CIDPバリアント

CIDP Variants

慢性炎症性脱髄性多発ニューロパチー（chronic inflammatory demyelinating polyneuropathy：CIDP）は，慢性脱髄性ニューロパチーを基礎としてさまざまな臨床症候や経過，治療反応性を示すheterogeneousな症候群である。典型的CIDPのほか，多巣性CIDP，遠位型CIDPは，CIDPの三大病型で，その臨床的・電気生理学的・病理学的な検討から，それぞれが異なる病態生理を持つ疾患単位である可能性が示唆されている。

Abstract

Chronic inflammatory demyelinating polyneuropathy (CIDP) is a heterogeneous syndrome that has several variants. Although they share macrophage-associated demyelination, clinical, neurophysiological, and pathological investigations have demonstrated that each subtype has a different pathophysiology. Multifocal CIDP exhibits a chronic course with asymmetrical symptoms. Its neurophysiological significance involves multifocal demyelination at intermediate nerve sites. Distal CIDP has a prolonged chronic course, presenting sensory and motor symptoms in a length-dependent manner. Furthermore, it frequently coexists with IgG M proteinemia or other hematologic disorders. Motor CIDP displays symmetric muscle weakness similar to typical CIDP but lacks sensory involvement. Often, motor CIDP is associated with malignancy or inflammatory diseases. Although acute deterioration after corticosteroid therapy in patients with motor CIDP is well-known, the available evidence to support this is limited.