Background: Klüver-Bucy syndrome (KBS) is a rare neuropsychiatric disorder, and it can be associated with a variety of neurological disorders. It is characterized by visual agnosia, placidity, hyperorality, hypersexuality, dietary changes, amnesia, and hypermetamorphosis. KBS is mainly a clinical diagnosis, with at least three symptoms sufficient to diagnose the condition.
Case Description: The case describes a 49-year-old Filipino woman with a history of hypertension who presented with symptoms strongly suggesting KBS following subarachnoid hemorrhage, including behaviors such as hyperorality, hypermobility, placidity, hypermetamorphosis, and hypersexuality along with memory disturbance. She was managed as a case of brief psychotic disorder initially with olanzapine, then on the second presentation as a case of delirium with risperidone.
Conclusion: Among many symptoms of KBS, only three symptoms are required for the diagnosis clinically. Numerous neurological conditions can cause KBS. Symptomatic treatment is the mainstream treatment currently for KBS. While different differential diagnoses are present, neurologists, psychiatrists, neurosurgeons, and radiologists should collaborate and be vigilant for the diagnosis of KBS, especially with the presence of one of its etiologies.
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http://dx.doi.org/10.25259/SNI_358_2024 | DOI Listing |
Alzheimers Dement
December 2024
Stanford University School of Medicine, Stanford, CA, USA.
Recent advances in biomarkers, enabling the in vivo detection of pathological aggregates of alpha-synuclein (asyn), allow a shift from a clinical to a biological definition of Parkinson's disease (PD) and dementia with Lewy bodies (DLB). The newly proposed "Neuronal alpha-Synuclein Disease (NSD)" is defined by the presence of pathologic neuronal (n-asyn) species detected in vivo (S), irrespective of any specific clinical syndrome. Additional biological anchors include dopaminergic neuronal dysfunction (D).
View Article and Find Full Text PDFAlzheimers Dement
December 2024
University of California, Irvine, Irvine, CA, USA.
Background: Recruitment challenges in people with and without Down syndrome (DS) can delay research progress and risk sample bias. This study identified and quantified differences in research attitudes across populations of research enrollment decision-makers for individuals with and without DS.
Method: We compared scores on the Research Attitudes Questionnaire (RAQ) of individuals enrolled in two recruitment registries: the UCI Consent to Contact [C2C (N = 4818)] and DS-Connect (N = 976).
Alzheimers Dement
December 2024
Department of Neurosciences, University of California, San Diego, La Jolla, CA, USA.
Background: Increased APP gene dosage is both necessary and sufficient to result in Down Syndrome Alzheimer's Disease (DSAD) in humans and AD-related degenerative changes in mouse models of DS.
Method: We tested antisense oligonucleotides (ASOs) designed to suppress APP expression via RNAseH1-mediated degradation in the Dp(16)1Yey or Dp(16) model of Down Syndrome. Dp(16) is trisomic for human chromosome 21 syntenic regions on murine chromosome 16, containing 115 genes including APP.
Background: Clinical assessments utilized in trials to measure progression in sporadic Alzheimer's disease (SAD) such as the MMSE, ADAS-Cog, CDR-SB and ADCS-ADL are well established. Distinct assessments are utilized for Down Syndrome-related Alzheimer's disease (DSAD). Although these assessments are less well established, they probe comparable domains of cognition and function.
View Article and Find Full Text PDFAlzheimers Dement
December 2024
University of Kansas Medical Center, Kansas City, KS, USA.
Background: Evidence in adults without Down syndrome (DS) suggests that exercise during mid-life improves cognitive function and decreases risk of later life dementia. Studies supporting this relationship in adults with DS are limited. The purpose of this study was to examine changes in cognitive function after a 12-mo exercise intervention in adults with DS without dementia.
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