11/74. Improvement in sundowning in dementia with lewy bodies after treatment with donepezil. Sundowning, manifested as a recurring increase in restlessness and agitation in the evening, is described in a 71-year-old man with clinically diagnosed dementia with lewy bodies. An objective measure of activity using the activity electronic monitoring technique indicated a marked increase in activity level during the evening compared to earlier in the day. After treatment with donepezil, a cholinesterase inhibitor, ratings of behavioural symptoms improved. In addition, there was a marked reduction in evening activity and an increase in daytime activity. cognition and parkinsonism also improved. Possible explanations for this finding are discussed. ( info) |
12/74. Successful use of donepezil for the treatment of dementia with lewy bodies. OBJECTIVE: To report a case of the successful use of donepezil for treatment of cognitive and noncognitive symptoms in a patient with dementia with lewy bodies. CASE SUMARY: An 86-year-old white woman with dementia was experiencing early-onset significant fluctuation of her cognitive status, functional impairment, visual hallucinations, aggression, and parkinsonism. She was intially diagnosed with alzheimer disease and Parkinsons disease and prescribed donepezil 5 mg/d and benztropine 1 mg twice daily. On reexamination of the case by a neurologist, the diagnoses were revised to dementia with lewy bodies. The benztropine was discontinued, and donepezil was increased to 10 mg/d. The patient's cognitive and functional status significantly improved, as did her visual hallucinations. DISCUSSION: This case supports previous reports of the marked responsiveness of patients with dementia with lewy bodies to acetylcholinesterase inhibitors. This may be explained by the marked cholinergic deficit observed in patients with dementia with lewy bodies and the evidence linking cognitive as well as noncognitive symptoms to this deficit. CONCLUSIONS: The present case suggests that patients with dementia with lewy bodies respond well to acetylcholinesterase inhibitors. Controlled trials are necessary to further define the role of these drugs for this disease. ( info) |
| OBJECTIVE: To report a case initially fulfilling the clinical criteria for probable alzheimer disease, although later clinical features suggested dementia with lewy bodies. oxygen 15-labeled positron emission tomograms revealed a pattern of hypometabolism characteristic of alzheimer disease. At post mortem, there was no evidence of the pathological features of alzheimer disease, but diffuse cortical lewy bodies were seen in the pigmented brainstem nuclei and cerebral cortex. DESIGN: A case report. SETTING: Tertiary referral center. PATIENT: A 65-year-old white man presented with a 3-year history of memory loss and language difficulties. RESULTS: oxygen 15-labeled positron emission tomograms revealed hypometabolism in the frontal, temporal, and parietal lobes, more severe on the left than right. metabolism in the left caudate was just outside the 95% reference range. Occipital metabolism was normal. CONCLUSIONS: Positron emission tomographic studies have been reported to show occipital hypometabolism in dementia with lewy bodies, in addition to the characteristic posterior bitemporal biparietal pattern of alzheimer disease. We suggest that although this finding may favor a diagnosis of dementia with lewy bodies, it is not necessary for diagnosis. ( info) |
14/74. [18F]FDG-PET study in dementia with lewy bodies and Alzheimer's disease. 1. The authors report two siblings with dementia with lewy bodies (DLB). Both the older brother and the younger sister underwent positron emission tomography (PET) studies with [18F]-2-fluoro-deoxy-D-glucose (FDG) during life. The FDG-PET study demonstrated unique and pronounced metabolic impairment in the occipital cortex in both patients. The clinical diagnosis of DLB in the sister was confirmed by autopsy. 2. FDG-PET images from 11 patients with Alzheimer's disease (AD), 7 patients with DLB and 10 age-matched normal subjects were obtained and analyzed by the statistical parametric mapping (SPM) method. The SPM demonstrated a widespread metabolic reduction in the DLB group. The reduction was particularly pronounced in the visual association cortex in the DLB group compared to the AD group irrespective of clinical severity of the disease. 3. These findings suggest that functional neuroimaging techniques, including FDG-PET, will provide a valuable diagnostic aid to differentiate DLB from AD, and this will help detect DLB patients in the early stage of the disease. ( info) |
15/74. parkinson disease and its differentials. Diagnoses made easy. BACKGROUND: parkinson disease is a common neurological disorder that is both underdiagnosed and inaccurately diagnosed. There is no reliable biological marker or test that can differentiate between causes of parkinsonism. Even for experienced clinicians, the clinical diagnostic accuracy compared to post mortem findings is about 80%. OBJECTIVE: To discuss the clinical features that differentiate parkinson disease from other important causes of parkinsonism. DISCUSSION: Although parkinson disease is a common cause of parkinsonism, other candidates such as drug reactions, benign essential tremor, vascular disease and Lewy body dementia need to be differentiated. Incorrect diagnosis can result in complications related particularly to the use of levodopa and antipsychotic agents. Diagnostic accuracy is important to ensure appropriate management, to avoid complications and to assist patients to have realistic expectations and prognostic information about their condition. ( info) |
