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Numerous kindreds with familial frontotemporal dementia and/or amyotrophic lateral sclerosis have been linked to chromosome 9, and an expansion of the GGGGCC hexanucleotide repeat in the non-coding region of chromosome 9 open reading frame 72 has recently been identified as the pathogenic mechanism. We describe the key characteristics in the probands and(More)
(3,4-methylenedioxymethamphetamine) is an amphetamine analogue that produces euphoric and stimulant effects and a feeling of closeness towards others. 1,2 For more than a decade, MDMA (colloquially known as " Ecstasy " or " E ") has been widely used by young adults as a dance-party drug. The usual recreational oral dose is 1–2 tablets (each containing about(More)
OBJECTIVE To determine the risk factors associated with dementia with Lewy bodies (DLB). METHODS We identified 147 subjects with DLB and sampled 2 sex- and age-matched cognitively normal control subjects for each case. We also identified an unmatched comparison group of 236 subjects with Alzheimer disease (AD). We evaluated 19 candidate risk factors in(More)
OBJECTIVE Rapid eye movement sleep behavior disorder (RBD) is associated with neurodegenerative disease and particularly with the synucleinopathies. Convenience samples involving subjects with idiopathic RBD have suggested an increased risk of incident mild cognitive impairment (MCI), dementia (usually dementia with Lewy bodies), and Parkinson disease (PD).(More)
OBJECTIVE To determine whether adding REM sleep behavior disorder (RBD) to the dementia with Lewy bodies (DLB) diagnostic criteria improves classification accuracy of autopsy-confirmed DLB. METHODS We followed 234 consecutive patients with dementia until autopsy with a mean of 4 annual visits. Clinical diagnoses included DLB, Alzheimer disease (AD),(More)
Pain and disability are cardinal symptoms in osteoarthritis. The literature is reviewed in order to identify causes of these symptoms at the articular, kinesiological, and psychological level. It is concluded that pain and disability are associated with degeneration of cartilage and bone (articular level), with muscle weakness and limitations in joint(More)
OBJECTIVE To report the clinical, electroencephalographic, and neuroradiologic findings in a kindred with a novel insertion in the prion protein gene, PRNP. DESIGN Clinical description of a kindred. SETTING Mayo Clinic Alzheimer Disease Research Center (Rochester, Minnesota). SUBJECTS Two pathologically confirmed cases and their relatives. MAIN(More)
STUDY OBJECTIVES Sleep deprivation and daytime somnolence impair numerous aspects of physical, cognitive, and memory performance. However, most studies examining the effect of somnolence on brain function focus on acute sleep restriction in young adults. We examine the relationship between chronic daytime somnolence and connectivity in six brain networks in(More)
OBJECTIVE To report the phenotypic characterization of monozygotic twins with mutations encoding progranulin (PGRN). METHODS We studied a twin pair with an exon 4 gene deletion in the PGRN gene. Both twins had clinical and neuropsychological examinations as well as structural MRI and fluorodeoxyglucose PET (FDG-PET) scans. PGRN gene sequencing was(More)
In this paper we question the guidance offered to neurologists by the Ethics, Law and Humanities Committee of the American Academy of Neurology (Larriviere, Williams, Rizzo & Bonnie, 2009) on how to respond to requests for "neuroenhancement": the use of pharmaceuticals to enhance cognitive function in cognitively normal people. The guidance assumes that the(More)