(Kiosk 3) Sensory Ganglionopathy Mistaken for Guillain-barré Syndrome: A Case Report

Case Diagnosis: Symptoms of weakness and sensory deficits originally diagnosed as Guillain-Barré Syndrome, but found to be sensory ganglionopathy through electrodiagnostic study.

Case Description or Program Description: The patient was admitted for subacute weakness and sensory deficits in his bilateral upper and lower limbs. He had inconsistent strength testing, global areflexia, limb incoordination, decreased light touch sensation, but intact pain/temperature sensation. Upon admission, he tested positive for COVID-19. Serologies and imaging were unremarkable. Cerebrospinal fluid analysis demonstrated albuminocytologic dissociation. He was diagnosed with Guillain-Barré Syndrome (GBS) secondary to COVID-19 and completed a five-day course of intravenous immunoglobulin (IVIG) with mild symptom improvement. He was offered inpatient rehabilitation, but declined and went home. One month later, he returned reporting impaired ability to self-care and continued weakness. He was directly admitted to the rehabilitation unit, started on IVIG for suspected recurrence of GBS, and underwent an electrodiagnostic study (EDX). EDX demonstrated diffuse absent sensory nerve conduction study (NCS) responses, prolonged R1 latencies on blink reflex testing, normal motor NCS, and relatively unremarkable electromyography testing. Further workup showed positive anti-Sjogren’s-Syndrome-related antigen A (anti-SSA) autoantibodies and elevated erythrocyte sedimentation rate (ESR).

Setting: Veterans Affairs Hospital

Assessment/Results: These EDX results, in combination with positive autoimmune workup, clinical signs of early limb incoordination, subacute onset of asymmetric, non-length-dependent sensory impairment in the upper and lower limbs, severely impaired proprioception, intact pain/temperature sensation, and inconsistent muscle strength are suggestive of probable sensory ganglionopathy based on proposed diagnostic criteria in the literature.

Discussion (relevance): This case highlights the importance of EDX for GBS diagnosis, which was not completed on initial admission. Further, poor response to treatment and clinical sensory-based changes should direct the need for further investigation.

Conclusions: The low prevalence of sensory ganglionopathies may lead to misdiagnosis especially without absolute established diagnostic criteria. EDX studies may aid in obtaining this diagnosis and guide appropriate workup, management, and treatment.