Inflammatory demyelinating polyneuropathy in a haemophiliac associated with human immunodeficiency virus infection, responding to high dose intravenous immunoglobulin

血友病患者合并人类免疫缺陷病毒感染引起的炎症性脱髓鞘性多发性神经病,对大剂量静脉注射免疫球蛋白治疗有反应

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Abstract

We describe what we believe to be the first case of chronic inflammatory demyelinating polyneuropathy (CIDP) occurring in a haemophiliac infected with the human immunodeficiency virus (HIV), and the first patient to show a clinical response to treatment with high dose i.v. immunoglobulin. A 55 year old, severe haemophiliac, known to be positive for HIV antibody, presented with a short history of motor weakness and variable sensory loss in both lower limbs. Clinical examination, electrophysiology and sural nerve biopsy confirmed a diagnosis of CIDP. He was treated with a 4-day course of high dose i.v. immunoglobulin, given as a daily infusion. This resulted in dramatic improvement in his neurological status which was evident both clinically and functionally. We conclude that CIDP can occur in HIV-positive haemophiliacs as it does in homosexuals and drug abusers infected with the virus. We also suggest that high dose i.v. immunoglobulin may be effective in the treatment of this condition, especially when practical difficulties preclude the use of plasmapheresis and the use of immunosuppressive drugs is considered hazardous.

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