Demenz mit Lewy-Körperchen

Dementia with Lewy Bodies (DLB) is the second most common form of dementia in the elderly. Core features of the DLB are fluctuating cognitive symptoms, visual hallucinations and spontaneous parkinsonism. The clinical diagnostic criteria are very useful in the differentiation between DLB and Alzheimer's disease. The deficits in cholinergic neurotransmission are pronounced and associated with cognitive and psychotic symptoms. An 83 years old patient with DLB showed well formed recurrent visual hallucinations and fluctuating cognition and attention. There was no response to treatment with atypical neuroleptics. The patient responded within few days to treatment with Donepezil. Both cognitive and behavioural symptoms were improved significantly.     

Visual symptoms in Parkinson's disease and Parkinson's disease dementia

Visual symptoms are common in PD and PD dementia and include difficulty reading, double vision, illusions, feelings of presence and passage, and complex visual hallucinations. Despite the established prognostic implications of complex visual hallucinations, the interaction between cognitive decline, visual impairment, and other visual symptoms remains poorly understood. Our aim was to characterize the spectrum of visual symptomatology in PD and examine clinical predictors for their occurrence. Sixty-four subjects with PD, 26 with PD dementia, and 32 age-matched controls were assessed for visual symptoms, cognitive impairment, and ocular pathology. Complex visual hallucinations were common in PD (17%) and PD dementia (89%). Dementia subjects reported illusions (65%) and presence (62%) more frequently than PD or control subjects, but the frequency of passage hallucinations in PD and PD dementia groups was equivalent (48% versus 69%, respectively; P = 0.102). Visual acuity and contrast sensitivity was impaired in parkinsonian subjects, with disease severity and age emerging as the key predictors. Regression analysis identified a variety of factors independently predictive of complex visual hallucinations (e.g., dementia, visual acuity, and depression), illusions (e.g., excessive daytime somnolence and disease severity), and presence (e.g., rapid eye movement sleep behavior disorder and excessive daytime somnolence). Our results demonstrate that different "hallucinatory" experiences in PD do not necessarily share common disease predictors and may, therefore, be driven by different pathophysiological mechanisms. If confirmed, such a finding will have important implications for future studies of visual symptoms and cognitive decline in PD and PD dementia.     

Hallucinations, cognitive decline, and death in Alzheimer's disease

The relation of psychotic symptoms to cognitive decline and mortality in Alzheimer's disease (AD) was examined during a mean of 2.2 years in 478 persons selected from clinical settings. Psychotic symptoms were ascertained at baseline and cognition was assessed semiannually with nine tests from which a global measure was formed. In analyses that controlled for age, sex, race, and education, hallucinations (29.6%), especially visual ones, were associated with more rapid global cognitive decline and increased mortality, even after controlling for baseline level of cognition and use of antipsychotic medication, and the association with mortality increased with higher level of education. Delusions and misperceptions were not strongly related to cognitive decline or mortality. The results suggest that hallucinations in Alzheimer's disease, particularly visual ones, are associated with more rapid progression.     

Prospective study of hallucinations and delusions in Parkinson's disease

OBJECTIVE: This study was undertaken to determine the prevalence of hallucinations and delusions in Parkinson's disease, to describe such symptoms phenomenologically, and possibly to determine factors associated with their development. In addition, the role of the visual system in relation to visual hallucinations was examined. METHOD: 102 consecutive patients diagnosed with strictly defined Parkinson's disease were examined for the presence of hallucinations and delusions and assessed for visual acuity, cognition, depression using the geriatric depression scale, disease severity as measured by the UPDRS, and other clinical variables. RESULTS: Of 102 consecutive patients, 29.4% (n=30) had hallucinations or delusions, four (3.9%) were determined to be psychotic due solely to delirium and were excluded from further analysis. Of the 98 remaining patients, 26.5% (n=26) had visual hallucinations. Among these, one patient also had delusions, two had auditory hallucinations, and one had gustatory hallucinations. Visual hallucinations were significantly associated with worse visual acuity, lower cognitive score, higher depression score, and worse disease severity. Hallucinations were not associated with history of psychiatric disease, dose or duration of levodopa or other antiparkinsonian medication treatment, or duration of illness. CONCLUSIONS: Visual hallucinations are common symptoms in Parkinson's disease and are most likely of multifactorial origin. Although higher doses of levodopa are known to be related clinically to hallucinations in individual patients, the results suggest that several underlying characteristics of patients with Parkinson's disease (disease severity, dementia, depression, worse visual acuity) may be more important determinants of which patients experience hallucinations. The data also provide preliminary evidence that abnormality of the visual system may be related to visual hallucinations in Parkinson's disease, as has been found in other disorders with visual hallucinations.     

