特发性正常压力脑积水临床管理中国指南(2022)

步态障碍、认知障碍和膀胱功能障碍临床三联征在老年人群中涉及多种疾病,其中,特发性正常压力脑积水(iNPH)因其早期诊断和早期治疗可有效逆转病情而显得尤为重要。中国微循环学会神经变性病专业委员会脑积水学组联合中华医学会老年医学分会、北京神经变性病学会,组织国内神经内科、神经外科、影像科、精神科、康复科、流行病学等相关领域专家,在2005年版欧美iNPH国际指南,2012年日本iNPH指南(第二版)以及2016年中国iNPH诊治专家共识的基础上,密切结合中国脑积水联盟成立近五年来国内iNPH诊疗实践,并参考2021年日本iNPH第三版指南,总结了国内外iNPH诊疗和研究领域的新证据,共同撰写了本指南。本指南含流行病学、临床特征、诊断和治疗等内容,形成26条推荐意见,作为我国iNPH的诊疗规范,供相关学科临床实践中参考,以便有利于这一危害老年人身心健康的疾病能得到有效管理。     

A case of myoedema and normal pressure hydrocephalus

A cachectic 55-year-old man presented with ataxia and metabolic instability. On physical examination, he had prominent myoedema in all muscle groups. In addition, a CT scan and lumbar puncture showed extensive nonobstructive hydrocephalus. Further clinical evaluation revealed elevated creatine phosphokinase and liver enzyme levels, although the patient was euthyroid. The patient improved neurologically and metabolically with supportive therapy but the myoedema persisted. Previous cases have emphasized that myoedema is a localized, electrically silent, benign myopathic disorder of unknown cause. As with a previous case with ventricular enlargement, myoedema may be part of systemic pathology. Finally, as in most other reports, myoedema is a rare condition; only 3 cases (of 44) with palpable (but not visible) myoedema were uncovered in this study.     

When to consider normal pressure hydrocephalus in the patient with gait disturbance

Normal pressure hydrocephalus (NPH) is a chronic adult disorder of unknown cause. It is characterized by gradual onset of gait impairment, cognitive dysfunction, and urinary incontinence in the presence of enlarged ventricles. NPH is a relatively rare cause of these 3 common symptoms. Diagnosis is made based on suspicion of NPH symptoms, the additional finding of ventriculomegaly on imaging, and confirmatory testing with a trial of CSF drainage, which can predict improvement with CSF shunting. The differential diagnosis must consider common causes of each of the symptoms and include Alzheimer's disease (AD), Parkinson's disease (PD), vascular dementia, and spinal stenosis. Treatment involves CSF diversion, usually through implantation of a shunt from the ventricles to the peritoneal cavity. After surgery and in the absence of other contributing factors, the benefit of surgical intervention can be durable over years.     

老年特发性正常压力脑积水患者的手术治疗及中期随访

目的 评价使用可调压分流管治疗老年人特发性正常压力性脑积水的短期和中期疗效.方法 通过腰穿放液试验和腰大池外引流试验,选择29例患者行脑室腹腔分流手术.利用MMSE评分和10米行走试验对患者进行随访.结果术后短期内患者步态及认知改善明显,中期随访发现症状改善率下降,3例患者分流管堵塞,1例出现硬膜下积液,1例患者死于肺部感染,2例患者随访期间死于心脏疾病.结论 腰穿放液试验和腰大池外引流试验对预测分流术是否有效具有较高的准确性,脑室腹腔引流术可以改善患者的近期症状,手术的中期疗效下降.采用可调压分流管可以提高手术疗效.

Abstract：

Objective To evaluate the short-term and mid-term outcomes in geriatric patients with idiopathic normal pressure hydrocephalus treated with adjustable valve. Methods The 29 patients were selected for the ventriculo-peritoneal shunt by the means of preoperative lumbar tap test and external lumbar drainage test. All patients were assessed by Mini Mental State Examination (MMSE) and timed 10-meter walk test. Results Short-term improvement in gait disturbance and cognitive function could be achieved obviously, and mid-term improvement could not be sustained. The 3 patients had shunt obstruction, 1 had subdural hygroma. 1 case died of pulmonary infection and 2 died of cardiac disease. Conclusions The lumbar tap test and external lumbar drainage test are highly prognostic procedures for identifying patients with idiopathic normal pressure hydrocephalus who mostly likely benefit from shunt surgery. The short-term improvement can be achieved, whereas mid-term improvement can not be sustained. Surgical outcome can be improved by the use of adjustable valve.     

