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PP13.11 - 2904: Severe onset and rapidly progressive acute motor axonal neuropathy in a 2-year-old male
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文摘
Guillain-Barré syndrome (GBS) is an immunomediated polyneuropathy and an important cause of acute neuromuscular paralysis. AMAN is a relatively aggressive disease comparing to Acute Inflammatory Demyelinating Polyradiculoneuropathy (AIDP) and its onset is usually more severe.

Methods

Herein we present an AMAN case which was presented with rapidly progressive flaccid paralysis.

Results

2 year-old male patient was brought to emergency room because of losing ability to walk. According to his parents' statements, he started to have difficulties while walking 12 hours ago, and he couldn't walk at all in the last hour. Patient lost motor control of his head in the follow up on the sixth hour. Because of this rapid and progressive onset and ascending flaccid tetraplegia patient was taken into the intensive care unit. On the thirty-sixth hour of the observation, patient required intubation and mechanical ventilation, due to cranial neuropathy and respiratory failure. His cerebrospinal fluid protein level was 48 mg/dl (15–45 mg/dl), and no cells were detected. Similarly, stool culture was negative for campylobacter jejuni. GQ1B, GD1B, GD1A, GM1 antibodies were studied and results were negative. Lumbosacral spinal magnetic resonance imaging was performed and contrast enhancement in conus medullaris level was detected. Electromyography was performed and findings were associated to AMAN. Initial treatment regime was 400 mg/kg/day intravenous immunoglobulin for five days. However, because of the ongoing progression, consecutive intravenous immunoglobulin and plasma exchange therapies were given. After four weeks of progression, patient stayed in the plateau phase for another four weeks, and descendent healing began subsequently.

Conclusion

There are a variety of forms of the GuillainBarré Syndrome. Although it can be seen in all age groups, it is relatively rare in patients under 2 years of age In selected cases like this one, consecutive plasmapheresis and intravenous immunoglobulin therapies should be considered.

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