Guillain - Barre syndrome in a patient with acute myocardial infarction with ventricular septal defect repair treated with plasma exchange.

Guillain - Barre syndrome (GBS) is an acute, frequently severe progressive illness of peripheral nervous system that is autoimmune in nature. GBS after myocardial infarction (MI) with ventricular septal defect (VSD) is uncommon with high mortality rate if not treated promptly.[1] We report a successful outcome of GBS post MI with VSD in a 60-year-old male patient who was on a ventilator treated successfully with therapeutic plasma exchange.

Introduction

The Guillain — Barre syndrome (GBS) is an acute progressive illness affecting the peripheral nervous system. It is one of the common causes of acute areflexic paralysis in India. The reported incidence rate worldwide varies from 0.4 to 1.7/1000,000 persons per year.[1] There is an association with Epstein — Barr virus, measles, Campylobacter jejuni (causing diarrhea), HIV, Cytomegalovirus, post-vaccinal and postsurgical events.[2] The current hypothesis is in favor of immunological reaction due to hypersensitivity, perhaps as a result of unidentified allergen directed against the myelin sheath of peripheral nerves leading to an acute post infective polyradiculoneuropathy.[2] Severe forms show axonal degeneration.[2]

We describe a case of GBS post myocardial Infarction (MI) and ventricular septal defect (VSD) repair, which was treated with therapeutic plasma exchange (TPE) successfully in the Intensive Cardiac Care Unit.

Case Report

A 60-year-old male patient presented with a complaint of acute onset of chest pain associated with nausea and perspiration. Acute inferior MI was evident on electrocardiography and biochemically. Full blood count, erythrocyte sedimentation rate, liver function tests, chest X-ray film, plasma electrolyte, urea and glucose concentrations were normal. Streptokinase was given to the patient for the treatment. Next day he was operated for VSD. On postsurgical 6th day, patient had intermittent fever associated with night sweats. He complained of severe weakness of both upper and lower limbs. He also noticed numbness and loss of superficial sensation such as touch, pain and temperature below the level of umbilicus. After 4 days of onset of weakness, there was total paralysis of both upper and lower limbs. He also complained of difficulty in breathing. He was put on a ventilator. Patient had a history of diabetes mellitus, high blood pressure and smoking. Patient had areflexic quadruparesis, so nerve conduction test was done, which was suggestive of acute inflammatory demyelinating polyradiculoneuropathy, one of the variant of GBS. He was diagnosed as GBS on basis of Brighton case definitions which includes bilateral and flaccid weakness of limbs, decreased deep tendon reflexes in weak limbs, monophasic course and time between onset-nadir 12 h to 28 days, cerebrospinal fluid (CSF) cell count <50/μl and absence of the alternative diagnosis for weakness.[3]

Guillain — Barre syndrome is included in Category I indication for TPE as per American Society for Apheresis (ASFA).[4] So one volume TPE was performed with removal of 40 ml of plasma/kg as per patient weight, height, hematocrit and total blood volume on Spectra Optia apheresis machine (TERUMO BCT) in combination with replacement of 20 ml/kg fresh frozen plasma (FFP) and 15 ml/kg normal saline. Continuous monitoring of vitals, e.g., pulse, blood pressure and respiratory rate was carried out during the procedure to prevent any adverse events related to the procedure. Total 5 cycles of TPE were performed on alternate day in a period of 10 days with the removal of 200 ml/kg plasma. A few days after initiation of this therapy neurological improvement was seen. Patient was extubated from ventilator and his muscle power improved from grade 0 (complete paralysis) to grade-III (movement possible against gravity, but not against resistance) (Grading of muscle power is as per Medical Research Council Scale) after completion of five cycle of TPE.[2]

Discussion

A typical case of GBS begins with symmetric leg weakness and distal paresthesia. In more severe cases these symptoms worsen and spread as the face, respiratory and oropharyngeal muscles are involved. About one-third of the patients need mechanical ventilation at some point due to involvement of respiratory muscle. In the worst cases, patients have quadriplegia and a marked sensory deficit and may remain ventilator dependent for months.[5] Demyelination is usually associated with some evidence of associated inflammation, pathogenic evidence suggests that GBS is the consequence of misdirected humoral immune response to a preceding infection most likely C. jejuni, Epstein — Barr virus, Varicella zoster virus, Lyme disease, Mycoplasma and HIV.[5] The development of a GBS after MI with VSD is an uncommon, but highly lethal complication.[6]

The most important laboratory aids are electrodiagnostic studies and CSF examinations. CSF is usually under normal pressure and is acellular. Nerve conduction studies are a dependable and an early diagnostic indicator of GBS and in cases with a typical clinical presentation.

Guillain — Barre syndrome patients need constant monitoring and support of vital functions. TPE is particularly effective for patients who receive the treatment within 7 days of onset and is the treatment of choice in acutely ill patients.

1-1.5 plasma volume should be exchanged within 12-24 h of the decision to perform total plasma exchange. TPE involves the removal of injurious macromolecules from the plasma of patients with various medical conditions. The ASFA states that TPE is indicated for many autoimmune diseases, and its benefit occurs through the removal of all inflammatory mediators and toxic proteins, including autoantibodies, complement components and cytokines.[4]

Therapeutic plasma exchange is relatively safe. It shortens the course of hospitalization and reduces the mortality and incidence of permanent paralysis.[7] Minor complications like hypotension, allergic reaction due to FFP were observed in the procedure, but these can be managed easily. To our knowledge only two cases of GBS after MI have been reported and in both cases, patient were treated successfully with TPE.[1] In our case patient has developed VSD after MI, which is a lethal complication. Patient had a respiratory infection which might have precipitated autoimmune response and caused the acute neurologic disorder.[8]

Thus, patients of GBS after MI with VSD repair can be managed easily by TPE.

Conclusion

The patients who complain of weakness of limbs after MI should undergo careful neurologic examination for early diagnosis of GBS. GBS after MI with VSD is a rare but fatal disease and associated with high mortality if not treated promptly. TPE procedure can be performed in these patients continuous cardiac monitoring. TPE should be started as early as possible after diagnosis of GBS for early recovery and better outcome.