| To investigate similarities and differences between Alzheimer's disease (AD) and dementia with lewy bodies (DLB), we undertook a demographic analysis of 277 patients from the Kathleen Price Bryan brain Bank with an antemortem diagnosis of probable AD. patients with additional, possibly confounding clinical and pathologic diagnoses such as infarcts, hematomas, neoplasms, and other neurodegenerative disorders, were excluded from the analysis. Neuropathologically, AD alone was present in 192 subjects (69%), and DLB was found in 85 subjects (31%). All of the DLB cases had neuropathologic evidence of AD sufficient to meet CERAD criteria for a diagnosis of definite AD plus nigral lewy bodies. Gender, apolipoprotein E (APOE) genotype, brain weight, age at death, duration of disease and Braak stage were compared between the two groups. Statistical analyses were performed using Fisher's exact test for comparisons of categorical data and Student's t-test for comparison of means for continuous outcomes. The proportion of males and females was balanced in the combined AD and DLB populations. There was a highly statistically significant increased frequency of APOE 3/4 in males with DLB (P = 0.007). We found higher brain weights in males with DLB versus males with AD (P = 0.012). AD was more frequent in females and DLB was more frequent in males (P = 0.019). Our findings with respect to age at death, duration of disease and Braak stage within diagnostic groups confirm previously reported findings. These data suggest that lewy bodies are more common in males affected with dementia, especially those with the APOE 3/4 genotype. ( info) |
17/74. dementia with lewy bodies treated with rivastigmine: effects on cognition, neuropsychiatric symptoms, and sleep. dementia with lewy bodies (DLB) is a common cause of the dementia syndrome. Symptomatic treatment of the fluctuating cognition, visual hallucinations, and sleep disturbance that characterize this condition is challenging; neuroleptics are relatively contraindicated. We describe eight patients fulfilling the consensus diagnostic criteria for probable DLB who were treated with rivastigmine. Clinical features rated were: cognition by the Modified Mini-Mental State Examination (3MS); and behavioral and psychiatric symptoms by the Neuropsychiatric Inventory (NPI). Additional information was obtained from family and nursing reports. Seven patients showed resolution or improvement in cognition and neuropsychiatric symptoms as demonstrated by improvement in their 3MS and NPI scores. They also became more independent in mobility and activities of daily living, and the majority returned to live in their own home. Of the seven patients with sleep disruption, six improved. One case had no improvement in his symptomatology and the rivastigmine was stopped. Outcomes in this case series suggest that rivastigmine is well tolerated in clinical practice. ( info) |
18/74. Lewy body dementia: case report and discussion. BACKGROUND: Lewy body dementia is a common but frequently underdiagnosed cause of dementia often mistaken for the more familiar entity of alzheimer disease. Clinically the distinction is important, because it can have profound implications for management. methods: The medical literature was searched using the keywords "lewy bodies," "Lewy body dementia," "Alzheimer dementia," and "parkinsonian disorders." A case of Lewy body dementia is described. RESULTS: An elderly man had long-standing diagnoses of alzheimer disease and parkinson disease. After he was evaluated thoroughly, the diagnosis was revised to Lewy body dementia, leading to changes in treatment that were associated with dramatic improvement in the patient's mental status. Evidence from the literature suggests that Lewy body dementia can be diagnosed in primary care settings based on clinical criteria. The physician should be alert to this diagnosis, and special attention should be paid to dementia patients who exhibit parkinsonism, hallucinations, fluctuating cognition, or prominent visuosperceptual deficits. CONCLUSIONS: The diagnosis of Lewy body dementia has important implications. It is associated with a high incidence of neuroleptic sensitivity, necessitating great caution in the use of these common antipsychotic agents. Early studies indicate cholinesterase inhibitors can be beneficial for treating the hallucinations and behavior disturbances that afflict these patients and might also improve cognition. ( info) |
19/74. Improvement of both psychotic symptoms and Parkinsonism in a case of dementia with lewy bodies by the combination therapy of risperidone and L-DOPA. A 69-year-old female of dementia with lewy bodies (DLB) with fluctuating cognitive impairment, visual and auditory hallucinations, persecutory delusions and Parkinsonism was treated by the combination of 1 mg/day risperidone and 300-750 mg/day L-DOPA. By this combination therapy, both the psychotic symptoms and Parkinsonism improved, while cognitive function did not deteriorate. This report suggests that the combination therapy of risperidone and L-DOPA may be worth considering in the special cases of DLB. ( info) |
| BACKGROUND: Familial diffuse lewy body disease (DLBD) is rare and not yet associated with a defect in the synuclein gene. In the differential diagnosis of the parkinsonian syndromes, defects in vertical gaze tend to be identified with progressive supranuclear palsy. False-positive diagnosis of progressive supranuclear palsy can occur, and defects in vertical gaze have been reported in DLBD, although so far a pure vertical gaze palsy associated with pathological abnormalities in the substrate for vertical gaze has not been described. OBJECTIVES: To report the clinical and pathological findings in 2 siblings with DLBD, and to relate the distribution of the pathological abnormalities in the brainstem to centers for vertical gaze. MATERIALS: For several years, 2 Irish siblings experienced a progressive parkinsonism-dementia complex associated in one with a defect in vertical gaze and in both with visual hallucinations. RESULTS: In both patients, results of pathological examination revealed (1) Lewy bodies positive for ubiquitin and alpha-synuclein together with cell loss and gliosis in the substantia nigra, locus ceruleus, and neocortex; and (2) similar findings in the rostral interstitial nucleus of the medial longitudinal fasciculus, the posterior commissure, and the interstitial nucleus of Cajal (substrates for vertical gaze). CONCLUSIONS: Familial DLBD (not shown to be genetically as distinct from environmentally transmitted) has been shown to exist in an Irish family. Caution should be enjoined in the interpretation of defects in vertical gaze in the differential diagnosis of the parkinsonian syndromes. ( info) |