JNNP: IgG1 Pan-Neurofascin antibody identified a serious but treatable neurological disease with high mortality

Guillain-Barré syndrome (GBS) is characterized by weakness of the limbs, weakened deep tendon reflexes, and the course of unipolar disease reaches its lowest point within 4 weeks
.

Cranial nerve and autonomic nerve dysfunction are common, with neuromuscular respiratory failure in approximately 25% of patients

The local current must pass through the membrane at the Lang's junction to form a loop at the myelin sheath, and conduct skipping conduction.

The pathology of the lymph nodes is called " nodo/paranodopathy ", which is related to certain subtypes of GBS, in which anti-ganglioside antibodies are found
.

This article describes 8 patients with very severe neuropathy related to IgG ₁pan-neurofascin( panNF ) antibody

nodo / paranodopathy IgG ₁PAN-Neurofascin₁ ( panNF ) This article was published in "Neurology, Neurosurgery and Psychiatry" ( Journal of Neurology, Neurosurgery & Psychiatry )

From July 2017 to May 2020, serum samples from 649 patients with suspected inflammatory neuropathy and 210 controls were tested using cell-based live cell analysis (CBA) to detect lymph nodes (NF186) and accessory lymph nodes (NF155, CNTN1 and Caspr1) IgG antibodies to cell adhesion molecules
.

Use standardized application forms to collect clinical data

Serum samples from 649 patients with suspected inflammatory neuropathy and 210 controls were tested to detect IgG antibodies against cell adhesion molecules in lymph nodes (NF186) and paralymph nodes (NF155, CNTN1 and Caspr1)

Eight patients (1.
2%) had IgG antibodies that cross-reacted with lymph node/axon NF186 and NF140 subtypes and glial NF155 subtypes (called "panNF")
.

These antibodies were only IgG1, and IgG3 and/or IgG4 subclass antibodies were not detected

Eight patients (1.

All 8 panNF antibody-positive patients, 15 of 17 NF155 patients, 11 of 11 CNTN1 patients, 8 of 9 CNTN1/Caspr1 positive patients, and 194 of 603 seronegative patients provided Clinical details

Serological, radiological and histological examination results

PanNF antibody-positive patients are severely affected and rapidly develop severe quadriplegia
.

Compared with seronegative patients, they are more likely to have acute or subacute exacerbations and are initially diagnosed with GBS

PanNF antibody-positive patients are severely affected and rapidly develop severe quadriplegia

Clinical characteristics of panNF antibody patients and comparison with NF155, CNTN1, CNTN1/Caspr1 antibody patients and seronegative cohorts

During the treatment of neuropathy or shortly thereafter, two panNF antibody-positive patients were found to have IgG lambda protein and were subsequently diagnosed with lymphoproliferative diseases (Hodgkin’s lymphoma and chronic lymphocytic leukemia
.

Standard neuropathy screening for all patients) Blood
tests (including anti-GM1 and GQ1b ganglioside antibodies) were negative .
In panNF-positive patients, the cerebrospinal fluid (CSF) protein was normal or only slightly elevated when it appeared
.
All patients received intravenous immunoglobulin within 5 days (IVIg) 2 g/kg
.
In two cases, there was slight and/or transient improvement in neurological function
.
Six patients underwent at least one cycle of plasma exchange (PLEx), of which three patients showed mild but non-continuous The nerve recovery
.
The remaining four patients received rituximab treatment 3-4 months after the onset (repeating 1 gram after 2 weeks), and all four patients who received rituximab function were treated with rituximab.
It gradually improved, regained the ability to move independently, and was finally discharged home
.

All patients received intravenous immunoglobulin (IVIg) 2 g/kg within 5 days
.
In two cases, there was a slight and/or transient improvement in neurological function
.
Six patients underwent at least one cycle of plasma exchange (PLEx), and three of them showed mild but non-sustainable neurological recovery
.
The remaining four patients received rituximab treatment 3-4 months after the onset (repeating 1 gram after 2 weeks).
All four patients who received rituximab have gradually improved their functions and recovered.
He was able to move independently and was finally discharged home from the hospital
.

In short, IgG ₁ panNF antibody positivity defines a very serious autoimmune neuropathy
.
This article recommends targeted immunotherapy trials for this serological group of patients
.

₁ Fehmi  J ,  Davies  AJ ,  Walters  J Fehmi  J Fehmi Davies  AJ Davies Walters  J Walters , et al ₁ Journal of Neurology, Neurosurgery & Psychiatry  Published Online First:  16 August 2021.
  Online First: doi:  10.
1136/jnnp-2021-326343 doi: leave a message here