The clinical value of circadian variations of blood pressure

Writing an editorial on the clinical relevance of the diurnal rhythm of blood pressure bears a two-sided risk. There is little doubt that 24 h ambulatory monitoring (ABPM) is the only practical way to investigate the diurnal rhythm of blood pressure and that acceptance of ABPM by physicians and regulatory authorities is largely dependent upon acceptance of the concept that knowledge of blood pressure values during day and night is clinically valuable information. However, underestimation of the many open methodological aspects regarding definition and reproducibility of daytime and night-time blood pressure and emphasis on positive reports showing an association between a blunted day-night blood pressure difference and cardiovascular damage would bear the risk of one being perceived as an uncritical reviewer of a hypothesis fitting with one's personal data [1-6]. At the opposite extreme, the exigency of neutrality could lead one to place excessive emphasis on the less positive or frankly negative evidence in this field (for a review, see [7]). In this issue of the Journal, several groups that are actively involved in clinical research projects with ABPM provide detailed and balanced contributions on several topics related to the diurnal changes in blood pressure.     

临床及肾穿病理参数对狼疮肾炎预后的影响

目的 研究狼疮肾炎（LN）患者的临床、实验室及肾穿病理参数对患者生存率（SR）和肾脏生存率（RSR）的影响。方法 对73例作肾活检的LN患者进行随访,按WHO关于LN的病理分型进行分类。用Kaplan-Maier方法计算SR和RSR。用Cox比例风险模型分析各临床和实验室变量对SR和RSR影响的风险程度。用多变量逐步回归法分析肾穿病理参数对SR和RSR的独立预测价值。结果 73例LN患者5年的SR和RSR分别为95．3％和83．3％,10年为78．8％和65．3％。不同病理组间患者SR和RSR的差异无显著性。多变量回归分析表明是否得到早期诊断和血清肌酐水平对SR和RSR均有明显的影响,而24h尿蛋白定量水平对RSR的影响明显。肾活检病理参数中,RSR的下降与肾脏的慢性病理改变如肾小管的萎缩、肾间质的纤维化、CI＞3以及肾小动脉的损伤均明显相关,而肾小管的萎缩还与SR的降低显著相关。结论 早期诊断对延长LN的SR和RSR起重要作用。临床上血清肌酐和24h尿蛋白定量的水平是影响预后的最重要指标。病理上肾脏的慢性病理改变及肾动脉的损伤是影响生存率的独立危险因素。     

Diagnostic value of clinical and bacteriological findings in pertussis

Clinical and bacteriological findings in the diagnosis of pertussis were evaluated in 300 consecutive patients with parental or the patient's own suspicion of the disease. Serology was used as a reference method. Of the 285 (95%) patients fully sampled, 163 (57%) were diagnosed as having pertussis while the remaining 122 patients constituted the non-pertussis control group. The clinical and epidemiological data were collected at the first visit made on median day seven of illness. In this population of mainly unimmunised children, the highest predictive values were obtained for the physician's diagnosis of pertussis (100%) and for the physician's diagnosis of some other illness (93%). The only clinical symptom with a high predictive value for pertussis was the report of whoops (92%). Among epidemiological data the highest predictive value (90%) was obtained for reported household exposure in unimmunised children more than 1 year of age. Culture of Bordetella pertussis was found to have an overall 50% sensitivity. Isolation of other bacteria had no predictive value in the differential diagnosis of pertussis.     

