2-(4--(methylamino)phenyl)-6-hydroxybenzothiazole and Multiple-System-Atrophy

Recent reports show that dementia occurs in 5-26% of multiple system atrophy (MSA) patients. However, the structural or pathological correlates of dementia in MSA are unclear yet.. Of 152 patients with MSA, 59 fulfilled the criteria of probable MSA and 9 (15%) had dementia. Six of those patients and 9 without dementia, in addition to 10 controls, were included. All subjects underwent clinical evaluation including UMSARS, neuropsychological examinations, 3T-MRI, and Pittsburgh Compound B (PIB) PET imaging. The cortical thickness was assessed using surface-based morphometry.. Age and disease duration were similar between MSA with dementia and without dementia, while motor disability was more severe in MSA with dementia. In neuropsychological tests, attention, visuospatial function, and language function were impaired in MSA with dementia. Mean PIB binding was similar among the three groups. Cortical thickness was reduced in precuneus/cuneus, uncus, and posterior cingulate in MSA with dementia compared to the controls, and in parahippocampal and lingual cortices compared to MSA without dementia.. Dementia was found in 15% of the probable MSA patients, which was similar to those reported in previous studies. It appears that amyloid pathology has limited role in dementia in MSA, although some patients had increased cortical amyloid burden. Cortical thinning in MSA-D was observed in areas where cortical thinning was reported in Alzheimer disease or Parkinson disease dementia, but its pathological relevance is unclear. The neuropathological processes leading to the development of dementia in MSA appears to be multifactorial and heterogenous.

Topics: Aged; Aniline Compounds; Brain; Dementia; Female; Humans; Image Processing, Computer-Assisted; Magnetic Resonance Imaging; Male; Middle Aged; Multiple System Atrophy; Neuropsychological Tests; Positron-Emission Tomography; Psychiatric Status Rating Scales; Thiazoles

Multiple Systems Atrophy (MSA) and Dementia with Lewy bodies (DLB) can present with both REM behavior disorder and severe autonomic dysfunction. In rare occasions, patients with MSA progress to cognitive impairment and even dementia. Positron emission topography (PET) imaging using both the amyloid ligand Pittsburgh Compound B (11C-PiB) and 18 flurodeoxyglucose (18F-FDG) was used to ascertain the presence of amyloid and pattern of glucose metabolic derangement in both disorders.. Patients diagnosed with probable DLB or MSA, with clinical symptoms of either REM Behavior Disorder (RBD), Parkinsonism, or dysautonomia were prospectively identified. All underwent both 11C-PiB and 18F-FDG PET imaging. Statistical comparison between DLB, MSA, and normal controls was performed.. Six patients, 3 with DLB, 2 with Parkinson predominant MSA (MSA-P), and 1 with cerebellar predominant MSA (MSA-C) were identified. Increased level of PiB retention was noted in all patients diagnosed with DLB, but was absent in MSA. In those with DLB, glucose hypometabolism corresponded with regions of amyloid presence, and included prefrontal, parietotemporal, occipital and primary visual cortex regions. MSA patients were distinguished by cerebellar glucose hypometabolism.. These findings emphasize the distinguishing characteristics between the alpha-synuclein related disorders of DLB and MSA. The absence of amyloid in the cases of MSA is a possible distinguishing characteristic of the disorder.

Topics: Aged; Amyloid; Aniline Compounds; Brain Mapping; Female; Fluorodeoxyglucose F18; Glucose; Humans; Lewy Body Disease; Male; Middle Aged; Multiple System Atrophy; Positron-Emission Tomography; Thiazoles; Tomography, X-Ray Computed