A Case of Severe Guillain-Barré Syndrome Which Was Difficult to Provide Early Diagnosis

Abstract

We present a case of a 2-year-old girl with severe Guillain-Barré syndrome (GBS), which is difficult to diagnose early. At day X, she had a fever of 39.0°C, which persisted for 3 days. As soon as the fever resolved, a rash appeared on her trunk and face. She was diagnosed with exanthem subitum. At day X+6, she could not stand and sit alone gradually. At day X+8, she was admitted to our hospital. Physical examination showed nuchal rigidity, extraocular muscle paralysis, lower limb muscle weakness, and tendon reflex weakness. Examination of cerebrospinal fluid revealed an increase in cell number, and nerve root enhancement of the cauda equina was detected on magnetic resonance imaging (MRI) myelography. There was no delay in conduction velocity in the lower limb peripheral nerve conduction examination, but F waves disappeared to 0%. We administered steroid pulse therapy and immunoglobulin therapy (1 g/kg) after considering acute flaccid paralysis. However, at day X+10, she suffered from respiratory failure due to aspiration pneumonia. Pediatric intensive care unit (PICU) management had been administered for 10 days. After an improvement in the respiratory condition, albuminocytologic dissociation was detected in the reexamination of cerebrospinal fluid, and a marked conduction block was detected in the nerve conduction reexamination of the upper and lower limbs. She was diagnosed with GBS, and immunoglobulin therapy (400 mg/kg/day, 3 consecutive days) was added. Rehabilitation was also introduced, and the paralysis of the lower limbs showed gradual improvement. She was able to stand and walk and was discharged at day X+47. Diagnosis of GBS may be delayed due to a lack of significant findings in cerebrospinal fluid and peripheral nerve conduction velocity examinations in the early stage of the disease. However, F wave and MRI myelography may be useful examinations for early diagnosis. In cases of rapidly progressing muscle weakness and respiratory failure, it is important to administer medical treatment with suspicion of GBS.