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目的探讨特发性正常压力脑积水(i NPH)的临床特点及误诊原因。方法回顾性分析2011年3月~2013年11月在本院神经内科确诊的50例i NPH患者的临床资料和误诊情况。结果 (1)临床特点:50例患者中男31例(62%),女19例(38%);年龄60~89岁,平均年龄(76.4±6.94)岁;病程9个月~10年,平均病程(3.78±1.98)年;三联征中有步态异常50例(100%),认知功能障碍48例(96%),排尿障碍33例(66%),具备三联征者32例(64%);常伴有运动减少、动作迟缓、淡漠、肢体震颤、幻觉、妄想等表现;以步态改变起病34例(68%),以记忆力下降起病16例(32%);(2)辅助检查:头颅MRI/CT显示脑室扩大,Evans指数均>0.3,平均Evans指数为(0.33±0.14);MRI上呈蛛网膜下腔不成比例扩展性脑积水(DESH)征象19例(38%),胼胝体后部接合处夹角平均(88.5±20.3)度。Tap试验后步态异常均有不同程度的改善;(3)治疗与随访情况:9例(18%)接受脑室-腹腔分流手术,有1例术后因脑出血死亡,其余3月后步态异常明显改善。41例(82%)未手术患者予药物治疗,3月后病情稳定28例,症状加重11例,死亡2例(肺炎1例、脑血管事件1例),症状好转0例;(4)误诊情况:首诊误诊率高达66%(33/50),误诊为帕金森病14例(28%)、血管性痴呆13例(26%),阿尔茨海默病4例(8%),路易体痴呆1例,Wernicke脑病1例;误诊2次以上9例,仍以帕金森病、血管性痴呆常见。结论早期诊断i NPH并非易事,多数患者因误诊而错失手术最佳时机。临床表现与影像学检查是诊断i NPH重要依据。脑脊液动力学检查帮助准确诊断,其中Tap试验简便且安全。及时行脑脊液分流术可获得较好的疗效。
Objective To investigate the clinical features and causes of misdiagnosis of idiopathic normal pressure hydrocephalus (i NPH). Methods The clinical data and misdiagnosis of 50 patients with i NPH diagnosed in our department from March 2011 to November 2013 were retrospectively analyzed. Results (1) Clinical features: Among 50 patients, 31 (62%) were male and 19 (38%) were female. The average age was (76.4 ± 6.94) years old from 60 to 89 years old. The course of disease ranged from 9 months to 10 years The average course of disease was 3.78 ± 1.98 years. There were 50 cases of gait abnormalities (100%), 48 cases of cognitive dysfunction (96%), 33 cases of urinary disorders (66%), 32 cases of triadic symptoms 64%); often accompanied by decreased exercise, slow movement, indifference, limb tremor, hallucinations, delusions and other performance; gait change in onset of 34 cases (68%), memory decline in 16 cases (32% 2) Auxiliary examination: the head MRI / CT showed ventricular enlargement, Evans index were> 0.3, the average Evans index was (0.33 ± 0.14); MRI showed subarachnoid scalability of the signs of dehydration (DESH) in 19 cases 38%). The posterior junction of the corpus callosum had an average angle of (88.5 ± 20.3) degrees. (3) The treatment and follow-up situation: 9 cases (18%) underwent ventricle-peritoneal shunt surgery, 1 case died of cerebral hemorrhage after surgery, and the other 3 months after the gait Abnormal significantly improved. Forty-one patients (82%) underwent non-surgical treatment and 28 patients were stable after 3 months. Symptoms were aggravated in 11 patients and 2 died (1 pneumonia and 1 cerebrovascular event). The symptoms were improved in 0 patients. (4) Misdiagnosis Case: Misdiagnosed as Parkinson’s disease in 14 cases (28%), vascular dementia in 13 cases (26%), Alzheimer’s disease in 4 cases (8%), Louis 1 case of dementia, 1 case of Wernicke’s encephalopathy, 9 cases of misdiagnosis more than 2 times, still common in Parkinson’s disease and vascular dementia. Conclusion Early diagnosis of i NPH is not an easy task. Most patients miss the best time for surgery because of misdiagnosis. Clinical manifestations and imaging examination is an important basis for the diagnosis of i NPH. Cerebrospinal fluid kinetic tests help diagnose accurately, with the Tap test being easy and safe. Time line cerebrospinal fluid shunt can get better effect.