A 34-year-old woman presented with a 1-year history of progressive apathy, executive dysfunction, and memory impairment. Examination revealed moderate frontal dysfunction and bipyramidal signs. MRI brain (figure 1) showed a symmetric leukoencephalopathy sparing subcortical U-fibers.
Background and Objectives Several clinical and neuroimaging biomarkers have been proposed to identify individuals with Parkinson disease (PD) who are at risk for ongoing cognitive…
Objectives Vanishing white matter (VWM), an inherited leukoencephalopathy affecting the brain and the spinal cord, is most often a childhood-onset progressive disorder, generally presenting with…
Background and Objectives We investigated the genetic basis and brain metabolism and blood flow of a Japanese family with spinocerebellar degeneration (SCD), with multiple affected…
Background and Objective This was a multicenter study aimed at investigating the characteristics of cognitive decline, neuropsychiatric symptoms, and brain imaging in individuals with subjective…
Background and Objectives Disease-modifying therapeutic trials for genetic frontotemporal dementia (FTD) are underway, but sensitive cognitive outcome measures are lacking. The aim of this study…
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