Chronic acquired demyelinating polyneuropathy

Chronic acquired demyelinating polyneuropathy, also known as chronic inflammatory demyelinating polyneuropathy (CIDP), is an immune-mediated disorder affecting the peripheral nervous system. In this condition, the body's immune system mistakenly attacks the myelin sheath — the protective insulating layer surrounding peripheral nerves — leading to progressive or relapsing weakness and impaired sensory function in the legs and arms. The disease primarily affects the peripheral nervous system, including motor and sensory nerves, resulting in symptoms such as progressive symmetric weakness of both proximal and distal muscles, numbness, tingling, pain, fatigue, and impaired balance. Deep tendon reflexes are typically reduced or absent. The course may be chronic progressive or relapsing-remitting. Diagnosis is based on clinical features, electrodiagnostic studies showing demyelinating features (such as prolonged distal latencies, reduced conduction velocities, conduction block, and temporal dispersion), elevated cerebrospinal fluid protein, and sometimes nerve biopsy. CIDP must be distinguished from hereditary demyelinating neuropathies such as Charcot-Marie-Tooth disease and from other acquired neuropathies. Treatment options include immunomodulatory therapies. First-line treatments are intravenous immunoglobulin (IVIg), subcutaneous immunoglobulin, and corticosteroids. Plasma exchange (plasmapheresis) is also effective. For refractory cases, immunosuppressive agents such as azathioprine, mycophenolate mofetil, or rituximab may be considered. Many patients respond well to treatment, though some experience residual disability. Long-term management often requires ongoing immunotherapy to prevent relapses.

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