Objective:

Discuss an atypical presentation of ataxic GBS with positive GD1b antibody. 

Background:

The clinical spectrum of Guillain-Barre syndrome (GBS) ranges from asymptomatic to rapidly progressive and fatal. Ataxic GBS is one of the rare presentations. It is characterized by profound ataxia without ophthalmoplegia.  We report a case of atypical presentation of GBS with rapidly progressive neurological deficits in an elderly male. 

Design/Methods:

N/A 

Results:

A 73-year-old right-handed male with past medical history of hypertension and hyperlipidemia presented with gait imbalance and distal extremity numbness for five days prior to presentation. He noted he was having difficulty walking but denied weakness. He reported "electricity" type pain in his arms and legs and perioral tingling. He denied any other neurological deficits. He denied recent illness or vaccination. Physical exam was remarkable for severe ataxia, areflexia, and mild nystagmus. An MRI of the brain, cervical and thoracic spine showed no pathology. Cerebrospinal fluid (CSF) sampling showed protein of 46 mg/dL and no cells with a negative CSF culture. An electromyogram showed motor-sensory non-length dependant non-axonal loss without demyelination. A diagnosis of variant Guillain-Barre syndrome was suspected, and the patient received a 5-day course of intravenous immunoglobulins (IVIG). Despite this the patient developed neuromuscular respiratory failure necessitating intubation. GD1b antibody titter was found to be elevated at 1:800 (reference <1:100)  

Conclusions:

The ataxic variant of GBS is a very uncommon disease pathology. This case highlights the importance of considering different subtypes and presentations of GBS and related disorders. Early diagnosis and treatment with IVIG are important for a good outcome.