Neuropsychological correlates of reality distortion in schizophrenic patients

The present study aims at exploring the relationship between content-related aspects of delusions and hallucinations in schizophrenia and the basic domains of cognition, controlling for the other clinical and demographic variables that could produce bias in the interpretation of the results. Seventy stable schizophrenic patients were evaluated through psychiatric assessment and a neuropsychological battery including tests on attention, memory, perceptual-motor speed and executive functions. We found that the severity of negative symptoms was strongly correlated with poor performance in almost all domains of cognitive functions, while only the attentional deficit was correlated with positive symptoms. The relationships between different cognitive domains and specific types of delusions and hallucinations showed that thought insertion, guilt, grandiose, religious and somatic delusions were associated with impairment in different cognitive functions (verbal and visual memory, attention and executive functions). Voices arguing and tactile hallucinations were correlated to delay-recall memory function. Our results suggest that no specific cognitive pattern is associated with typical-content delusions and hallucinations. On the basis of our findings, cognitive impairments associated with delusions and hallucinations, as measured by our battery, seem not to play a central role in the genesis and the maintenance of these symptoms, suggesting a more complex model of pathogenesis.     

帕金森病视幻觉及相关因素临床分析

目的：研究视幻觉在帕金森病（PD）患者中的发生率、临床表现特点及可能的相关因素。方法：选取神经内科门诊连续175例原发性PD患者为研究对象。对有幻觉的PD患者采用改良幻觉调查表问询调查,并对视幻觉出现的频率、严重度及夜间病情等进行评分。分析群体中视幻觉的发生率,通过单因素分析及Logistic回归分析等统计学方法对PD合并视幻觉的相关因素予以分析。结果：175例PD患者中无视幻觉126例（无视幻觉PD组）。存在视幻觉49例（有视幻觉PD组）,发生率为28．0％。其中,男性发生率为28．7％（31／108例）,女性发生率为26．9％（18／67例）。49例PD视幻觉患者中,18例（36．7％）为轻度幻觉／错觉,31例（63．3％）为复杂型视幻觉。单因素分析显示：轻度幻觉／错觉组和复杂型视幻觉组间的累计评分差异有统计学意义（P〈0．05）。单因素分析结果表明视幻觉组与无视幻觉组间在认知评分、病程、年龄、是否服用左旋多巴及剂量、是否服用普拉克索的差异有统计学意义（P〈0．05）;Logistic回归分析显示认知损害、年龄和病程与视幻觉有密切关系,是视幻觉发生的危险因素。结论：PD患者的视幻觉主要表现为复杂型视幻觉,可与其他精神症状伴随;其发生与认知损害、年龄和病程密切相关;其病因和发生机制需要进一步研究。     

Differential changes in visual and auditory event-related oscillations in dementia with Lewy bodies

ObjectiveAside from the cognitive impairment, patients with dementia with Lewy bodies (DLB) have a high frequency of visual hallucinations and a number of other vision-related symptoms, whereas auditory hallucinations are less frequent. To better understand the differential dysfunction of the visual network in DLB, we compared auditory and visual event-related potentials and oscillations in patients with DLB.MethodsEvent-related potentials elicited by visual and auditory oddball tasks were recorded in 23 patients with DLB and 22 healthy controls and analyzed in time and time-frequency domain.ResultsDLB patients had decreased theta band activity related to both early sensory and later cognitive processing in the visual, but not in the auditory task. Patients had lower delta and higher alpha and beta bands power related to later cognitive processing in both auditory and visual tasks.ConclusionsIn DLB visual event-related oscillations are characterized by a decrease in theta and lack of inhibition in alpha bands.SignificanceDecreased theta and a lack of inhibition in alpha band power might be an oscillatory underpinning of some classical DLB symptoms such as fluctuations in attention and high-level visual disturbances and a potential marker of dysfunction of the visual system in DLB.     

