typical pattern of proximal greater than distal weakness, but diffuse muscle weakness may be seen.
  31. 31.  The pathophysiologic mechanisms leading to critical illness polyneuromyopathy are not well understood. PATHOLOGY
  32. 32. pure neuropathic changes, pure myopathic changes, and combined neuropathic and myopathic changes The relative distribution of neural versus muscle involvement in CIPNM has been difficult to define PATHOLOGY
  33. 33. PATHOLOGY  type II myocyte atrophy with thick filament (myosin) destruction  widespread myocyte necrosis with intracellular vacuolization and phagocytosis of muscle fibers
  34. 34. Type of Critical Illness Myopathy Acute necrotizing myopathy Occur after sepsis and trauma Generalized muscle weakness High serum creatine kinase Myoglobinurea Myopathy associated with NMB or Cortico- steroid Diffuse muscle weakness Muscle atrophy
  35. 35. CIPN Mechanism Unclear Axonal Degeneration 70 % Intact Nerves 30 %
  36. 36. Spectrum of Incidence Acute ARDS 3 % Organ Transplant 7 % Severe Sepsis 33 % MOF 50 % CIPNM IV STEROIDS
  37. 37. PATHOPHYSIOLOGY
  38. 38. RISK FACTORS SEPSIS/SIR S STEROIDS Neuro- Blockers Hyperglycemia OTHERS TPN Aminoglycosides Female Gender Duration of Illness Renal Failure CIPNM
  39. 39. RESPIRATORY CARE • SEPTEMBER 2006 VOL 51 NO 9 RISK FACTORS  SEPSIS/SIRS  CORTICOSTEROIDS  NEUROMUSCULAR AGENTS  HYPERGLYCEMIA
  40. 40. Diagnostics  CPK levels may not be elevated  The diagnosis is usually based on clinical suspicion, confirmed by electrodiagnostic studies and muscle biopsy.
  41. 41. Electrophysiology The electrophysiological changes in CIPNM show both motor and sensory axonal dysfunction of upper and lower extremities Electroneurography and electromyography (ENG–EMG) is the gold standard for diagnosis
  42. 42. Zink, W. et al.(2009)Critical illness polyneuropathy and myopathy in the intensive care unit Nat. Rev. Neurol.doi:10.1038/nrneurol.2009.75 (CMAP) (SNAP) The first electrophysiological sign can occur very early, even within 2 to 5 days after the onset of critical illness
  43. 43. Comparative Features of Conditions Associated with ICU-Acquired Neuromuscular Weakness
  44. 44. Is electrophysiology necessary to diagnose CIPNM or is clinical evidence of a polyneuropathy sufficient? No uniform Dxtic criteria In the prospective study by Berek et al., Electrophysiological examination diagnosed the presence of CIPNM earlier than the clinical investigation. 2006 EFNS European Journal of Neurology 13, 1203–1212
  45. 45. Differential Diagnosis CIP GBS Normal nerve conduction velocity X CIP Rhabdomyolysis normal creatine phosphokinase, lack of muscle tenderness, and minimal electromyographic changes X CIP Cachectic Myopathy not usually associated with severe malnutrition X
  46. 46. Muscle Biopsy Muscle biopsy is the diagnostic method of choice for detection of structural abnormalities, but it is invasive and cannot reasonably be repeated in the same patient Routine muscle biopsy to diagnose CIM does not always exhibit clear pathology and may be questionable
  47. 47. PREVENTION  prevention of ventilator-associated pneumonia through use of semirecumbent positioning  reduction of ventilator-induced lung injury in patients with acute lung injury  limitation of sedative infusions through protocols that provide daily interruptions of sedative infusions.
  48. 48.  Efforts to prevent and aggressively treat sepsis  If duration of intensive care is associated with CIPNM, measures that reduce ICU length of stay may also decrease CIPNM. PREVENTION
  49. 49. CRITICAL ILLNESS MYOPATHY Severity of the Disease SIRS/ Sepsis Steroids and NMB’s Hypergly cemia TREATMENT Aggressive Management Aggressive Management D/C or Dose Adjustment Good Glycemic Control There is no established treatment for patients with ICU acquired weakness
  50. 50. In one RCT, the use of insulin to maintain serum glucose at 80 to 110 mg/dL, compared with a conventional range of 180 to 200 mg/dL, reduced critical illness polyneuropathy in patients in the ICU for at least 1 week by 44%. PREVENTION
  51. 51. TREATMENT Supportive Physical therapy and rehabilitation may be helpful in accelerating recovery Avoid Occurence of Infection
  52. 52. 2010
  53. 53. Take Home Message 2012 CIPNM is an Acute motor polyneuropathy in Critically ill Onset in 3 days of 3 D’s (3 Days, Difficulty Weaning, Distal Weakness with DTR) EPS studies are valuable and sensitive No definitive Treatment Weeks to months for possible Recovery

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