Neurology: Neurofascin-155 immunoglobulin subtypes, clinicopathological associations and neurological outcomes

Neurofascin-155 (NF155) autoantibody is one of the most common nodular and paranodular antibodies, accounting for 4% to 18% of all chronic demyelinating polyneuropathy (CIDP) cases
.

Despite the increasing application of these antibodies in clinical practice, research to evaluate long-term results and histopathological characteristics is limited

With this, Shahar Shelly and others at the Mayo Clinic explored the frequency of NF155 autoantibodies in a large cohort of demyelinating neuropathy
.

And evaluate the phenotype and histopathological specificity, as well as the difference in results between NF155-IgG4 seropositive, NF155 pan-IgG and NF155-IgM seropositive cases

Method
.

In this study, NF155-IgG4, NF155-IgG, and NF155-IgM autoantibodies were tested in the serum of neuropathic patients who attended the Mayo Clinic

They found 32 NF155 patients (25 NF155-IgG positive [20 NF155-IgG4 positive] and 7 NF155-IgM seropositive)
.

Patients with NF155-IgG4 seropositive have clinically more distal muscle weakness than proximal muscle weakness, positive sensory symptoms (tingling, asymmetric paralysis, neuropathic pain) and gait ataxia

Electrical diagnostic tests (EDX) show demyelinating polyneuropathy (19 / 20.
95%)
.

Autonomic nerve involvement occurred in 45% of patients (9 patients, median CASS score 3.

Diagnostic immunity

NF155-IgG positive NF155-IgG4 negative (NF155-IgG positive) and NF155-IgM positive patients are phenotypically different from NF155-IgG4 seropositive patients
.

NF155-IgG positive NF155-IgG4 negative (NF155-IgG positive) and NF155-IgM positive patients are phenotypically different from NF155-IgG4 seropositive patients
.

Compared with MAG-IgM neuropathy, sensory ataxia, neuropathic pain, cerebellar dysfunction, and root/plexus MRI abnormalities are significantly more common in NF155-IgG4-positive patients
.

Compared with NF155-IgG4 positive cases, the chronic immunosensory polyneuropathy (CISP)/CISP-plus phenotype is more common in Contactin-1 neuropathy
.

Compared with MAG-IgM seropositive cases, NF155-IgG4 positive cases responded well to immunotherapy.
Compared with contactin-1 IgG, distally acquired demyelinating symmetric neuropathy (P<0.
001) had better long-term clinical efficacy.
Good (P=0.
04)
.

The important significance of this study lies in the discovery of long-term follow-up and clinical results of NF155-IgG4 patients
.

Patients with NF155-IgG4 (rather than IgM or IgG) have unique clinical-electrodiagnostic characteristics

Patients with NF155-IgG4 (instead of IgM or IgG) have unique clinical-electrodiagnostic characteristics

Original source:
Shelly S, Klein C, Dyck PJB, et al.

Neurofascin-155 Immunoglobulin Subtypes: Clinicopathologic Associations and Neurologic Outcomes.
 Neurology.
Published online October 11, 2021:10.

Neurofascin-155 Immunoglobulin Subtypes: Clinicopathologic Associations and Neurologic Outcomes.

Leave a message here