Visual hallucinations in patients from an ophthalmology clinic and medical clinic population

Although visual hallucinations have been associated with patients with visual disorders, no study has specifically examined whether visual hallucinations are indeed more prevalent than in a general medical population. In this study, 127 consecutive visual disorder patients and 100 consecutive general medical patients were screened for complex visual hallucinations. A total of 6.3% of visual disorder patients and 2% of general medical patients had visual hallucinations. Interestingly, the two medical patients with visual hallucinations also had visual disorders. Factors significantly associated with visual hallucinations were female sex (p = .029) and lower cognitive score (p = .001). Data from a previous study of patients with the visual disorder age-related macular degeneration were combined with this study to increase the sample size of visual hallucinators. Factors significantly associated with visual hallucinations in the combined sample were female sex (p = .015), living alone (p = .019), having hearing problems (p = .047), older age (p = .013), and lower cognitive score (p < .001). Implications and future research are discussed.     

Classification of psychotic symptoms in dementia sufferers

Little attention has been payed to the classification of psychotic symptoms in dementia sufferers. This article compares the etiology of delusions, visual hallucinations and delusional misidentification and examines the value of factors generated from principal components analysis as a possible classificatory system in a group of 125 patients with DSM-III-R dementia in contact with clinical services who were prospectively evaluated using standardized instruments to describe in detail individual psychotic symptoms. The assessment also included the Geriatric Mental State Schedule, the History and Aetiology Schedule and the CAMCOG. Delusions and visual hallucinations had a distinct cognitive profile as did delusions and delusional misidentification, although there was an overlap between visual hallucinations and delusional misidentification. Four factors were generated from principal components analysis. Three of these closely mirrored the 3 symptom groups delusions, visual hallucinations and delusional misidentification, although the phantom-boarder delusion was correlated with the visual hallucination factor and not delusional misidentification. The fourth factor included visual hallucinations of relatives and delusions that relatives were in the house. This factor was strongly inversely associated with emotional distress and could perhaps best be seen as a comfort phenomena. The pattern of cognitive deficits and etiological associations of each of the factors were independent of one another, supporting the notion that it is useful to consider them as separate entities.     

Charles Bonnet hallucinations: a case series.

Charles Bonnet hallucinations are complex visual hallucinations which occur during clear consciousness in patients who do not suffer from psychosis, substance abuse, sleep disorders, focal neurological lesions or acute eye disease. They are well-defined images of patterns, scenes, animals or humans combined with normal perceptions and elicit a neutral or pleasant emotional response. Because of conflicting reports about cognitive or visual impairments among patients with these hallucinations, I describe the cognitive and visual status of 13 patients. One patient had preserved cognition and vision; three had preserved cognition and impaired vision; three had impaired cognition and preserved vision; and six had both impaired cognition and vision. It is unlikely that cognitive or visual deficits alone caused the hallucinations, but may have contributed to a state of sensory deprivation with visual phenomena.     

Are hallucinations and extrapyramidal signs associated with a steeper cognitive decline in degenerative dementia patients?

The aim of this study was to verify if visual hallucinations and extrapyramidal signs are associated with a high speed of cognitive decline in degenerative dementia patients with amnesic onset. The analysis of 1082 consecutive patients showed that hallucinations, but not extrapyramidal signs, were associated with a faster cognitive decline. However, patients with hallucinations were also more severe at the time of examination with the Milan Overall Dementia Assessment (MODA). One interpretation is that hallucinations are the marker of a distinct process of nervous degeneration that specifically causes a steeper cognitive decline from onset. Another interpretation is that the speed of cognitive decline is set along a continuum, and the incidence of hallucinations is simply proportional to the level of cognitive decline. The statistical analysis of our data was consistent with the latter interpretation, in that we were able to quantify the probability of presenting visual hallucinations on the basis of the overall severity of cognitive decline. Authors’ names are listed in alphabetical order as the contributions of all were essential to the study. E.C. provided the statistical workout; A.F. made the proposal of the study and rendered the data from the archives suitable for the analyses; H.S. wrote the paper     

Dementia in two histologically confirmed cases of multiple sclerosis: one case with isolated dementia and one case associated with psychiatric symptoms.

