神经梅毒延迟诊断一例临床分析

目的探讨麻痹性痴呆的临床特征、诊断方法及延迟诊断原因。方法结合1例麻痹性痴呆患者的临床资料进行讨论,阐述新时期神经梅毒的隐蔽性和造成延误诊断的原因。结果麻痹性痴呆中年人发病较多;起病隐匿,进行性加重,主要表现为认知障碍、精神症状、癫疒间发作等;梅毒螺旋体明胶凝集试验(TPPA)阳性;头颅MRI无特征性改变;通过驱梅治疗麻痹性痴呆患者MOCA评分提高。结论神经梅毒临床表现复杂,早期误诊率高;建议梅毒血清抗体检测应作为青壮年卒中或痴呆患者的常规检查项目;麻痹性痴呆是一种可治的痴呆,应及早诊断及早治疗。     

曾被误诊的麻痹性痴呆23例分析

目的:研究麻痹性痴呆的诊断与治疗。方法:对曾被误诊的23例麻痹性痴呆,使用简明精神病评定量表(BPRS)及简易精神状态检查(MMSE)评定。并予驱梅毒及抗精神病药治疗。结果:驱梅毒治疗后可显著提高疗效。结论:麻痹性痴呆应及早正确诊断,作驱梅毒及抗精神病治疗。     

麻痹性痴呆14例临床特征

目的探讨麻痹性痴呆(GPI)的临床类型、临床表现。方法分析14例麻痹性痴呆患者的临床资料。结果 14例麻痹性痴呆主要表现为精神症状和神经系统症状,门诊与入院诊断的误诊率高达100%,住院后均经实验室检查梅毒螺旋体凝集试验(TPPA)阳性而确诊。结论麻痹性痴呆易被误诊,诊断依据临床流行病学资料、临床表现、血清和脑脊液梅毒抗体检测阳性可确诊。作为一种可治性痴呆,早期的预防和观察识别有着非常重要的意义。     

麻痹性痴呆的临床及影像特点

目的:分析麻痹性痴呆(GPI)的临床及影像特征及提供早期诊断依据。方法:回顾性分析经临床及实验室确诊的1例麻痹性痴呆患者的有关临床资料。结果:麻痹性痴呆早期以认知及精神症状为主要表现,影像上主要为脑室扩大,实验室检查有特征性改变。结论:麻痹性痴呆早期临床容易误诊,应重视梅毒血清和脑脊液抗体的检查,结合CT、 MRI可以诊断,大剂量足疗程青霉素治疗能取得显著疗效。     

麻痹性痴呆25例临床分析

目的研究麻痹性痴呆的特征;探讨治疗及预后。方法回顾性分析25例麻痹性痴呆患者的临床资料,智能的检测采用简易智力状态检查量表(MMSE)评分,入选条件是MMSE≤20分。结果25例患者中有4例合并卒中发作,11例合并癫发作,1例合并脊髓功能障碍,1例合并视神经功能障碍;所有患者血液梅毒螺旋体血凝试验(TPHA)阳性,脑脊液快速血浆反应素环状试验(RPR)或性病研究实验室试验(VDRL)均为阳性;25例脑脊液蛋白定性为+~++,18例脑脊液白细胞数>5×106/L;11例脑电图中~重度异常,表现为广泛性中~高波幅θ波或伴有阵发性单个小尖波;15例CT示脑萎缩,2例合并脑积水,4例CT示放射冠、基底节、额叶、顶叶腔隙性脑梗死,其中1例并有右顶枕叶脑回肿胀,1例脑室周围大量脱髓鞘病变;误诊及漏诊率47%;所有患者经青霉素驱梅治疗后临床症状均有明显好转或好转,出院时13例血液及脑脊液TPHA滴度下降,脑脊液白细胞数下降或消失,12例血液及脑脊液TPHA滴度无改变,脑脊液RPR或VDRL仍为阳性。结论麻痹性痴呆常合并其他神经系统症状及体征,极易误诊,足剂量、足疗程的青霉素治疗是预后的关键。     

