title: Rapidly progressive diffuse Lewy body disease.
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.
authors.affiliation: Neurology Service, Hospital Clínic of Barcelona, Barcelona, Spain.
authors.forename: Carles
authors.initials: C
authors.lastname: Gaig
authors.forename: Francesc
authors.initials: F
authors.lastname: Valldeoriola
authors.forename: Ellen
authors.initials: E
authors.lastname: Gelpi
authors.forename: Mario
authors.initials: M
authors.lastname: Ezquerra
authors.forename: Sara
authors.initials: S
authors.lastname: Llufriu
authors.forename: Mariateresa
authors.initials: M
authors.lastname: Buongiorno
authors.forename: Maria Jesús
authors.initials: MJ
authors.lastname: Rey
authors.forename: Maria Jose
authors.initials: MJ
authors.lastname: Martí
authors.forename: Francesc
authors.initials: F
authors.lastname: Graus
authors.forename: Eduardo
authors.initials: E
authors.lastname: Tolosa
mesh_terms: D000368:Aged; D001921:Brain / Q000473:pathology*; D003693:Delirium / Q000209:etiology / Q000473:pathology / Q000503:physiopathology; D018450:Disease Progression; D017809:Fatal Outcome; D005260:Female; D006212:Hallucinations / Q000209:etiology / Q000473:pathology / Q000503:physiopathology; D006801:Humans; D020961:Lewy Body Disease / Q000150:complications / Q000473:pathology* / Q000503:physiopathology*; D008297:Male; D008499:Medical Records; D009207:Myoclonus / Q000209:etiology / Q000473:pathology / Q000503:physiopathology; D020734:Parkinsonian Disorders / Q000209:etiology / Q000473:pathology / Q000503:physiopathology; D012720:Severity of Illness Index