539 - Rare case of Acute Sensory and Autonomic Neuronopathy in a 21-year-old male initially diagnosed with Guillain Barre Syndrome (GBS): a case report

Rare case of Acute Sensory and Autonomic Neuronopathy in a 21-year-old male initially diagnosed with Guillain Barre Syndrome (GBS): a case report

Case Description: Ten days after running a marathon, a previously healthy 21-year-old male presented with progressive back pain, weakness and bilateral dorsal foot numbness. He underwent two lumbar punctures; the first being unremarkable and the second, performed one week later, revealing cytoalbuminologic dissociation leading to a GBS diagnosis. He underwent an IVIG course with minimal improvement in strength and was transferred to acute rehab. His hospital course included recurrent intubations and ultimately tracheostomy placement, sensory ataxia, and severe autonomic dysfunction including labile blood pressures (often fluctuating daily from 70/40mmHg to 190/100mmHg) as well as severe gastrointestinal dysmotility with recurrent small bowel ileus. EMG demonstrated sensory greater than motor neuropathy in the extremities. Cervical and thoracic spine MRI revealed longitudinally extensive cord signal changes within the dorsal columns. Given his complex clinical picture and diagnostic studies, the diagnosis of acute sensory and autonomic neuronopathy (ASANN) was made.

Discussions: ASANN is a rare but devastating disease characterized by ganglionopathy affecting sensory and autonomic ganglia. Diagnosis is made clinically with the support of NCS, autonomic testing, and spinal cord MRI. It is thought to be an immune-mediated disease, although no specific antibody has been found. Immunomodulatory therapies have helped with mild symptom relief. As demonstrated with this case, many patients with ASANN are initially diagnosed with GBS. However, unlike GBS, those with ASANN have a limited recovery and a poor prognosis.

Conclusions: This case highlights the rare diagnosis of acute sensory and autonomic neuronopathy and its similar clinical course to GBS. Given the rarity of the case, further studies and reports of ASANN are required to further investigate pathophysiology and treatment modalities.