Clinical Presentations of Different Types of Nodopathies.

BACKGROUND AND

OBJECTIVES

Nodoparanodopathies are subtypes of inflammatory polyneuropathies that differ in clinical presentation, duration, and response to intravenous immunoglobulins. Antineurofascin antibodies are detected by cell-based assays from serum or cerebrospinal fluid. The aim of our study was to describe the clinical presentation, electrophysiology, antibody assay, and response to treatment in different types of nodopathies.

METHODS

During the last 1 year, we came across three patients with neurofascin 140/186 positivity and one patient with neurofascin 155 antibody positivity. The clinical features, electrophysiology, treatment response, and outcome were studied after obtaining informed consent from the patients.

RESULTS

Both groups improved with treatment; however, patients with neurofascin 140/186 positivity were older, with proximal weakness and a good response to intravenous immunoglobulins, while the patient with neurofascin 155 positivity was younger, with cranial and respiratory involvement, gait ataxia, high cerebrospinal fluid protein, a prolonged clinical course, and an inadequate response to intravenous immunoglobulins. The interesting observation made was that both groups of patients had varying conduction blocks without temporal dispersion on nerve conduction studies, which reversed rapidly with treatment.

CONCLUSION

The spectrum of illness, electrophysiology, and treatment varied widely in affected patients. Ultimately, the outcome depended on one's clinical acumen, antibody levels, and prompt treatment, as illustrated in the patients mentioned. This patient series was small, but the results were illustrative of the complexities of the disorder and the necessity for testing for nodopathies in patients with atypical clinical presentations, a prolonged clinical course, or conduction blocks in nerve conduction studies.