Nodo-/Paranodopathien - eine neue Entität der peripheren Neuropathien

In the last few years, the term nodo-/paranodopathy was suggested as a third entity besides demyelinating and axonal peripheral neuropathy to describe peripheral neuropathies with primary damage to the node of Ranvier. Nerve conduction studies of patients with nodo-/paranodopathies share some findings of demyelinating neuropathy, but histological work-up does not detect any features of de- or remyelination. Autoantibodies against nodal or paranodal structures are supposed to cause nodo-/paranodopathies. Whereas gangliosides are known to be an antigenic target for several years, autoantibodies against the paranodal proteins neurofascin-155, contactin-1 and Caspr have only been described in the last few years. These autoantibodies belong to the immunoglobulin subclass IgG4. Patients show a typical clinical picture of severe motor-dominant peripheral neuropathy with subacute onset and poor response to the first-line therapy with intravenous immunoglobulins or steroids, but good response to rituximab or plasma exchange. Additionally, some patients suffer from a debilitating tremor.