尿抗原检测法诊断军团菌肺炎的临床价值

目的 探讨尿抗原检测法诊断军团菌肺炎的临床价值.方法 连续入选沈阳3家医院呼吸科病房2011年5月至2013年11月收治的疑似军团菌肺炎患者56例,均进行尿抗原检测,尿抗原阳性病例进行军团菌体外培养、聚合酶链式反应(PCR)及双份血清军团菌抗体检测.结果 尿抗原阳性诊断军团菌肺炎的患者13例,其中1例从下呼吸道分泌物中分离出了军团菌;8例行PCR检测军团菌核酸,4例阳性;12例行双份血清军团菌抗体检测,7例阳性.13例中有环境接触史9例,下呼吸道分泌物呈橘黄色4例,伴消化道症状6例,伴精神神经症状8例,低钠血症10例.氧合指数＜ 300 mmHg(1 mmHg=0.133 kPa) 11例,肺炎严重程度指数(PSI)评分风险分级为Ⅴ级(＞130分)者8例.胸部CT 6例为双侧病变,11例表现为实变合并磨玻璃影,11例合并少量胸腔积液,各有1例患者表现为空洞形成及反晕轮征.13例均应用喹诺酮类药物治疗,11例好转出院,2例因多脏器功能障碍死亡,其中1例死亡患者病程中出现二重感染.结论 尿抗原检测法对军团菌肺炎具有重要的诊断价值;对疑诊病例应关注患者的环境接触史、多脏器受累情况,橘黄色痰可能提供诊断线索;对危重病例应关注二重感染及高浓度吸氧可能造成的肺损伤加重.     

Syndrome of inappropriate secretion of antidiuretic hormone associated with idiopathic normal pressure hydrocephalus.

A 79-year-old woman suffering from urinary incontinence and unsteady gait was diagnosed as having idiopathic normal pressure hydrocephalus (NPH) with hyponatremia due to the syndrome of inappropriate secretion of antidiuretic hormone (SIADH). The concentration of antidiuretic hormone was high while the plasma osmolality was low in the presence of concentrated urine during the episodes of hyponatremia. Magnetic resonance imaging (MRI) of the head showed enlargement of the third and lateral ventricles. After ventriculoperitoneal shunt surgery, the symptoms of NPH and hyponatremia improved. It may be possibly explained that mechanical pressure on the hypothalamus from the third ventricle is responsible for hyponatremia.     

特发性正常压力脑积水颅内体积参数与临床症状关系的研究

目的探讨特发性正常压力脑积水(iNPH)患者术前颅内体积参数与术前临床症状严重程度及术后1年临床症状改善程度的关系。方法收集我院神经外科2016年1月至2017年2月符合国际iNPH指南诊断标准,并行脑脊液分流术的患者21例。所有患者术前行头颅磁共振扫描,并于术前及术后1年门诊复诊采用一致的临床症状指标进行评估,评估内容包括3 m计时起立行走试验(TUG)、简易精神状态量表检查(MMSE)、日本特发性正常压力脑积水分级评分(iNPHGS)与改良Rankin量表(mRS)。测量患者术前头颅影像资料中的脑室体积、脑实质体积、脑室外脑脊液体积、颅内容积,并计算相对脑室体积、脑实质比、脑室外脑脊液比、脑室内外脑脊液比,同时测量所有患者术前的Evans指数。结果iNPH患者术后无论是步态、认知功能还是排尿功能的评分较术前均有所改善(均P<0.05)。患者术前颅内体积参数(相对脑室体积、脑实质比、脑室外脑脊液比、脑室内外脑脊液比)、Evans指数与术前步态、认知功能及排尿功能的严重程度以及术后临床症状改善情况的相关性均无统计学意义(均P>0.05)。在分流术后1年随访点,mRS(17例比4例)、TUG(18例比3例)、MMSE(10例比11例)以及iNPHGS(17例比4例)有改善患者与无改善患者比较颅内体积参数的差异均无统计学意义(均P>0.05)。结论分流手术可以使iNPH患者步态、认知功能及排尿功能障碍等症状得到改善,但iNPH患者术前颅内体积参数(相对脑室体积、脑实质比、脑室外脑脊液比、脑室内外脑脊液比)、Evans指数与术前临床症状严重程度、术后1年症状改善程度无相关性,无法用于预测分流手术是否可以改善特定的临床症状。