Guillain-Barre syndrome masquerading as acute respiratory failure in an infant.

Guillain-Barré syndrome (GBS) is a rare entity in infants. We report a case of GBS in a 5-month-old girl. The child presented with cough, loose stools, breathing difficulty, and listlessness. The child was treated as pneumonia with respiratory failure. Due to difficulty in weaning from ventilation with areflexia, marked hypotonia, and reduced power in all four limbs; possibilities of spinal muscular atrophy, poliomyelitis, and myopathies were kept. Nerve conduction velocity study was suggestive of mixed sensory-motor, severe axonal, and demyelinating polyradiculoneuropathy. Cerebrospinal fluid study revealed albuminocytological dissociation. Child was diagnosed as GBS and treated with intravenous immunoglobulin. Child recovered completely on follow-up. GBS should be considered as a differential diagnosis in acute onset respiratory failure with neuromuscular weakness in infants.

Introduction

Guillain-Barré syndrome (GBS) has a worldwide annual incidence of 1.3 cases/100,000.[12] The incidence is lower in children (age <15 years), 0.38 and 0.91 cases/100,000 with a mortality rate of 3–5%.[123] Very few cases have been reported in infants.[45] We report a case of GBS in a 5-month-old girl.

Case Report

A 5-month-old girl presented with cough, loose stools for 5 days, breathing difficulty, and listlessness for 3 days. Child had received DPT vaccine 25 days back. Child was treated as pneumonia; however, due to worsening respiratory distress, she was referred to our center. At arrival, she was gasping and started on mechanical ventilation. Chest examination revealed bilateral crepitations and rest systems were normal. A working diagnosis of pneumonia with respiratory failure was made, and child started on intravenous piperacillin-tazobactam. Investigations revealed hemoglobin 9.2 g/dL, white blood count 15,200/cum, neutrophil - 62%, lymphocytes - 32%, platelet count - 288000/cmm, C-reactive protein - 1.08 mg/L, and procalcitonin - 0.27 ng/ml. Chest X-ray was normal. A detailed neuromuscular examination revealed areflexia, marked hypotonia, and reduced power in all four limbs. In the light of minimal ventilator requirements, normal chest radiograph, and neuromuscular weakness with areflexia possibilities of spinal muscular atrophy (SMA), poliomyelitis, and myopathies were kept. Serum potassium was 3.70 mmol/L, and serum calcium was 9.03 mg/dL. Creatine phosphokinase was 46 U/L. Stool specimen for polio virus isolation and SMN gene studies were sent. Nerve conduction velocity showed absent compound muscle action potential (CMAP) in both common peroneal nerves and increased distal latency and decreased CMAP amplitude and conduction velocity in the left median, left ulnar, and both tibial nerves. F-wave was absent in left median, left ulnar, both common peroneal, and posterior tibial nerves. Sensory nerve action potential was also absent in left median, left ulnar, and both sural nerves, suggestive of mixed sensory-motor, severe axonal, and demyelinating polyradiculoneuropathy. Cerebrospinal fluid (CSF) was acellular with protein - 146.3 mg/dL. Child was given two doses of intravenous immunoglobulin at 1 g/kg/day. Child required tracheostomy for prolonged ventilation. There was a gradual improvement in power in all limbs over next 4 weeks, child was weaned off from the ventilator and tracheostomy tube was decannulated before discharge. A review of literature revealed only two reported cases of GBS in infants until date worldwide. On follow-up after 1 month, the child was doing well and attained age appropriate milestones. The stool for poliovirus and genetic studies for SMN gene were negative.

Discussion

GBS describes a heterogeneous condition with a number of variants. The classical presentation is characterized by an acute monophasic, nonfebrile, postinfectious illness manifesting as ascending weakness, and areflexia. Sensory, autonomic, and brainstem abnormalities may also be seen. The clinical suspicion of GBS in our case was based on the presence of a weakness, flaccidity, and areflexia. The age of the patient was the cause of diagnostic dilemma with only two younger children reported until date with postnatally acquired GBS on an extensive literature search.[45] Differential diagnoses such as poliomyelitis, SMA, congenital myopathies, and muscular dystrophies had to be considered. Diagnosis of GBS was made with albuminocytological dissociation in CSF and suggestive electrophysiological findings.

This case is one of the youngest reported cases of GBS. We would wish to reemphasize and underline the importance of having a high index of suspicion of GBS in critically sick and ventilated infants with acute flaccid paralysis, irrespective of the age. A preceding history suggestive of etiology may or may not be available. Hence, a thorough clinical examination, lumbar puncture for CSF analysis, and electrophysiological studies to confirm the diagnosis are recommended.

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Conflicts of interest

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