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Rapidly progressive diffuse Lewy body disease.
| Content Provider | Semantic Scholar |
|---|---|
| Author | Gaig, Carles Valldeoriola, Francesc Gelpí, Ellen Ezquerra, Mario Llufriu, Sara Buongiorno, Mariateresa Rey, María Jesús Fernández-Trujillo Martí, María José Tarrazó Graus, Francesc Tolosa, Eduardo |
| Copyright Year | 2011 |
| Abstract | BACKGROUND Lewy body syndromes (mainly Parkinson's disease and dementia with Lewy bodies) share many clinical features and usually have a slowly progressive course. Some patients may show rapid symptoms progression. OBJECTIVE To evaluate clinical and neuropathological features of patients with a rapidly progressive diffuse Lewy Body disease. METHODS Review clinical records and pathological findings of 6 cases with diffuse Lewy Body disease and rapid disease progression (less than 18 months before death). RESULTS Mean age at disease onset was 72.5 years, and mean disease duration was 9 months. Onset consisted of delirium in 3 patients and rapidly progressive dementia in the other three. All cases presented visual hallucinations and delusions; cognitive symptoms were fluctuating in two, parkinsonism occurred in four, and myoclonus in three. Brain MRI did not show cortical or basal ganglia hyperintensities. Periodic sharp waves were absent on EEG. 14.3.3 protein in CSF was negative. Myocardial (123) I-metaiodo-benzyl-guanidine SPECT showed marked reduction in tracer uptake in the 2 patients tested. Neuropathological studies did not identify any particular feature that could differentiate rapidly progressive diffuse Lewy body disease from classical diffuse Lewy body disease. CONCLUSIONS Diffuse Lewy body disease is a possible cause of rapidly progressive dementia and should be included in the differential diagnosis of confusional states of undetermined cause. In patients with rapidly progressive dementia, the presence of fluctuating cognition, parkinsonism, hallucinations, delusions, or severe dysautonomia, should raise the suspicion of diffuse Lewy body disease. Neuroimaging studies such as (123) I-metaiodo-benzyl-guanidine myocardial scintigraphy may support the clinical diagnosis of diffuse Lewy body disease. © 2011 Movement Disorder Society. |
| Starting Page | 1807 |
| Ending Page | 1814 |
| Page Count | 8 |
| File Format | PDF HTM / HTML |
| Alternate Webpage(s) | http://www.movementdisorders.org/MDS-Files1/Resources/PDFs/md_article7.pdf |
| PubMed reference number | 21484863v1 |
| Alternate Webpage(s) | https://doi.org/10.1002/mds.23506 |
| DOI | 10.1002/mds.23506 |
| Journal | Movement disorders : official journal of the Movement Disorder Society |
| Volume Number | 26 |
| Issue Number | 7 |
| Language | English |
| Access Restriction | Open |
| Subject Keyword | Basal Ganglia Diseases Cessation of life Confusion Delirium Delusions Dementia Dysautonomia Guanidine Hydrochloride Hallucinations Lewy Body Disease Movement Disorders Myocardial Scintigraphy Myoclonus Neurobehavioral Manifestations Neuroimaging Parkinsonian Disorders Patients Progressive Disease Radionuclide Imaging Syndrome Tracer |
| Content Type | Text |
| Resource Type | Article |