神经梅毒四例误诊分析并文献复习

目的 探讨神经梅毒诊断要点,提高临床医师对该病的认识,降低误诊和漏诊率.方法 报告4 例神经梅毒患者临床诊断与治疗经过,结合文献就其临床表现、分型及诊断要点进行回顾分析.结果 4 例最终诊断为脑膜血管梅毒合并麻痹性痴呆、麻痹性痴呆、脊髓痨和梅毒性树胶肿患者,分别被误诊为脑梗死、阿尔茨海默病、多系统萎缩和椎管内肿瘤.检索万方数据库2000 年至今收录的中华医学会系列学术期刊关于神经梅毒的文献共计29 篇,132 例患者,男106 例,女26 例,男女比例约4.08∶1;发病年龄1 ～ 75 岁,平均(44.09 ± 12.93)岁.其中28 例(21.21%)有梅毒感染史,55 例(41.67%)有冶游史,35 例(26.52%)曾被漏诊或误诊.临床分型包括无症状性神经梅毒1 例(0.76%),脑(脊)膜梅毒14 例(10.61%),脑(脊)膜血管梅毒43 例(32.58%),麻痹性痴呆44 例(33.33%),脊髓痨13 例(9.85%),梅毒性树胶肿15 例(11.36%),特殊表现梅毒2 例(1.51%).结论 神经梅毒临床表现复杂多样,误诊和漏诊率高,临床诊断应结合患者临床特征、梅毒血清学检测阳性、脑脊液细胞计数和蛋白定量升高、脑脊液美国性病研究实验室试验呈阳性反应、影像学检查及手术后病理检查等多项检查综合考虑.     

以痴呆为主要表现的麻痹性痴呆、HIV相关性痴呆和克雅病的临床特征分析

目的探讨并分析梅毒所致麻痹性痴呆(麻痹性痴呆)、HIV相关性痴呆和克雅病等中枢系统感染性疾病所致痴呆的临床特征。方法检索19例患者(麻痹性痴呆8例、HIV相关性痴呆6例、克雅病5例)临床资料,回顾分析其临床表现、实验室检查、脑电图、神经影像学及治疗转归特点。结果 3组患者临床表现均以认知损害为主,并广泛累及多系统(锥体系、锥体外系、小脑)及多组脑神经。辅助检查显示,麻痹性痴呆患者快速血浆反应素环状卡片试验和苍白密螺旋体抗体明胶颗粒凝集试验阳性(8例),脑脊液美国性病研究实验室试验阳性(4例),MRI呈现不同程度脑萎缩(6例);HIV相关性痴呆患者血清HIV抗体筛选试验及Western blotting检测阳性(6例),脑脊液平均蛋白定量明显升高(2例)、潘氏试验阳性(2例),MRI以脑内多发占位病变或大片异常密度影为特征;克雅病患者脑脊液Western blotting检测1433蛋白阳性(4例),脑电波呈弥漫性慢波(4例)伴典型三相波(1例),散发型患者MRI脑叶皮质区沟、回呈肿胀样改变(3例),变异型患者可伴丘脑"曲棍球样"改变(1例)。结论麻痹性痴呆、HIV相关性痴呆及克雅病等中枢系统感染性疾病所致痴呆临床表现复杂多样,诊断时应结合患者病史、实验室血清学和脑脊液指标,以及脑电图和神经影像学表现等综合考虑,明确诊断。     

麻痹性痴呆二例的临床特征与诊断

目的分析麻痹性痴呆(GPI)的临床特征及提供早期诊断依据。方法回顾性分析经临床和实验室检查确诊的2例麻痹性痴呆患者的有关临床资料。结果GPI的临床特征(1) 2例患者慢性起病,进行性发展; (2)痴呆是核心症状, 2例患者入院时长谷川痴呆量表评分为4分和17分,均伴夸大妄想、病理性欣快等精神症状; (3) 1例患者的瞳孔左右不等大,光反射迟钝或消失但辐辏反射保持, 2例患者均有构音障碍、肌张力增高及反射异常等; (4) 2例患者血清、脑脊液梅毒抗体反应均为阳性,脑脊液蛋白含量增加、细胞数增多(以淋巴细胞为主); (5)头颅MRI示2例患者均有脑萎缩, 1例患者有脑实质异常信号影,为多发、散在病灶,广泛脑白质纤维变性。结论GPI误诊率高,临床表现、实验室及影像学检查是诊断的重要依据。     

