S3373 Atypical GBS as the Presenting Symptom of Celiac Disease: Recognizing Extra-intestinal Presentations of Celiac Disease

Abstract

Introduction: Celiac disease (CD) is an immune-mediated enteropathy in response to gluten that occurs in 1% of the population. Classic presenting symptoms include bloating, weight loss, and diarrhea. But, extraintestinal symptoms such as dermatitis herpetiformis, iron deficiency anemia, asymptomatic liver abnormalities, and more rarely, neurologic symptoms can be seen. We present a case of a patient who presented with worsening polyneuropathy and hepatitis leading to a final diagnosis of CD. Case Description/Methods: A 44-year-old male with history of recent left-chest shingles complicated by paralysis of right face and left leg weakness was initially admitted for suspected atypical Guillain Barre Syndrome (GBS) and treated with prednisone and a 5-day course of intravenous immunoglobulin (IVIG). The patient was readmitted a few weeks later with worsening neurologic status. A thorough infectious workup was unremarkable. The patient was also noted to have abnormal liver enzymes on prior admission that initially improved, but were noted to be worse on readmission up to ALP 1103, AST 634 and ALT 689. Given no prior history of liver disease, patient underwent a thorough workup including negative viral hepatitis, normal liver US, abdominopelvic CT showed scattered hypoattenuating lesions in the liver, one measuring up to 2.2 cm. He underwent a liver biopsy which demonstrated acute hepatitis with findings concerning for autoimmune versus drug-induced hepatitis. Autoimmune hepatitis panel was negative, however, he was found to have a tissue transglutaminase IgA >250 U/mL. Finally, he underwent upper endoscopy with biopsies that demonstrated findings consistent with celiac disease. Liver enzymes improved with gluten avoidance, but neurologic impairments persisted. Discussion: Atypical extra-intestinal symptoms at presentation of CD can result in diagnostic delay and permanent neurological disability if not treated. CD has been found in as many as 9% of patients with elevated liver enzymes. It is imperative not only to recognize neurologic presentations of CD but also the spectrum of possible liver impairments. Adherence to a strict gluten-free diet has been shown to improve symptoms of both neuropathy and liver enzyme abnormalities, but must be instituted quickly.