eMedicine Specialties > Neurology > Neuromuscular Diseases

Acute Inflammatory Demyelinating Polyradiculoneuropathy: Treatment & Medication

Author: Tarakad S Ramachandran, MBBS, FRCP(C), FACP, Professor of Neurology, Clinical Professor of Medicine, Clinical Professor of Family Medicine, Clinical Professor of Neurosurgery, State University of New York Upstate Medical University; Chair, Department of Neurology, Crouse Irving Memorial Hospital
Coauthor(s): Richard A Sater, MD, PhD, Consulting Staff, High Point Neurological Associates
Contributor Information and Disclosures

Updated: Jan 15, 2009

Treatment

Medical Care

Advances in supportive medical care have resulted in improved survival rates in acute inflammatory demyelinating polyneuropathy (AIDP).

  • Mechanical ventilatory assistance is required in about one third of patients with AIDP and lasts for an average of 49 days. Intubation should be performed when FVC drops to less than 15 mL/kg or negative inspiratory pressure is worse than -25 cm H2 O. Tracheostomy is usually recommended if mechanical ventilation will be required for more than 2-3 weeks. Bedridden patients need prophylaxis against thromboembolism. Subcutaneous heparin is the most common agent. Some may also need GI prophylaxis with an H2-blocker (or similar agent).
  • Enteric nutrition is necessary for patients on mechanical ventilation. Nasogastric tubes or Dubhoff tubes can be used initially. Those requiring more than 2 or 3 weeks or enteric nutrition may require gastrostomy or jejunostomy tube feedings.
  • Cardiac monitoring is necessary. Chronic sinus tachycardia often responds to beta-blockers or calcium channel blockers. Bradycardia requires atropine treatment, if symptomatic. Heart block may require temporary pacing. Hypertension responds well to beta-blockers. These treatments should be administered cautiously under the direction of a cardiologist or critical care specialist, since one of the main causes of death is iatrogenic hypotension, especially in patients with autonomic failure.
  • Constipation is common in intubated patients with AIDP, and a bowel regimen is usually necessary. Some patients may also require enemas. Ileus is rare. If it occurs, bowel rest is usually necessary and parenteral nutrition can be used during that time.
  • Skilled nursing care of intubated patients is necessary to avoid skin breakdown. Special mattresses are available in most intensive care or step-down units. Communication difficulties can lead to frustration and exacerbate depression. Involvement of speech therapy, physical therapy, and occupational therapy is highly recommended. Many patients may require a rehabilitation unit after being weaned off a ventilator.
  • Conventional immunosuppressant treatments with corticosteroids have failed to show benefit. But immunomodulation with IVIg and plasmapheresis has led to faster recovery, relatively mild disability, and shorter hospital stays. IV steroid therapy alone is not indicated for the treatment of AIDP. Treatment is less likely to be effective if initiated more than 2 weeks after the onset of symptoms. Some patients with mild weakness, especially those presenting during the plateau, may not require immunomodulatory therapy. Plasmapheresis had shown to cut the respirator time and time to independent ambulation, by about half when treatment was given during the first week of the disease.
  • In their study of immunotherapy in Guillain-Barr é syndrome, Alshekhlee et al found an increasing use of IVIg over plasma exchange (PE). Older population and those with pulmonary or sepsis complications were likely treated with PE. The mortality rate was higher in patients treated with PE.12

Surgical Care

Tracheostomy is necessary in many intubated patients. Those requiring long-term enteral nutrition typically require a gastrostomy or jejunostomy.

Consultations

  • Neurology: For patients on general medicine or other services, neurological consultation is indicated to manage diagnostic studies and to help determine appropriate treatment.
  • Critical care: About one third of patients require mechanical ventilation. Any intubated patient or patient who is transferred to an ICU for monitoring should be monitored by a critical care or pulmonary specialist.
  • Surgery: Some patients may require tracheostomy or a feeding tube for parenteral nutrition.
  • Cardiology: Patients with arrhythmias in addition to sinus tachycardia or major cardiac rhythm abnormalities should be evaluated by a cardiologist.
  • Physical medicine and rehabilitation

Diet

No special diet is required.

