Objective:

Background:

GBS is an acute inflammatory demyelinating disease affecting children aged 2 and older that presents as ascending weakness. Miller Fisher Syndrome is a variant of GBS characterized by descending weakness, ophthalmoplegia, and areflexia. Presentation of GBS and its variants in patients younger than 2 years of age presents significant diagnostic challenges.

Design/Methods:

N/A

Results:

A 10-month-old male presents with 10 hours of acute onset drowsiness, right eye fluttering and lateral deviation, and imbalanced movements with recent diagnosis of acute otitis media. Physical exam was notable for continuous eyelid fluttering and sleepiness. Remainder of the neurologic exam was normal. CTH showed AOM and LP and EEG were both negative. Over the following 2 days, he developed stridor, wheezing, bradycardia to the 70s, and hyporeflexia in addition to continued weakness, worsening ptosis, and transient anisocoria and miosis. EMG was not consistent with botulism and MRI showed enhancements of the conus medullaris, cauda equina, and CN3 and CN5. Patient was started on IVIG due to increased suspicions of GBS and showed improvement, no longer desatting or bradycardic. Repeat LP on day 7 showed a protein of 243 confirming our suspicion of GBS. Upon discharge, he was able to roll, lift his head, and had improved truncal control although he will require PT. Subsequently his anti-GQ1b antibody came back positive, indicating MFS variant of GBS.

Conclusions:

This case highlights that treatment for GBS should not be withheld if concern for GBS is high. Diagnostic uncertainly can be mitigated with a second LP and/or MRI but must be weighed with the risks of sedation, especially when there is already cardiopulmonary involvement.