Neurorenal Syndrome: Two Cases of Tip-Variant Focal Segmental Glomerulosclerosis Associated with Guillain-Barré Syndrome
Bibliographic record
Abstract
Introduction: Glomerular disorders have been associated with immune-mediated polyneuropathies in previous case reports. We present two cases of tip-variant focal segmental glomerulosclerosis (FSGS) associated with a variant of Guillain-Barré syndrome (GBS) in Winnipeg, Manitoba. Case Description: Two previously healthy males aged 62- and 55-years old presented to our hospital with extremity weakness and paresthesias. They each progressed to flaccid paralysis and respiratory failure despite IV immunoglobulin (IVIG) and plasma exchange therapy (PLEX). Initial investigations were consistent with Acute Motor-Sensory Axonal Polyneuropathy (AMSAN), a variant of GBS. Nephrotic syndrome was identified after four months in case one and immediately in case two. Each patient had 20 g/day of proteinuria, preserved renal function, and histologic diagnosis of tip-variant FSGS on renal biopsy. Both cases responded to high-dose corticosteroids initially. Case one relapsed during his taper requiring re-initiation of steroids and addition of mycophenolate mofetil (MMF). He was discharged following thirteen months in hospital with complete remission of proteinuria and ongoing neurologic recovery. Case two achieved complete remission of proteinuria and was discharged after six months with ongoing neurologic recovery. Discussion: Our cases have similar presentations and responses to therapy suggesting they may share a common circulating autoantibody reacting against shared neural and glomerular podocyte antigens. Circulating autoantibodies including anti-contactin-1 and neurofascin have previously been implicated in chronic inflammatory demyelinating polyneuropathy (CIDP), a chronic variant of GBS. Identifying the culprit immune target in primary FSGS is limited due to the absence of immune complex deposition. The timing of podocytopathy development compared to GBS is highly variable in cases reported throughout the literature. The onset and diagnosis of FSGS in Case 1 was either delayed or unrecognized illustrating the importance of educating clinicians about this neuro-renal syndrome. Although not routinely used in GBS, corticosteroids have led to favorable outcomes in our cases and those reported throughout the literature. Recognition of a co-existing nephrotic syndrome with GBS could significantly change management and impact treatment outcomes.
Fetched live from OpenAlex and de-inverted. Abstracts are not stored in this database: the inverted indexes are 8.6 GB of the frame’s 9.3 GB of text, and the host has 13 GB free.
How this classification was reachedexpand
Full frame machine prediction
Teacher imitationNot calibrated prevalence, not ground truth. Human validation pending. The Gemma side is a direct model label for every work in the frame, read from the title-only record. The Codex side is a classifier learned from the 10,348 direct Codex labels and calibrated to design-weighted sample rates; fields without enough sample support carry no Codex call. Candidate is the union of the two sides; consensus is their intersection. These outputs are machine_predicted_unvalidated and are not human labels.
Distilled classifier scores by category (both heads)
| Category | Codex | Gemma |
|---|---|---|
| Metaresearch | 0.000 | 0.003 |
| Meta-epidemiology (narrow) | 0.003 | 0.002 |
| Meta-epidemiology (broad) | 0.001 | 0.001 |
| Bibliometrics | 0.003 | 0.002 |
| Science and technology studies | 0.002 | 0.002 |
| Scholarly communication | 0.002 | 0.001 |
| Open science | 0.002 | 0.002 |
| Research integrity | 0.003 | 0.002 |
| Insufficient payload (model declined to judge) | 0.002 | 0.001 |
Machine scores (provisional)
The two teacher heads of the student model, read on this work. A score orders the frame for review; it never asserts a category, and the validation status ships verbatim with every row.
Baseline scores from an immature model (maturity gate not passed, 7 training rounds). Scores rank; they never assert a category.
score_only:v0-immature-baseline · verbatim from the scoring run: score_only means the number may rank works, and no category label ships from itClassification
machine, unvalidatedMachine predicted; a candidate call from one source (direct Gemma or distilled Codex), not a consensus.
How this classification was reached, model by model and score by score, is at the end of the page under "How this classification was reached".