Acute Inflammatory Demyelinating Polyradiculoneuropathy Follow-up

Updated: Jun 08, 2017
  • Author: Tarakad S Ramachandran, MBBS, MBA, MPH, FAAN, FACP, FAHA, FRCP, FRCPC, FRS, LRCP, MRCP, MRCS; Chief Editor: Nicholas Lorenzo, MD, MHA, CPE  more...
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Follow-up

Further Outpatient Care

Generally, all patients in whom AIDP is suspected should be admitted for further monitoring and treatment.

Patients who present with mild neurologic impairment after already reaching a plateau can be treated as outpatients with close supervision.

Upon discharge, patients require several follow-up visits to ensure that relapses do not occur and to help coordinate home-health services if necessary. Physical and occupational therapy, either in a long-term rehabilitation unit or at home, help many patients return more rapidly to their baseline level of activity.

Relapses occur (10-20%) following completion of plasma exchange, and these relapses frequently respond to a second course of treatment. Similarly, relapses that follow IVIG therapy also respond to a second course.

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Further Inpatient Care

Based on the severity of symptoms, patients with acute inflammatory demyelinating polyradiculoneuropathy (AIDP) may require further inpatient services.

  • Patients should have cardiac monitoring to confirm and treat arrhythmias.
  • Pulmonary function tests such as FVC and negative inspiratory pressure should be performed 3-4 times a day until a patient has reached a plateau for several days.
  • Transfer to an ICU is recommended for patients with worsening respiratory effort (ie, FVC < 20 mL/kg) or cardiac arrhythmias.
  • Physical therapy should be initiated early to help increase patient activity and mobility. Patients who do not recover quickly benefit by transfer to an inpatient rehabilitation center before returning home.
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Transfer

Transfer patients to the ICU when respiratory failure is impending or when cardiac arrhythmias are occurring.

Transfer patients to regional or tertiary hospitals if a community hospital does not have an ICU or is unable to provide IVIg or plasmapheresis therapy.

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Complications

Critically ill patients are susceptible to the same complications as other intubated patients, including pneumonia, sepsis, skin decubiti, deep venous thrombosis, and urinary tract infections. Patients with AIDP have some unique complications that may cause significant morbidity, the most common being pain, labile blood pressure, and increased sensitivity to cardiac medications.

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Prognosis

About 75% of patients have an excellent recovery and regain their premorbid condition. Some of these patients experience easy fatigability for many years.

Almost all of the remaining patients have mild or moderately severe impairment but remain independent in most functions. Residual complaints include dysesthesias, foot drop, and intrinsic hand muscle weakness.

Severe disability occurs in fewer than 5% of patients, who do not recover full independence. Patients with residual deficits are usually those who required mechanical intubation. Improvement is usually complete by 6 months. In more serious cases, recovery may continue for 18-24 months.

Death occurs in only 2-6% of patients and is usually due to cardiac arrest, ARDS, pulmonary embolism, severe bronchospasm, pneumonia, or sepsis.

About 10% of patients have a relapse 1-6 weeks after completing immunomodulatory therapy. These patients can be treated with a second course of immunomodulation.

Fewer than 1% of patients have AIDP 1 or more years after onset of symptoms. In some cases, the recurrence follows immunization. This recurrence differs from CIDP.

Sporadic cases of recurrent Guillain-Barré syndrome [20] and rare cases of recurrent Guillain-Barré syndrome after a long asymptomatic period [21] have been reported. Some authors consider recurrent Guillain-Barré syndrome a variant of CIDP, while others maintain that they are 2 different entities. Martic et al describe a patient who developed Guillain-Barré syndrome as a child and experienced a full relapse after 19 years with another innocuous episode 10 years later. [22]

Several prognostic factors have been identified, including the following:

  • In general, younger patients have a better prognosis than older patients. Those patients with more severe weakness and those who are intubated have a worse prognosis than those with milder weakness.
  • Diarrhea as an antecedent association often is associated with C jejuni infection. These patients may have a more prolonged recovery.
  • Early improvement in strength during treatment is associated with a more rapid recovery. Low compound muscle action potential (CMAP) amplitudes (< 20% of normal) are considered a bad prognostic indicator.

In spite of therapy with plasma exchange or IVIG, the decrease in mortality has often been attributed to improved aggressive supportive treatment than to any drug treatment. This has included close monitoring with the avoidance of hypoxia, pain, and arrhythmogenic stimuli.

In the presence of dysautonomia, hypoxia can trigger cardiac arrhythmias. Tracheal suction can also at times result in cardiac arrhythmias. Ideally, these patients should be given extra oxygen before tracheal toilet.

Subcutaneous heparin to avoid venous thromboembolism, treatment of pain with analgesics including narcotics, treatment of hypotension and hypertension, as the case be and treatment of severe bradyarrhythmia all go a long way in decreasing mortality. Carbamazepine and gabapentin may help.

Persistent fatigue following Guillain-Barré syndrome is common and may be helped by a graded exercise program. C jejuni is often treated with a course of erythromycin.

Hyponatremia is due to inappropriate antidiuretic hormone secretion (SIADH) is best managed by fluid restriction coupled by the avoidance of hyponatremic fluids. Need for immunization should be reviewed on an individual basis.

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