Department of Neurology, Sanjay Gandhi PGIMS, Lucknow, India.
Neurol India. 2011 Sep-Oct;59(5):664-8. doi: 10.4103/0028-3886.86537.
The clinical presentation, neurophysiological findings, and outcome may vary between primary and secondary chronic inflammatory demyelinating polyradiculopathy (CIDP).
To compare clinical and electrodiagnostic features of primary and secondary CIDP.
Tertiary care teaching referral hospital.
The CIDP patients who were diagnosed as per European Federation of Neurological Societies/Peripheral Nerve Society criteria were included and subjected to detailed history and examinations. The clinical disability was graded on a 0-10 scale. Neurophysiology included motor and sensory nerve conductions and F wave studies of all four limbs. Based on investigations for underlying diseases, the patients were categorized into primary or secondary CIDP. Prednisolone was prescribed in all and azathioprine added in resistant cases. The secondary CIDP group received specific treatment in addition. The outcome was assessed at 3 months, 6 months, and last follow-up.
A total of 65 patients aged 17 to 72 years were included and 20 were females. Twenty-five patients had secondary CIDP and include diabetes mellitus (16), POEMS (polyneuropathy, organomegaly, endocrinopathy, M protein, and skin changes) (4), monoclonal gammopathy of undetermined significance (2), myeloma (1), lymphoma (1), and malignancy (1). The secondary CIDP patients were older (48.35 vs 41.0 years), had less relapsing remitting (0 vs 6) and more frequent dysautonomia (7 vs 1). The demyelinating features were more marked in primary CIDP group and had better outcome compared with secondary CIDP.
Of the total patients with CIDP, 38.5% of patients had secondary CIDP which was associated with progressive course, less demyelinating features, and worse prognosis.
原发性和继发性慢性炎症性脱髓鞘性多发性神经病（CIDP）的临床表现、神经生理学发现和预后可能不同。
比较原发性和继发性 CIDP 的临床和电诊断特征。
三级保健教学转诊医院。
根据欧洲神经病学会联合会/周围神经学会标准诊断为 CIDP 的患者被纳入并接受详细的病史和检查。临床残疾程度按 0-10 分评定。神经生理学包括四肢的运动和感觉神经传导和 F 波研究。根据潜在疾病的调查结果，将患者分为原发性或继发性 CIDP。所有患者均给予泼尼松龙治疗，耐药患者加用硫唑嘌呤。继发性 CIDP 组除了给予泼尼松龙治疗外，还接受了特异性治疗。在 3 个月、6 个月和最后一次随访时评估结局。
共纳入 65 名年龄在 17 至 72 岁之间的患者，其中 20 名为女性。25 名患者患有继发性 CIDP，包括糖尿病（16 例）、POEMS（多发性神经病、器官肿大、内分泌病、M 蛋白和皮肤改变）（4 例）、意义未明的单克隆丙种球蛋白病（2 例）、骨髓瘤（1 例）、淋巴瘤（1 例）和恶性肿瘤（1 例）。继发性 CIDP 患者年龄较大（48.35 岁 vs 41.0 岁），缓解复发较少（0 例 vs 6 例），自主神经功能障碍更常见（7 例 vs 1 例）。原发性 CIDP 组脱髓鞘特征更明显，与继发性 CIDP 相比，预后更好。
在所有 CIDP 患者中，38.5%的患者患有继发性 CIDP，其特征为进行性病程、较少的脱髓鞘特征和较差的预后。