Activity

Keep patients ambulatory if they are able to walk without assistance. Most patients who are admitted to the hospital require bedrest.

Medication

Immunomodulatory therapy with either IVIg or plasmapheresis has been demonstrated to result in more rapid recovery of AIDP than other treatments or no treatment. Recent large studies have demonstrated that the 2 treatments are equal in efficacy. Bedridden and critically ill patients also require treatment to prevent complications.

The mechanism of action of plasma exchange is not known. Suggested mechanisms include the removal of antibody, complement components, immune complexes, lymphokines, and acute-phase reactants. The generally recommended regimen includes every other day plasma exchange, totaling 6 exchanges in 2 weeks, with 3-3.5 L exchanged per treatment. If venous access is not of sufficient quality to ensure rapid blood withdrawal, a central line should be a consideration (in about 20% of cases).

Plasmapheresis (PE) is more frequently associated with severe adverse effects requiring cessation of therapy, including a bleeding diathesis. In addition, PE requires special, appropriate equipment and trained personnel. Also, younger children may be at risk for bleeding after insertion of wide catheters. Transient hypotension, which might occur, is corrected by adjusting the inflow-to-outflow ratio. Other common side effects include paresthesias, and rarely hypersensitivity reactions and hypocalcemia.

Immunomodulatory agents

AIDP is believed to be caused by immune dysregulation resulting from an attack against myelin. Therapy directed at the immune system can result in more rapid recovery. IVIG is especially proven highly effective in children.


IV Immunoglobulin (IVIg) or gamma globulin (many manufacturers)

IVIg is prepared from serum pooled from many donors by fractionation and purification. Most manufacturers include a detergent step to help prevent spread of viruses. Mechanism of action is poorly understood. However, it is believed to act by down-regulating antibody and cytokine production and by neutralizing antibodies specific for myelin. Also appears to down-regulate pro-inflammatory cytokines, such as IL-1 and gamma-IFN. Other proposed mechanisms are Fc receptor blockade and interference with complement cascade (ie, interfering with opsonization).

Adult

0.4 g/kg/d for 5 d has been used most often
Alternative regimen is 1-2 g/kg/d for 2 d

Pediatric

Administer as in adults

Documented hypersensitivity; IgA immunodeficiency (if present, low-IgA preparations available)
Severe congestive heart disease is relative contraindication

Pregnancy

C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus

Precautions

Common adverse effects include headache and itching—pretreatment with acetaminophen and diphenhydramine help prevent these effects
Patients with prior cardiac history are at risk for pulmonary edema—if it occurs, furosemide is drug of first choice
Rarer adverse effects include aseptic meningitis, stroke, skin rashes, renal tubular necrosis; hepatitis C has been transmitted by IVIg in past, but current preparations include detergent step
Can artificially decrease serum sodium and elevate ESR


Plasmapheresis or plasma exchange

This treatment entails removing blood from body, spinning it to separate cells from plasma, and replacing cells suspended in fresh frozen plasma, albumin, or saline. Can be performed using either 2 large-bore peripheral IV sites or multiple lumen central line.
May not be effective if started more than 2 wk after onset of symptoms.

Adult

Typical protocol: 200-250 mL/kg for each of 4 or 5 exchanges during an 8- to 10-d period

Pediatric

Administer as in adults

Recent myocardial infarction; coronary artery disease; arrhythmias; severe renal failure; severe hepatic failure; bleeding disorder

Pregnancy

C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus

Precautions

Back-to-back plasmapheresis sessions may remove clotting factors and can alter coagulation test results
Common adverse effects include headaches
Rare cases of myocardial infarction and stroke have been reported

Anticoagulant agents

Bedridden patients are at risk for deep venous thrombosis. This risk can be reduced by mild anticoagulation.


Heparin

Given subcutaneously, interacts with antithrombin III to decrease clot proliferation. This can result in decreased incidence of deep venous thrombosis.

