Chronic inflammatory demyelinating polyradiculoneuropathy

被引:1
作者
Ehler, E. [1 ,2 ]
机构
[1] Neurol Klin FZS UP, Kyjevska 44, Pardubice 53203, Czech Republic
[2] Pardubicke Krajske Nemocnice As, Kyjevska 44, Pardubice 53203, Czech Republic
关键词
chronic inflammatory demyelinating neuropathy; demyelination and remyelination; electrophysiology; diagnostics; immunotherapy; INTRAVENOUS IMMUNOGLOBULIN; CONTROLLED-TRIAL; PLASMA-EXCHANGE; POLYNEUROPATHY; DIAGNOSIS; CIDP; NEUROPATHY; IVIG; CHALLENGES; GUIDELINE;
D O I
10.14735/amcsnn2018252
中图分类号
Q189 [神经科学];
学科分类号
071006 ;
摘要
Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is a chronic demyelinating polyneuropathy with a relapsing or progressive course. This is a dysimmune polyneuropathy with activation of humoral and cellular mechanisms with a primary lesion of myelin sheath in the peripheral nerves. Most frequent is a typical symmetrical polyneuropathy, multifocal neuropathy with a conduction block is less frequent, then distal symmetrical motor and sensory neuropathy, pure sensory or pure motor neuropathies occur rarely. Development of signs and symptoms beyond 2 months is a common feature for all variants. Diagnosis of CIDP is based on clinical findings, precisely developed electrophysiological criteria, and some laboratory findings - hyperproteinorrhachia with normal cell count, and MRI with thickening and enhancement of nerves in brachalis and lumbar plexuses or roots. Corticosteroids or intravenous immunoglobulins are used as induction therapy as the first-line treatment of CIDP. Therapeutic plasma exchange is a second-line therapy in patients with inefficacy or intolerability of the first-line treatment. As adjuvant therapy, they use azathioprin, mycofenolate mofetil, ciclosporin A, cyclophosphamide, and methotrexate. CIDP is a treatable neuropathy though in some patients it leads to invalidity.
引用
收藏
页码:252 / 264
页数:13
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