During the past 10 years, considerable attention has been devoted to cognitive impairment in multiple sclerosis. Occasionally this impairment may be so severe that multiple sclerosis presents as a dementia associated with only minor neurological signs and symptoms. The cases of two women affected by multiple sclerosis who presented with a pure dementia are reported. In the first patient, a progressive apragmatic behavioural disturbance with reduced short term memory and learning abilities were the main clinical features. Neuropathological examination of the brain disclosed numerous plaques in the periventricular white matter, with severe atrophy of the corpus callosum. Plaques were also seen in the white matter of both hippocampus and in the columns of the fornix. The impairment of short term memory could be linked to these lesions. Behavioural changes were probably related to the bilateral lesions of the long associative bundles that disconnected the frontal lobes from other parts of the cerebral hemispheres. In the second patient, visual hallucinations were associated with cognitive dysfunction. MRI showed large plaques in the white matter of both left frontal and temporal lobes. Smaller plaques were also present in the periventricular white matter of the occipital lobes, the nature of which were confirmed by a stereotactic biopsy.     

Occipital atrophy is associated with visual hallucinations in Alzheimer's disease

In this study of patients with Alzheimer's disease (AD), patients with visual hallucinations were compared with patients who did not have visual hallucinations to determine if selective occipital lobe atrophy is associated with visual hallucinations. Seven AD patients with visual hallucinations were matched by cognitive score to 7 AD patients without visual hallucinations and 3-D MRI images obtained. A ratio of measured occipital volumes to whole brain volumes was compared between the two groups. AD patients with visual hallucinations had a significantly smaller occipital/whole brain ratio than AD patients without visual hallucinations. These results suggest visual hallucinations in AD may be associated with neuropathology of the occipital lobe.     

Visual misperceptions and hallucinations in Parkinson's disease: Dysfunction of attentional control networks?

Visual misperceptions and hallucinations are a major cause of distress in patients with Parkinson's disease (PD), particularly in the advanced stages of the condition. Recent work has provided a framework for understanding the pathogenesis of these symptoms, implicating impairments from the retina to the integration of external information with preformed internal images. In this article, we propose a novel hypothesis that attempts to explain the presence of visual misperceptions and hallucinations in PD through the aberrant coordination of complimentary yet competing neural networks. We propose that hallucinations in PD reflect the relative inability to recruit activation in the dorsal attention network in the presence of an ambiguous percept, leading to overreliance on default mode network processing and salience arising from the ventral attention network. This inability is proposed to stem from improper function across cortical and subcortical structures secondary to the presence of Lewy body pathology. This hypothesis may be empirically tested by the use of targeted cognitive paradigms. In turn, this may assist our understanding of the pathophysiological mechanisms and cognitive processes contributing to visual misperceptions and hallucinations and ultimately may inform more effective treatment strategies for this troubling symptom.     

Complex visual hallucination and mirror sign in posterior cortical atrophy

Objective: In posterior cortical atrophy (PCA), visual hallucinations are rare symptoms and mirror sign has not been described. Method: Single case report. Results: We reported a 60‐year‐old woman with PCA who reported complex visual hallucinations, such as a man walking in her room, and mirror sign, which was the perception of a stranger staring at her when she looked into a mirror. She could not recognize images of herself in the mirror correctly, although she could recognize that a person standing next to her and the images of that person reflected in the mirror were the same person. Conclusion: Early complex visual hallucinations in this patient appeared to be more characteristic of dementia with Lewy body than Alzheimer's disease (AD). It is hard to explain mirror sign in this patient as being because of either prosopagnosia, Balint's syndrome or advanced AD. This patient may have other underlying cognitive dysfunction.     

