Preclinical Detection of Alpha-Synuclein Seeding Activity in the Colon of a Transgenic Mouse Model of Synucleinopathy by RT-QuIC

In synucleinopathies such as Parkinson’s disease (PD) and dementia with Lewy body (DLB), pathological alpha-synuclein (α-syn) aggregates are found in the gastrointestinal (GI) tract as well as in the brain. In this study, using real-time quaking-induced conversion (RT-QuIC), we investigated the presence of α-syn seeding activity in the brain and colon tissue of G2-3 transgenic mice expressing human A53T α-syn. Here we show that pathological α-syn aggregates with seeding activity were present in the colon of G2-3 mice as early as 3 months old, which is in the presymptomatic stage prior to the observation of any neurological abnormalities. In contrast, α-syn seeding activity was not detectable in 3 month-old mouse brains and only identified at 6 months of age in one of three mice. In the symptomatic stage of 12 months of age, RT-QuIC seeding activity was consistently detectable in both the brain and colon of G2-3 mice. Our results indicate that the RT-QuIC assay can presymptomatically detect pathological α-syn aggregates in the colon of G2-3 mice several months prior to their detection in brain tissue.

Oral Cenesthopathy Proceeding Dementia with Lewy Body: a Case Report

We herein report a case of oral cenesthopathy that proceeded dementia with Lewy body (DLB). A 70-year-old female presented with oral cenesthopathy. She was diagnosed with major depression/late-onset schizophrenia and treated with paroxetine and perospirone. Subsequently, she developed severe parkinsonism. Her diagnosis changed to DLB based on clinical features and her magnetic resonance imaging, MIBG scintigraphy, and DAT scans. After tapering off paroxetine and perospirone, the patient was treated with donepezil and levodopa. The DLB symptoms, including oral cenesthopathy and parkinsonism, were relieved. This case indicates that oral cenesthopathy may occur as an early symptom of DLB.

A-030 Sensitivity and Specificity of Measures for Dementia with Lewy Bodies

The present review aimed to examine sensitivity and specificity across neuropsychological tests for Dementia with Lewy Body (DLB), to enhance diagnostic utility. A systematic search of the literature was conducted. Databases used: PsycInfo, Discovery Service for Carlos Albizu University-Miami, PsycARTICLES. Data selection criteria entailed articles from 2010–2020. Search terms included: Dementia with Lewy body, sensitivity, specificity, neuropsychological assessment, neuropsychological testing. Original search yielded 27 results, from those, only 8 articles contained sensitivity and specificity regarding DLB. The target population of review were older adults 65 and older. The assessment measures analyzed were the BSID, HVLT, MMSE QSPT, SAI, MMSE, and ICS, and others. Findings of eight studies were evaluated regarding sensitivity and specificity across neuropsychological assessments for DLB. Among the most specific and sensitive measures were the Alba Screening Instrument (ASI; sensitivity 90.7%, specificity 93.6%), and a combination of the Auditory Verbal Learning Test (AVLT) percent retention, Block Design, Trail Making Test--Part A, and Benton Visual Form Discrimination (specificity 96.1% and sensitivity 88.6%). Lower specificity and sensitivity were found in Illusory contour (ICs-4; specificity and sensitivity of 37.1% and 88.6%) and the Minimental State Examination-Pentagon Test (MMSE-QSPT; specificity 78.67% and sensitivity 70.29%). Other tests and combinations were explored. Specificity ranged from 76% to 96.1%, while sensitivity ranged from 37.1% to 90.7% throughout the neuropsychological tests examined. One major limitation across the studies constituted lack of pathological, post-mortem, confirmation. Additionally, while the use of cutoff scores across assessments appeared to enhance the sensitivity and specificity, it seemed to compromise the diagnostic accuracy of AD.

Oral Cenesthopathy Proceeding Dementia With Lewy Body: A Case Report

Somatic hallucination is defined as the false sensation of an occurrence in the body. When the hallucination is grotesque and visceral, it is classified as a cenesthopathy (1). Cenesthopathy is likely influenced by culture; one study reported that, of the seven unique countries from which patients were recruited, those from Ghana and those with chronic schizophrenia were significantly more likely to report cenesthesia (2).

Oral cenesthopathy proceeding dementia with Lewy body: a case report

Background We herein report a case of oral cenesthopathy that proceeded dementia with Lewy body (DLB). Case presentation: A 70-year-old female presented with oral cenesthopathy. She was diagnosed with major depression/late-onset schizophrenia and treated with paroxetine and perospirone. Subsequently, she developed sever parkinsonism. Her diagnosis changed to DLB based on clinical features and her magnetic resonance imaging, MIBG scintigraphy, and DAT scans. After tapering off paroxetine and perospirone, the patient was treated with donepezil and levodopa. The DLB symptoms, including oral cenesthopathy and parkinsonism, were relieved. Conclusion This case indicates that oral cenesthopathy may occur as an early symptom of DLB.