慢性炎症性脱髓鞘性多发性神经病（CIDP）

慢性炎症性脱髓鞘性多发性神经病（CIDP）
原文链接;/articles/chronic-inflammatory-demyelinating-polyneuropathy
Chronic inflammatory demyelinating polyneuropathy (CIDP) is an acquired demyelinating disease involving peripheral nerves, and is generally considered the chronic counterpart to Guillain-Barré syndrome (GBS).
慢性炎症性脱髓鞘性多发性神经病（CIDP）是一种累及周围神经的获得性脱髓鞘疾病，通常认为是吉兰- 巴雷综合征（GBS）的慢性过程。

Clinical presentation
Patients typically present with a gradual and protracted (> 2 month) weakness of both proximal and distal musculature associated with areflexia and sensory changes. In some patients presentation can be acute and closely mimic Guillain-Barré syndrome (GBS).
临床表现
患者通常呈现渐进性或迁延的（> 2个月）近端和远端的肌无力，伴有腱反射消失和感觉异常。

在一些患者中，可以表现为急性过程，类似吉兰 - 巴雷综合征（GBS）症状。

Pathology
CIPD is an acquired demyelinating disease of the peripheral nervous system. Affected nerves demonstrate segmental infiltration with inflammatory cells (lymphocytes) anddemyelination . Over time there is proliferation of Schwann cells and deposition of collagen resulting in thickening of the nerve and the characteristic onion bulb appearance. Involvement of the nerves results in changes of denervation in the supplied muscles.
病理
CIPD是外周神经系统获得性脱髓鞘疾病。

受影响的神经表现为节段性的炎症细胞（淋巴细胞）浸润和脱髓鞘。

随着时间的推移，施旺细胞的增殖和胶原的沉积导致神经增粗和特征性洋葱球的出现。

受累神经支配的肌肉呈失神经改变。

Radiographic features
MRI
MRI is the modality of choice for imaging the changes of CIPD, the hallmark of which is thickening and enhancement of peripheral nerves, brachial and lumbosacral plexus and nerve roots. In many cases the nerves become so thickened that they resemble onion bulbs.
In addition the supplied muscles will demonstrated changes of denervation :
•acute to subacute
o diffuse increase in T2 signal
o diffuse increased enhancement (seen best on fat suppressed T1 C+)
•chronic
o fatty atrophy atrophy
影像学表现
MRI
MRI是用于成像CIPD变化的选择的模式，其标志是周围神经的增粗与强化，臂丛、腰骶丛和神经根。

在一些病例，神经变得非常稠密，使得它们类似于洋葱改变。

此外，受累神经支配的肌肉呈失神经改变：
•急性-亚急性
广泛的T2WI信号增高
增强后广泛的强化（在脂肪抑制T1 +C图像上显示最好）
•慢性
脂肪浸润、萎缩
Treatment and prognosis
CIDP can be treated with steroid, intravenous immunoglobulin (IVIG), plasmapheresis and immunosuppression.
治疗和预后
CIDP可以用类固醇，静脉注射免疫球蛋白（IVIG），血浆置换和免疫抑制治疗。

Differential diagnosis
•Guillain-Barré syndrome (GBS)
o acute presentation of CIPD can be similar
o difficult to differentiate in the first 6 weeks
o after 6-8 weeks GBS should be improving whereas CIDP will demonstrate chronic inflammation
鉴别诊断
吉兰 - 巴雷综合征（GBS）
•CIPD的急性表现可以相似
•前6周很难区分
•6-8周后，GBS应改善，而CIDP则会出现慢性炎症
CIDP病例附图：
Fig .1 Coronal and Axial T2WI IDEAL (Water), Coronal T1WI IDEAL (Water) and Coronal DWI. Hyperintensity and thickening of post-ganglionarspinal roots of the lumbosacral plexus from L2 up to S1 in a patient with CIDP. References: RadiolgyDepartment, FLENI, Buenos Aires, Argentina
Fig. 2 Contrast-enhanced T1-weighted MRI studies demonstrating massive hypertrophy of cervical nerve roots causing cervical spinal cord compression (A, B; dotted arrow: spinal cord; white arrows:nerve roots) and major hypertrophy of brachialplexi(C, white arrows). ---Neurology June 16, 2009 vol.
72 no. 24e121
FIig.3 Bilateral ophthalmic nerve hypertrophy and exophthalmos due to chronic inflammatory demyelinating polyradiculoneuropathy. Our patient exhibited bilateral exophthalmos (A, B). Cranial T2-weighted MRI (C) and 3D magnetic resonance cisternography (D) depict abnormal swelling of bilateral supraorbital and supratrochlear nerves, which are branches of ophthalmic nerves in the orbit. Cervical T2-weighted MRI depicts hypertrophic nerve roots (E). ---Neurology April 29, 2014 vol. 82 no. 171566-1567
Fig.4 Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP).Thirty-eight-year-oldwoman diagnosed of CIDP. Coronal T2-weighted fat saturated IDEAL image. (a) Significant bilateral symmetric thickening of lumbosacral plexus roots. (b) Involvement of both sciatic nerves.----Radiologia 2015;57:22-34 - DOI: 10.1016/j.rxeng.2014.07.001
Fig5. Sagittal (A through C) and parasagittal MR slices of the lumbar spine in a patient with chronic inflammatory demyelinating polyradiculoneuropathy. (A) T2-weighted image, showinglackof regular fluid-isointense signal, due to swollen cauda equinafibers(arrows). (B) Corresponding T1-weighted, fat-saturated image. Diffuse cauda equina enhancement (arrows) is depicted, indicating inflammation. (C) Parasagittal T1-weighted, fat-saturated image. Enlarged and enhancing rootfibersare shown, exiting the neuroforamen (arrows).----Neurology February 27, 2007 vol. 68 no. 9701。