Acute-onset chronic inflammatory demyelinating polyneuropathy with focal segmental glomerulosclerosis

Quek, Amy May Lin; Soon, Derek; Chan, Yee Cheun; Thamboo, Thomas Paulraj; Yuki, Nobuhiro

doi:10.1016/j.jns.2014.03.040

Cited by 11 publications

(6 citation statements)

References 35 publications

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“…Since 1973, a few cases of concomitant inflammatory demyelinating polyradiculoneuropathy and nephrotic syndrome have been reported. The neuropathy was either an acute inflammatory demyelinating polyneuropathy (AIDP) or a chronic inflammatory demyelinating polyneuropathy (CIDP), and the nephrotic syndrome was related to either a focal segmental glomerulosclerosis (FSGS) or a membranous glomerulonephritis (MGN) . Time course, severity, and response to treatment were heterogeneous, and evolution of neuropathy and nephropathy could be independent.…”

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confidence: 99%

“Neuro‐renal syndrome” related to anti‐contactin‐1 antibodies

et al. 2019

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confidence: 99%

“Neuro‐renal syndrome” related to anti‐contactin‐1 antibodies

et al. 2019

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“…By reviewing the cases reported in literature, Quek et al classified the clinical diagnoses as follows: GBS was time to nadir within 4 weeks, whereas CIDP followed a chronic course lasting beyond 2 months. [6] The patient in case 1 showed rapidly deteriorating muscle weakness within 19 days, while the 1 in case 2 presented as the same is a more progressive, recurrent, and chronic (more than 6 months) manner. Based on the CSF and nerve conduction studies, they were diagnosed as having GBS and CIDP.…”

Section: Discussionmentioning

confidence: 99%

Inflammatory demyelinating neuropathies with focal segmental glomerulosclerosis

et al. 2018

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Rationale:Inflammatory demyelinating neuropathies such as Guillain–Barré syndrome (GBS), chronic inflammatory demyelinating polyneuropathy (CIDP), and focal segmental glomerulosclerosis (FSGS) are autoimmune disorders that may have a common pathogenesis. Here, we describe 2 unique cases of FSGS, 1 with GBS and the other with CIPD. We believe that reviewing these multisystemic diseases will help in better understanding of FSGS pathogenesis.Patient concerns:The 1st patient, a 66-year-old woman, complained of tingling and numbness in the limbs and within 2 days, she developed progressive muscle weakness. The 2nd patient was a 63-year-old man with a complaint of lower-limb edema, lower-limb weakness, and numbness.Diagnosis:In the 1st patient, a diagnosis of GBS was confirmed with the nerve conduction velocity test as well as CSF studies. A renal biopsy revealed FSGS. The 2nd patient was diagnosed with CIDP and a subsequent renal biopsy revealed FSGS.Interventions:Large dose of steroid with calcineurin inhibitor, intravenous immunoglobulin, and supportive treatment.Outcomes:Neurologic symptoms disappeared, urine protein was maintained at low levels, and no further recurrences were noted in 2 cases. INF2 gene mutation was not found in either case.Lessons:Co-occurrence of inflammatory demyelinating polyneuropathy, GBS, CIDP, and FSGS suggests synergistic cellular and humoral autoimmune mechanisms related to either cross-reactivity within antigenic targets or mimicry epitopes. Further follow-up and intensive study for the pathogenesis are necessary.

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“…One of the case reports suggested inverted formin 2 expression on Schwann cells and podocytes to be the target of antibodies. 15 Two case reports discussed dysregulation of cellular and humoral responses resulting in the formation of antibodies to monosialoganglioside GM1 of the IgG1 subclass, along with deposition of a circulating immune complex in the setting of increased interleukin 2 and vascular endothelial growth factor levels and increased capillary permeability. 10 , 11 A decreased erythropoietin level in the setting of nephropathy and its role as a neurotrophic factor, vital to neuronal growth and myelin integrity, was also deemed responsible for neuropathy in another report.…”

Section: Discussionmentioning

confidence: 99%

Anti-Neurofascin–Associated Nephrotic-Range Proteinuria in Chronic Inflammatory Demyelinating Polyneuropathy

et al. 2020

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There are few case reports of concomitant chronic inflammatory demyelinating polyneuropathy (CIDP) and focal segmental glomerulosclerosis. A rare autoantibody to a neuronal and podocyte structural component, neurofascin, may be contributory. A Black man in his 40s presented with worsening polyneuropathy requiring mechanical ventilation and initially acute inflammatory demyelinating polyneuropathy was diagnosed. After a poor response to intravenous immunoglobulin, plasmapheresis was initiated. The patient also had concomitant new-onset nephrotic-range proteinuria. A limited kidney biopsy was interpreted as minimal change disease and was treated with prednisone. After some improvement, the patient was extubated; however, he later re-presented with worsening symptoms requiring mechanical ventilation and was re-treated with plasmapheresis. Due to the protracted course and poor response to intravenous immunoglobulin, acute-onset CIDP was diagnosed and a neuromuscular antibody workup returned positive for neurofascin, supporting the diagnosis of seropositive acute-onset CIDP. A repeat kidney biopsy demonstrated focal segmental glomerulosclerosis and acute tubular damage. The patient was treated with steroids and tacrolimus and later transitioned to rituximab. Neurofascin enzyme-linked immunosorbent assay then tested negative with concomitant resolution of both neuropathy and proteinuria. Further studies will help validate these findings and the treatment strategy.

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Acute-onset chronic inflammatory demyelinating polyneuropathy with focal segmental glomerulosclerosis

Cited by 11 publications

References 35 publications

“Neuro‐renal syndrome” related to anti‐contactin‐1 antibodies

“Neuro‐renal syndrome” related to anti‐contactin‐1 antibodies

Inflammatory demyelinating neuropathies with focal segmental glomerulosclerosis

Anti-Neurofascin–Associated Nephrotic-Range Proteinuria in Chronic Inflammatory Demyelinating Polyneuropathy

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