Successful therapeutic response of intravenous immunoglobulin in Guillain-Barre syndrome with onset more than two weeks: a case report

Abstract

Guillain-Barré syndrome (GBS) has a global incidence of 1–2 cases per 100,000 individuals annually and remains the most common cause of acute flaccid paralysis. Standard treatment includes plasma exchange within 4 weeks or intravenous immunoglobulin (IVIg) within 2 weeks of symptom onset. However, select late-presenting cases may still benefit from IVIg treatment. Here, we report a case of a 17-year-old male who presented with progressive bilateral limb weakness and paresthesia that began approximately 3 weeks prior to admission. Furthermore, he experienced a brief syncope episode and defecation syncope, thus indicating possible autonomic involvement. On hospital day 3, after shared decision-making, the family opted for IVIg over plasma exchange. The laboratory tests on admission and before IVIg treatment showed normal albumin and elevated c-reactive protein (CRP) levels, which resulted in a low CRP-albumin ratio (CAR = 0.12). Although the value of CAR has not been elucidated in GBS, studies on Kawasaki disease suggest that a lower CAR may be associated with a favorable response to IVIg treatment. The patient received IVIg at 0.4 g/kg/day for 5 days, starting on hospital day 4 (approximately day 24 of symptom onset). By day 3 of IVIg therapy, the patient exhibited improved limb strength and reduced paresthesia. Moreover, the GBS disability score improved from 4 to 2 upon completion of treatment. This case supports the potential benefit of IVIg beyond the conventional 2-week window in GBS, particularly in patients exhibiting autonomic symptoms. The use of CAR as a potential supportive marker for predicting the response of patients with GBS to IVIg treatment warrants further investigation.