老年人慢性炎症性脱髓鞘性多发性神经病临床病理分析

Clinicopathological analysis of chronic inflammatory demyelinating polyneuropathy in the elderly

目的研究老年人慢性炎症性脱髓鞘性多发性神经病（CIDP）的临床和病理特征。方法对11例老年CIDP患者的临床表现、脑脊液检查、肌电图检查及腓肠神经活检病理结果进行总结分析。结果小组患者发病前有上呼吸道感染2例；首发症状为四肢远端麻小无力3例，双下肢远端麻木无力5例，双上肢远端麻木无力2例，抬头困难1例；患者均有运动障碍，伴感觉障碍8例，肢体肌肉萎缩3例，伴有肌肉压痛1例，腱反射减弱或消失8例，颅神经损害5例，自主神经受累3例，累及呼吸肌1例，复发3例；发病高峰期改良Rankin评分平均3．02分；脑脊液检查有明显蛋白细胞分离5例；肌电图示神经原件损害10例，肌原性伴神经原性损害1例；病理检查结果示髓鞘脱失6例，炎性细胞浸润6例，明显髓鞘再生2例，轴索肿胀变性2例；激素治疗8例有效。结论老年CIDP患者首发症状多为肢体远端麻小无力；大部分有感觉障碍；可有颅神经和自主神经损害；腓肠神经活检有助于老年CIDP诊断；激素治疗大部分有效。

Objective To study the clinical and pathological features in the elderly patients with chronic inflammatory demyelinating polyneuropathy （CIDP）. Methods The features of the clinical manifestation, cerebrospinal fluid, electromyogram（EMG） and the biopsy results of sural nerve were presented and analyzed in 11 elderly patients with CIDP, Results Two cases had history of upper respiratory tract infection before the onset. As the initial symptoms , there were three cases with distal limb numbness, five cases with both distal lower extremities numbness, two cases with both distal upper extremities numbness and one case with difficulties to raise his head. Motor disorder was common to all the patients, There were eight patients with sensory dysfunction, three with limb muscle atrophy, one with muscle tenderness, eight with tendon reflexes weakened or disappeared, five with cranial nerve damaged, three with the autonomic nerve lesion, one with respiratory muscle involved, three with relapse, The score of the peak incidence as Modified Rankin was 3, 02 points on average, Five cases had obvious albuminocytolgoic dissociation by the examination of cerebrospinal fluid,ten cases had neurogenic damage and one case had a combined myogenic and neurogenic damage by the EMG. The biopsy showed that six cases were with amyelination,six cases with inflammatory cell infiltrated, two eases with obvious remyelination, two cases with auxiliary fibers degeneration, And the methylprednisolone therapy was effective for eight cases, Conclusions The numbness of the distal limb is the initial symptom of the elderly patients with CIDP,most of whom are with sensory dysfunction ,and some with cranial nerves and autonomic nerve damage, The sural nerve biopsy has an important value for the diagnosis of the elderly with CIDP. The methylprednisolone therapy is proved to be effective for most patients.