Chronic relapsing (dysimmune) polyneuropathy: pathogenesis and treatment

Area of Science:

• Neurology
• Immunology
• Pathology

Background:

• Chronic relapsing polyneuropathy (CRDP) is a distinct dysschwannian/demyelinating neuropathy.
• Its cause and chronicity mechanisms remain unclear, though immune dysregulation is suspected.
• Elevated CSF protein and nerve conduction slowing are characteristic.

Purpose of the Study:

• To evaluate the efficacy of corticosteroid therapy in CRDP.
• To determine optimal long-term management strategies for CRDP.
• To explore alternative treatments for refractory CRDP cases.

Main Methods:

• Retrospective analysis of 25 CRDP patients.
• Treatment involved high-dose daily prednisone, tapered to alternate-day low doses.
• Assessment of treatment response and recurrence rates.

Main Results:

• Prednisone therapy was highly successful in the majority of patients.
• A low alternate-day prednisone dose (10-20 mg) was effective for long-term recurrence prevention.
• Other immunosuppressants and plasmapheresis showed potential in difficult cases.

Conclusions:

• CRDP is a treatable idiopathic polyneuropathy with a presumed dysimmune basis.
• Alternate-day low-dose prednisone is an effective long-term maintenance therapy.
• Immunosuppressants and plasmapheresis may benefit refractory CRDP cases.