A Case of Guillain-Barré Syndrome Complicated by Nephrotic Syndrome and p-ANCA Positivity

Relapsing Guillain Barré Syndrome and nephrotic syndrome secondary to focal segmental glomerulosclerosis

“…Multifocal infiltration of lymphocytes and macrophage throughout the peripheral nervous system is the pathologic hallmark of GBS [7]. Its etiology is unknown; however, it may be preceded or associated with viral infection (cytomegalovirus, EpsteinBarr), bacterial infection (C. jejuni), and/or systemic illness (lymphoma, sarcoidosis) [8]. Although the pathogenesis of GBS remains obscure, it is generally held that it is an autoimmune disorder involving cellular and humoral immunity dysregulation [7].…”

Eosinophilic Granulomatosis with Polyangiitis Presenting as Acute Polyneuropathy Mimicking Guillain-Barre Syndrome

“…Ng et al first reported a case of both clinically and electrophysiologically mimicked GBS, but it was later found to be a case of EGPA due to findings of persistent eosinophilia, positive ANCAs, and eosinophilic vasculitis in a sural nerve biopsy [ 7 ]. In 1998, Keven et al reported the case of a patient diagnosed with GBS who later developed ANCA-positive nephrotic syndrome, suggesting that the polyneuropathy could be secondary to necrotizing vasculitis [ 8 ]. Since then, at least 3 other cases of EGPA presenting as GBS have been reported, all with acute ascending polyneuropathy and neurophysiologic studies suggestive of GBS [ 9 – 11 ].…”

Acute-onset chronic inflammatory demyelinating polyneuropathy with focal segmental glomerulosclerosis

“…Patients with GBS-like presentation who relapse shortly after treatment are classified as relapsing GBS (with treatment-related fluctuations), whereas those who deteriorate or relapse beyond 8 weeks from onset are classified as acute-onset CIDP [27]. Similar to those associated with membranous glomerulonephritis [9][10][11][12][13][14][15][16][17][18] and minimal change nephrotic syndrome [19][20][21][22], the neurologic presentations of previously reported cases of FSGS occurring in association with inflammatory neuropathies are diverse. These cases include patients with monophasic disease, like GBS [3,5,6], and those with a relapsing course, including CIDP [2,4,7,8] and relapsing GBS [1], suggesting heterogeneity in the time course and severity of neuropathy in these patients.…”

“…Their underlying glomerular pathologies include focal segmental glomerulosclerosis (FSGS) [1][2][3][4][5][6][7][8], membranous glomerulonephritis [9][10][11][12][13][14][15][16][17][18] and minimal change disease [19][20][21][22]. Here we describe a patient who synchronously developed acute-onset CIDP and FSGS, whose severe clinical manifestations were responsive to immunotherapy.…”