麻痹性痴呆临床特征分析

目的分析麻痹性痴呆的临床特征。方法回顾性分析13例麻痹性痴呆患者的临床表现、辅助检查及治疗。结果本组患者以认知及精神症状为主要表现;简易智能状态检查量表(MMSE)评分平均12±3分;所有患者血清和脑脊液TRUST及TPPA试验阳性,部分患者脑脊液白细胞数和蛋白含量增高;头颅MRI示脑萎缩;12例患者经大剂量青霉素治疗后有好转。结论麻痹性痴呆容易误诊;大剂量青霉素治疗有效。     

额颞叶痴呆患者的临床特征与早期诊断

目的:探讨额颞叶痴呆患者的临床特征,为早期诊断提供依据. 方法:回顾性分析16例额颞叶痴呆患者的临床特点. 结果:额颞叶痴呆早期的首发症状以人格改变(75％)、精神行为症状(12.5％)、语言障碍(25％)或(伴)记忆力下降(50％)等为主.伴随认知功能的逐步下降,患者会逐渐出现各种非特异的症状表现如淡漠、失眠、注意力不集中、异常或刻板行为、易激惹、口欲亢进、脱抑制及收藏癖.额颞叶痴呆的首次诊断符合率总体在50％左右. 结论:额颞叶痴呆患者症状丰富且无特异性,早期诊断需要综合考虑,特别要警惕痴呆患者的首发症状.     

Post‐traumatic stress disorder mistaken for behavioural and psychological symptoms of dementia: case series and recommendations of care

In late life, traumas may act cumulatively to exacerbate vulnerability to post‐traumatic stress disorder (PTSD). PTSD is also a risk factor for cognitive decline. Major neurocognitive disorder (MND) can be associated with worsening of already controlled PTSD symptoms, late‐life resurgence or de novo emergence. Misidentifying PTSD symptoms in MND can have negative consequences for the patient and families. We review the literature pertaining to PTSD and dementia and describe five cases referred for consultation in geriatric psychiatry initially for behavioural and psychological symptoms of dementia (BPSD), which were eventually diagnosed and treated as PTSD in MND subjects. We propose that certain PTSD symptoms in patients with MND are misinterpreted as BPSD and therefore, not properly addressed. For example, flashbacks could be interpreted as hallucinations, hypervigilance as paranoia, nightmares as sleep disturbances, and hyperreactivity as agitation/aggression. We suggest that better identification of PTSD symptoms in MND is needed. We propose specific recommendations for care, namely: clarifying diagnosis by distinguishing PTSD symptoms coexisting with different types of dementia from a specific dementia symptom (BPSD), gathering a detailed history of the trauma in order to personalise non‐pharmacological interventions, adapting psychotherapeutic strategies to patients with dementia, using selective serotonin reuptake inhibitors as first‐line treatment and avoiding antipsychotics and benzodiazepines. Proper identification of PTSD symptoms in patients with MND is essential and allows a more tailored and efficient treatment, with decrease in inappropriate use of physical and chemical restraints.     

麻痹性痴呆误诊的原因分析

目的探讨麻痹性痴呆易被误诊的可能原因,提高临床诊断率。方法回顾性分析14例曾被误诊的麻痹性痴呆患者的临床资料。结果以不典型的精神症状群首发而被误诊为功能性精神障碍10例,其中误诊例次率最高的是精神分裂症占47．3％,其次是躁狂症占31．6％;以进行性加重的痴呆为主要首发症状而被误诊为阿尔兹海默病的2例及血管性痴呆1例;以酒依赖伴有痴呆样症状为主被误诊为酒精所致精神障碍的1例。〈40岁的占总数的28．5％。否认或故意隐瞒治疗史9例,根本不知情5例。误诊时处于发病早期无神经系统的阳性体征8例;处中晚期有神经系统阳性体征且呈不同程度表现的共6例,其中仅1例伴有特异性阿-罗氏瞳孔特征。14例辅助检查均不够完善,均未及时进行相关梅毒血清学检查。结论麻痹性痴呆易被误诊的首要原因是首发精神症状不典型,其次是冶游史易被隐瞒、早期神经系统阳性体征不明显或中晚期缺乏阿-罗氏瞳孔特异性体征、辅助检查不够完善和及时。     

