Abstract
Dementia with Lewy Bodies (DLB) is the second most common neurodegenerative dementia after Alzheimer disease (ad). Its heterogeneous presentation and overlapping features with other dementias result in frequent misdiagnosis, which can delay optimal management. The present case illustrates complex symptomology, comprehensive assessment, and diagnostic challenges in a patient with DLB. A 64-year-old female presented to an academic medical center neurology clinic with rapid cognitive deterioration over the past 1.5years accompanied by visual disturbances, psychiatric and behavioral changes, and mild motor symptoms. Initial differential included Creutzfeldt-Jakob disease, atypical parkinsonism, frontotemporal lobar degeneration (FTLD), encephalopathy, and ad. Work-up in included neuropsychological, neuroimaging, cerebral spinal fluid (CSF), electroencephalogram (EEG), and skin biopsy assessments. Neuropsychological interview revealed fluctuating attention/alertness, visual hallucinations, hyposmia, paranoid and repetitive behaviors, and subtle evidence for dream enactment. Her cognitive profile showed global impairment with pronounced visuospatial and executive deficits. Recognition memory, verbal reasoning, and naming were relatively well-preserved. Magnetic resonance imaging showed mild atrophy; fluorodeoxyglucose positron emission tomography (PET) showed hypometabolism in frontal and temporal regions whereas uptake was highest in the occipital lobes and basal ganglia; skin biopsy was positive for synucleinopathy; CSF and EEG were broadly unrevealing. The preponderance of data was consistent with diagnostic criteria for probable DLB. Skin biopsy increased diagnostic confidence. Atypical features included relatively rapid cognitive decline, minimal parkinsonism, and PET imaging suggesting FTLD. The present case highlights the utility of a multidisciplinary approach (e.g., neurological exam, detailed symptom screening, neuropsychological testing, and advanced laboratory assessments) in a complex case of DLB.
Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
More From: Archives of clinical neuropsychology : the official journal of the National Academy of Neuropsychologists
Disclaimer: All third-party content on this website/platform is and will remain the property of their respective owners and is provided on "as is" basis without any warranties, express or implied. Use of third-party content does not indicate any affiliation, sponsorship with or endorsement by them. Any references to third-party content is to identify the corresponding services and shall be considered fair use under The CopyrightLaw.