Adult

5000 U SC tid

Pediatric

Not established

Digoxin, nicotine, tetracycline, and antihistamines may decrease effects; NSAIDs, aspirin, dextran, dipyridamole, and hydroxychloroquine may increase toxicity

Documented hypersensitivity; subacute bacterial endocarditis; active bleeding; history of heparin-induced thrombocytopenia

Pregnancy

C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus

Precautions

Some preparations contain benzyl alcohol as preservative and, when used in large amounts, may be associated with fetal toxicity (ie, gasping syndrome); preservative-free heparin recommended in neonates
Use with caution in patients with shock or severe hypotension

More on Acute Inflammatory Demyelinating Polyradiculoneuropathy

Overview: Acute Inflammatory Demyelinating Polyradiculoneuropathy
Differential Diagnoses & Workup: Acute Inflammatory Demyelinating Polyradiculoneuropathy
Treatment & Medication: Acute Inflammatory Demyelinating Polyradiculoneuropathy
Follow-up: Acute Inflammatory Demyelinating Polyradiculoneuropathy
References
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Further Reading

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Keywords

acute idiopathic polyneuritis, acute inflammatory demyelinating polyneuropathy, ascending paralysis, Guillain-Barré syndrome, Guillain-Barré-Strohl syndrome, AIDP, acute inflammatory demyelinating polyradiculoneuropathy

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Contributor Information and Disclosures

Author

Tarakad S Ramachandran, MBBS, FRCP(C), FACP, Professor of Neurology, Clinical Professor of Medicine, Clinical Professor of Family Medicine, Clinical Professor of Neurosurgery, State University of New York Upstate Medical University; Chair, Department of Neurology, Crouse Irving Memorial Hospital
Tarakad S Ramachandran, MBBS, FRCP(C), FACP is a member of the following medical societies: American Academy of Neurology, American Academy of Pain Medicine, American College of Forensic Examiners, American College of International Physicians, American College of Managed Care Medicine, American College of Physicians, American Heart Association, American Stroke Association, Royal College of Physicians, Royal College of Physicians and Surgeons of Canada, Royal College of Surgeons of England, and Royal Society of Medicine
Disclosure: Abbott Labs  Honoraria Consulting; Teva Marion Honoraria Consulting; Boeringer-Ingelheim Honoraria Speaking and teaching

Coauthor(s)

Richard A Sater, MD, PhD, Consulting Staff, High Point Neurological Associates
Richard A Sater, MD, PhD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Neurology, American Medical Association, and American Society of Neuroradiology
Disclosure: Nothing to disclose.

Medical Editor

Donald B Sanders, MD, EMG Laboratory Director, Professor of Medicine (Neurology), Division of Neurology, Duke University Medical Center
Donald B Sanders, MD is a member of the following medical societies: American Academy of Neurology, American Association of Neuromuscular and Electrodiagnostic Medicine, American Neurological Association, and New York Academy of Sciences
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: Nothing to disclose.

Managing Editor

Glenn Lopate, MD, Associate Professor, Department of Neurology, Division of Neuromuscular Diseases, Washington University School of Medicine; Chief of Neurology, St Louis ConnectCare, Consulting Staff, Barnes Jewish Hospital
Glenn Lopate, MD is a member of the following medical societies: American Academy of Neurology, American Association of Neuromuscular and Electrodiagnostic Medicine, and Phi Beta Kappa
Disclosure: Nothing to disclose.

CME Editor

Matthew J Baker, MD, Consulting Staff, Collier Neurologic Specialists, Naples Community Hospital
Matthew J Baker, MD is a member of the following medical societies: American Academy of Neurology
Disclosure: Nothing to disclose.

Chief Editor

Nicholas Y Lorenzo, MD, Chief Editor, eMedicine Neurology; Consulting Staff, Neurology Specialists and Consultants
Nicholas Y Lorenzo, MD is a member of the following medical societies: Alpha Omega Alpha and American Academy of Neurology
Disclosure: Nothing to disclose.

 
 
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