A 10-year study of the incidence of and factors predicting dementia in Parkinson's disease

OBJECTIVE: To compare the incidence of dementia in PD with that of a control group without PD, and to assess the relationship between dementia and other features of PD. METHODS: The authors recruited 83 patients with PD and 50 controls, all without dementia at initial assessment, and assessed them at regular intervals over a maximum period of 122 months. Dementia was diagnosed according to objective criteria, and included a judgment by researchers masked to subject group and to variables putatively associated with dementia. RESULTS: Seventeen patients fulfilled dementia criteria; no controls did so. The cumulative proportion of PD patients becoming demented by 112 months was 0.38 (95% CI 0.20 to 0.55), or 42.6 cases per 1000 years of observation. Univariate analyses showed that incident dementia in patients with PD was associated with older age at entry into the study, greater severity of neurologic symptoms, longer duration of PD, greater disability, and male sex. The association of age at onset of PD with incident dementia was of only borderline significance. Multivariate analysis found that age at entry into the study and severity of motor symptoms were significant predictors of dementia but duration of PD and age at onset of PD were not. CONCLUSIONS: Dementia in PD is likely to reflect interaction of the neuropathology of the basal ganglia and age-related pathology. The findings do not support the division of PD into early and late-onset cases.     

Initial symptoms, survival and causes of death in 115 patients with frontotemporal lobar degeneration

The early and differential diagnosis of the clinical phenotypes of frontotemporal lobar degeneration (FTLD), including frontotemporal dementia (FTD), semantic dementia (SD) and non-fluent progressive aphasia (NFPA), can be challenging. It may be difficult not only to differentiate these conditions from normal aging, psychiatric disorders, and other dementias, but also to distinguish between them. For early diagnosis, information on the initial and presenting symptoms of the FTLD phenotypes is essential. In the present study caregivers of 78 patients with FTD, 20 patients with SD and 17 patients with PA were interviewed about initial symptoms. In patients with FTD, the most frequent initial symptoms were alterations of personality, followed by forgetfulness and word finding difficulty. Patients with SD presented with word finding difficulty and behavioral disturbances. Almost all patients with PA developed word finding difficulty as the first manifestation of their disorder. Diagnostic latency - the time from disease onset to diagnosis was 4.1 years in FTD, 4.2 years in SD and 3.1 years in PA. Caregivers, and in some cases also patients, should be educated about the likely course and mortality of FTLD. To obtain information about survival time and cause of death associated with FTLD we analyzed follow-up data on 106 patients of whom 25 had died. The median survival time from the occurrence of first symptoms was 14 years. Mortality risk was significantly higher in patients with an early disease onset. Causes of death were varied, but pneumonia and sudden unexplained deaths were particularly frequent.     