Visual hallucinations in Parkinson's disease: a review and phenomenological survey

OBJECTIVES: Between 8% and 40% of patients with Parkinson's disease undergoing long term treatment will have visual hallucinations during the course of their illness. There were two main objectives: firstly, to review the literature on Parkinson's disease and summarise those factors most often associated with hallucinations; secondly, to carry out a clinical comparison of ambulant patients with Parkinson's disease with and without visual hallucinations, and provide a detailed phenomenological analysis of the hallucinations. METHODS: A systematic literature search using standard electronic databases of published surveys and case-control studies was undertaken. In parallel, a two stage questionnaire survey was carried out based on members of a local branch of the Parkinson's Disease Society and followed up with a clinical interview. RESULTS: The review disclosed common factors associated with visual hallucinations in Parkinson's disease including greater age and duration of illness, cognitive impairment, and depression and sleep disturbances. The survey comprised 21 patients with visual hallucinations and 23 without. The hallucinators had a longer duration and a greater severity of illness, and tended to show more depressed mood and cognitive impairment. The typical visual hallucination in these patients is a complex visual image experienced while they are alert and have their eyes open. The image appears without any known trigger or voluntary effort, is somewhat blurred, and commonly moves. It stays present for a period of "seconds" or "minutes". The content can be variable within and between hallucinators, and includes such entities as people, animals, buildings, or scenery. These features resemble those highlighted in hallucinations in the visually impaired (Charles Bonnet's syndrome). CONCLUSION: A consistent set of factors are associated with visual hallucinations in Parkinson's disease. The results of the phenomenological survey and those of visual hallucinations carried out in other settings suggest a common physiological substrate for visual hallucinations but with cognitive factors playing an as yet unspecified role.     

A semi-structured interview to assess visual hallucinations in older people

OBJECTIVE: Visual hallucinations are under-reported by patients and are often undiscovered by health professionals. There is no gold standard available to assess hallucinations. Our objective was to develop a reliable, valid, semi-structured interview for identifying and assessing visual hallucinations in older people with eye disease and cognitive impairment. METHODS: We piloted the North-East Visual Hallucinations Interview (NEVHI) in 80 older people with visual and/or cognitive impairment (patient group) and 34 older people without known risks of hallucinations (control group). The informants of 11 patients were interviewed separately. We established face validity, content validity, criterion validity, inter-rater agreement and the internal consistency of the NEVHI, and assessed the factor structure for questions evaluating emotions, cognitions, and behaviours associated with hallucinations. RESULTS: Recurrent visual hallucinations were common in the patient group (68.8%) and absent in controls (0%). The criterion, face and content validities were good and the internal consistency of screening questions for hallucinations was high (Cronbach alpha: 0.71). The inter-rater agreements for simple and complex hallucinations were good (Kappa 0.72 and 0.83, respectively). Four factors associated with experiencing hallucinations (perceived control, pleasantness, distress and awareness) were identified and explained a total variance of 73%. Informants gave more 'don't know answers' than patients throughout the interview (p = 0.008), especially to questions evaluating cognitions and emotions associated with hallucinations (p = 0.02). CONCLUSIONS: NEVHI is a comprehensive assessment tool, helpful to identify the presence of visual hallucinations and to quantify cognitions, emotions and behaviours associated with hallucinations.     

A case study in the treatment of dementia with Lewy bodies

OBJECTIVE: To describe the assessment and treatment of an elderly woman with parkinsonism, progressive memory and cognitive deficits, and visual hallucinations. METHOD: The patient presented with a 10-year history of hand tremors, an 8-year history of short-term memory problems, and a 3-4-year history of visual hallucinations. Treatment with donepezil and rivastigmine (successively) did not produce the desired benefits. Then she was started on galantamine 4 mg b.i.d. (escalated to 8 mg b.i.d.). RESULTS: The patient's social interaction improved and cognitive decline appeared to be stabilized; hallucinations and agitation were also better controlled. CONCLUSION: By current criteria, this subject would be labeled as having Parkinson's disease with dementia, although she exhibited the core features of dementia with Lewy body disease. As suggested in previous studies, cholinesterase inhibitors may be effective in treating psychotic symptoms; however, all currently available agents may not be equally effective.     

Peduncular hallucinosis caused by a tumour in the right thalamus. A case study

Peduncular hallucinations are vivid, complex visual hallucinations. They generally arise as a result of lesions in the midbrain. A 46-year-old man had peduncular hallucinations arising from a tumour in the right thalamus. The patient was not operated on for this tumour. The visual hallucinations were treated with psychiatric medications. This treatment reduced the patient's anxiety and visual hallucinations and he became better able to cope with his symptoms. Psychiatrists need to increase their knowledge with regard to the diagnosis and treatment of these symptoms.