Polyneuropathy with anti-sulfatide and anti-MAG antibodies: Clinical, neurophysiological, pathological features and response to treatment

- MAG and sulfatide are the main target antigens of IgM paraproteinemic neuropathy.
- Anti-MAG neuropathies have a homogeneous clinical and neurophysiological pattern.
- Anti-sulfatide neuropathies show heterogeneous picture and therapy response.
- Anti-sulfatide axonal neuropathies seem to be more refractory to therapy.
- Screening for anti-sulfatide antibodies may help in the therapeutic approach.

IgM paraproteins often present reactivity to myelin-associated glycoprotein (MAG) and sulfatide.We describe the clinical and neurophysiological findings, and therapy response in 21 patients with IgM paraproteinemic neuropathy (15 with anti-MAG antibodies, 1 with anti-sulfatide antibodies, and 5 with both reactivity), and in 2 with anti-sulfatide positivity and no hematological disease.All patients complained of sensory symptoms, the majority had demyelinating neuropathy. Indirect immunofluorescence on human normal sural nerves disclosed different staining patterns.Eight of 13 patients (6 anti-MAG, 1 anti-sulfatide, 1 both anti-sulfatide and anti-MAG antibodies) improved after Rituximab. IVIg, steroids and plasma-exchange were also administered with different responses.