皮质下缺血性抑郁和皮质下缺血性痴呆患者的抑郁症状分析

目的 比较皮质下缺血性抑郁和皮质下缺血性痴呆患者的抑郁症状。方法 连续收集2008年9月至2011年3月间符合入选标准的70例皮质下缺血性血管病（subcortical ischemic vascular disease,SIVD）患者,根据抑郁和痴呆的诊断标准及17项版汉密尔顿抑郁量表（Hamilton Rating Scale of Depression,HRSD）和简易智能状态检查（Mini-Mental State Examination,MMSE）分为无抑郁或痴呆组（29例）、抑郁组（19例）、痴呆组（12例）、抑郁伴痴呆组（10例）。应用HRSD对各组患者的抑郁症状进行评价,分析比较各组患者的抑郁症状的表现特点。结果 4组患者的年龄、性别比、社会经济因素及血管性危险因素的差异无统计学意义。就发生率,抑郁组以抑郁情绪（17例,89.5%）、精神性焦虑（16例,84.2%）、躯体性焦虑（15例,78.9%）最突出,而痴呆组和抑郁伴痴呆组以阻滞（8例,66.6%和9例,90.0%）最突出。就严重程度,除睡眠障碍外,抑郁组以焦虑躯体化症状[0.8（0.2,2）]最严重,而痴呆组和抑郁伴痴呆组以阻滞症状［1（0.3,1.8）和1.1（0.3,2）］最严重。抑郁组的抑郁程度较抑郁伴痴呆组更高（P=0.026）、焦虑躯体化更突出（P<0.01）。结论 皮质下缺血性抑郁和皮质下缺血性痴呆及两者共存的患者的情绪损害特点存在着差异,皮质下缺血性抑郁较偏向于经典抑郁症所致情绪障碍,皮质下缺血性痴呆和皮质下缺血性抑郁伴皮质下缺血性痴呆的患者则较偏向于皮质下缺血性病变所致情绪障碍。     

Diogenes syndrome in patients suffering from dementia

Diogenes syndrome (DS) is a behavioral disorder of the elderly. Symptoms include living in extreme squalor, a neglected physical state, and unhygienic conditions. This is accompanied by a self-imposed isolation, the refusal of external help, and a tendency to accumulate unusual objects. To explore the phenomenon of DS in dementia we searched for the terms: “Diogenes syndrome, self-neglect, dementia. ” It has long been understood that individuals with dementia often become shut-ins, living in squalor, in the Eastern Baltimore study, dementia was present in 15% of the elderly cases with moderate and severe social breakdown syndrome; twice as many as in the general population of the same age group. Researchers have underlined the frequent presence of DS (36%) in frontotemporal dementia (FTD): different neuropsychological modifications in FTD may contribute to symptoms of DS. The initial treatment should be a behavioral program, but there is not sufficient information regarding pharmacological treatment of the syndrome.     

The function of psychiatric hospitals in the treatment of dementia

In Japan, there are 531 special wards for the treatment of dementia in which patients with behavioural and psychological symptoms of dementia are treated. In these wards, 85% of the patients stay for more than 91 days, and 45% of them are judged as ready to discharge. The function of the wards declines as a result of the long‐stay patients. It is necessary to create two types of wards: one for acute treatment of behavioural and psychological symptoms of dementia within 90 days and another for patients requiring longer‐term treatment (i.e. more than 91 days) for chronic behavioural and psychological symptoms of dementia, deterioration of activity of daily living and somatic complications     

Clinical early symptoms and CT findings in Fahr syndrome

We present the results of an investigation on initial symptomatology of patients with bilateral, symmetrical intracerebral calcification of the basal ganglia (Fahr's syndrome). 62 patients, who because of various neurological or psychiatric symptoms or other reasons were referred to cranial computer-tomography, revealed clear manifestation of Fahr's syndrome. In these cases estimations of the volume of the opaque bodies were made, based on computer-integrated programmes (SO-MATOM D2). In 9 cases there was a history of thyroidectomy, on average 25 years previously. As a rule patients were referred in the age range between 40 to 60 years, those who had undergone thyroidectomy being on average somewhat older than the others. As presenting symptoms extrapyramidal syndromes, apoplexias, cephalea, affective organic alterations, alcoholism and dementia were noted. Cerebral localisation of bilateral, symmetrical calcification was most frequent in the pallidum, though this localisation may be responsible for numerous different neuropsychiatric symptoms. The dimensions of the opaque structures can be very greatly. Symptomatology seems to be practically independent of either cerebral localisation or volume, except that greater volumes of calcification seem to cause more pronounced neurological (extrapyramidal) symptomatology and dementia. The time-span between manifestation of the initial symptoms and diagnosis was shorter in neurological syndromes than in psychiatric cases. Like earlier workers, we found a high incidence (21%) of organic affective syndromes as an initial manifestation of Fahr's syndrome.