Acute Inflammatory Demyelinating Polyradiculoneuropathy Treatment & Management

Updated: Jul 26, 2021
  • Author: Emad R Noor, MBChB; Chief Editor: Nicholas Lorenzo, MD, CPE, MHCM, FAAPL  more...
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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).

Walgaard et al proposed the Erasmus GBS Respiratory Insufficiency Score (EGRIS) to predict the probability of respiratory insufficiency in the first week of hospital admission. The score included three factors: the number of days between onset of weakness and hospital admission, the severity of weakness indicated by sum of medical research council (MRC) score, and presence of bulbar and or facial weakness at admission. The total score ranges from 0 to 7 with low risk categorized as 0–2 and high risk being 5–7. [36]  

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.

Chakraborty et al conducted a retrospective review of 187 GBS patients admitted over the course of 17 years. The review found that quadriparesis, bulbar and neck flexor weakness, and mechanical ventilation were associated with dysautonomia. Patients with dysautonomia more commonly had cardiogenic complications, syndrome of inappropriate ADH secretion, posterior reversible encephalopathy syndrome, and higher GBS disability score. Mortality was 6% in patients with dysautonomia vs 2% in the entire cohort. [37]

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.

The pain component of GBS receives little attention. In 1984, A H Ropper and Shahni published an article regarding pain in GBS. They evaluated 29 consecutive patients with GBS and found that 55% had pain early in the course of disease. Pain was described as muscular discomfort. 

Yao et al explored the association of pain and abnormal laboratory tests in a retrospective cohort of 252 GBS patients. They found that CSF protein was positively associated with pain in GBS patients.

Pharmacotherapy is widely accepted as the important component of pain management. A systematic review found that gabapentin and carbamazepine reduced pain severity when compared with placebo, but evidence is limited. [38, 39, 40]

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. [41]

The American Academy of Neurology (AAN) recommendations from 2012 show level A evidence of IVIg being as effective as plasma exchange. [42]  

The pathogenesis of GBS is not fully understood, and the mechanism of how intravenous immunoglobulin (IVIG) cures GBS remains ambiguous. Hou et al. investigated lymphocyte subsets in patients with acute inflammatory demyelinating polyneuropathy (AIDP) and acute motor axonal neuropathy (AMAN) before and after treatment with IVIG to explore the possible mechanism of IVIG action. They concluded that the changes in T- and B-lymphocyte subsets, especially in CD4+T-lymphocyte subsets, might play an important role in the pathogenesis of AIDP, and in the mechanism of IVIG action against AIDP. [43]

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Surgical Care

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

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Consultations

See the list below:

  • 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

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Activity

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

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