1/29. Familial dementia with lewy bodies (DLB).We report a rare familial case of dementia with lewy bodies (DLB). The patient was a man who died at the age of 51. His parents were first cousins. Among three siblings, two were diagnosed as probable cases of DLB, and one was a possible case, according to the clinical diagnostic criteria of the consortium on DLB. Following the patient's autopsy, he was found to have had DLB without neurofibrillary tangles or senile plaques (pure form of diffuse lewy body disease). His other siblings have been followed for more than ten years. Although these patients with familial DLB displayed clinical variability, all three siblings showed progressive dementia of early onset and progressive language disorder with paraphasia and difficulty in finding words. Psychotic features were also seen in the three siblings. The patient's sister showed compulsive behavior, and the other two siblings showed symptoms of parkinsonism. Neuropathologically, in addition to the usual neuropathology of DLB, the autopsy findings showed numerous small spheroids in the stratum pyramidale from the subiculum to CA1 of the hippocampus. Significant neuronal loss in CA2-3 of the hippocampus was detected. Axonal flow disturbance may be involved in the hippocampal formations of this incidence of familial DLB.- - - - - - - - - - ranking = 1keywords = parkinsonism (Clic here for more details about this article) |
2/29. Electrophysiological observations in hereditary parkinsonism-dementia with Lewy body pathology.We studied the only two living affected individuals who are part of a previously reported kindred that expresses a hereditary parkinsonism-dementia syndrome with Lewy body pathology. The electrophysiological characteristics of the hyperkinetic movement disorders in these patients were examined to provide physiological insights into the clinical phenotype of this syndrome. Evaluation of both patients showed 7-9 Hz electromyographic discharges in upper extremity muscles during postural activation, and one patient showed a 4-5 Hz discharge pattern correlating to a rest tremor. Brief (<50 ms) myoclonic electromyographic discharges were seen in both patients, and a time-locked relationship to a focal cortical premovement electroencephalographic potential was elicited in one patient. Somatosensory evoked potentials were not enlarged and long latency reflexes were not enhanced. electroencephalography was normal in one patient but showed pathologic slow frequencies in the other. The electrophysiological findings show evolution which correlates with an apparent characteristic evolution of hyperkinetic movement disorders that accompanies the severe progression of parkinsonism-dementia in this kindred. These results have implications for the future study of this and similar syndromes.- - - - - - - - - - ranking = 6keywords = parkinsonism (Clic here for more details about this article) |
3/29. Distinctive neuropathology revealed by alpha-synuclein antibodies in hereditary parkinsonism and dementia linked to chromosome 4p.The identification of the alpha-synuclein gene on chromosome 4q as a locus for familial Lewy-body parkinsonism and of alpha-synuclein as a component of lewy bodies has heralded a new era in the study of Parkinson's disease. We have identified a large family with Lewy body parkinsonism linked to a novel locus on chromosome 4p15 that does not have a mutation in the alpha-synuclein gene. Here we report the clinical and neuropathological findings in an individual from this family and describe unusual high molecular weight alpha-synuclein-immunoreactive proteins in brain homogenates from brain regions with the most marked neuropathology. Distinctive histopathology was revealed with alpha-synuclein immunostaining, including pleomorphic lewy bodies, synuclein-positive glial inclusions and widespread, severe neuritic dystrophy. We also discuss the relationship of this familial disorder to a lewy body disease clinical spectrum, ranging from Parkinson's disease to dementia with psychosis.- - - - - - - - - - ranking = 6keywords = parkinsonism (Clic here for more details about this article) |
4/29. Brainstem-type lewy body disease presenting with progressive autonomic failure and lethargy.The authors report an autopsy case characterized by progressive lethargy and autonomic failure with a distinctive pattern of occurrence of lewy bodies. Autonomic dysfunction such as sleep apnea, orthostatic hypotension, dysuria, and hypohidrosis predominated with lethargy, whereas parkinsonism was not apparent. Numerous lewy bodies were widely evident microscopically in brainstem nuclei and the intermediolateral cell columns of the spinal cord, as well as in the sympathetic ganglia, but were rare or absent in the cerebral cortex and other supratentorial structures. Marked neuronal loss was seen in the locus ceruleus, raphe nuclei, dorsal vagal nuclei, and intermediolateral cell columns, but neurons in the substantia nigra, other brain regions, and sympathetic ganglia appeared undiminished. This case represents a specific clinicopathologic form of lewy body disease occurring predominantly in the brainstem, spinal cord, and sympathetic ganglia.- - - - - - - - - - ranking = 1keywords = parkinsonism (Clic here for more details about this article) |
5/29. FDG positron emission tomography in diffuse lewy body disease: a case report.lewy body disease is a clinicopathologic condition that includes Parkinson's disease at one end and diffuse lewy body disease at the other hand. The latter is often associated with progressive cognitive deterioration, levodopa-responsive parkinsonism, fluctuations of cognitive and motor functions, and visual and auditory hallucinations. In addition, it can be a familial disease. Clinical and positron emission tomographic findings are described in a patient with atypical dementia and movement disorder and a pathologically proved diagnosis of diffuse lewy body disease.- - - - - - - - - - ranking = 1keywords = parkinsonism (Clic here for more details about this article) |
6/29. 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.- - - - - - - - - - ranking = 1keywords = parkinsonism (Clic here for more details about this article) |
7/29. 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.- - - - - - - - - - ranking = 1keywords = parkinsonism (Clic here for more details about this article) |
8/29. 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.- - - - - - - - - - ranking = 3keywords = parkinsonism (Clic here for more details about this article) |
9/29. 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.- - - - - - - - - - ranking = 1.4395078359446keywords = parkinsonism, parkinsonian (Clic here for more details about this article) |
10/29. Familial diffuse lewy body disease, eye movement abnormalities, and distribution of pathology.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.- - - - - - - - - - ranking = 4.0488036889136keywords = parkinsonian syndrome, parkinsonism, parkinsonian (Clic here for more details about this article) |
| | Next -> |