REM sleep behavior disorder in a patient with frontotemporal dementia. 2012

Daniele Lo Coco, and Chiara Cupidi, and Alfredo Mattaliano, and Valentina Baiamonte, and Sabrina Realmuto, and Emanuele Cannizzaro
Sleep Disorders Clinic, Dipartimento di Neuroscienze, Ospedale Civico ARNAS, Piazza N. Leotta 4, 90127 Palermo, Italy. danielelococo@yahoo.com

We describe a patient with frontotemporal dementia (FTD), a tauopathy, who also showed clinical and polysomnographic features of REM sleep behavior disorder (RBD). The patient is a 78-year-old male with a 1 year history of behavioral dysfunction involving emotion, character and social functioning. Brain imaging and the results of neuropsychological testing were consistent with a diagnosis of FTD. Sleep symptom onset occurred some years before the behavioral changes, and consisted of unpleasant dreams, vocalization, and prominent motor behaviors. A polysomnography confirmed the diagnosis of RBD. Our findings support the hypothesis that RBD, although more frequent in synucleinopathies, might be a pathological stage in the development of almost every neurodegenerative disorder in which the pathological process involves the cerebral structures that regulate muscle tone during REM sleep.

UI MeSH Term Description Entries
D008297 Male Males
D004576 Electromyography Recording of the changes in electric potential of muscle by means of surface or needle electrodes. Electromyogram,Surface Electromyography,Electromyograms,Electromyographies,Electromyographies, Surface,Electromyography, Surface,Surface Electromyographies
D004585 Electrooculography Recording of the average amplitude of the resting potential arising between the cornea and the retina in light and dark adaptation as the eyes turn a standard distance to the right and the left. The increase in potential with light adaptation is used to evaluate the condition of the retinal pigment epithelium. EOG,Electrooculograms,Electrooculogram
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000368 Aged A person 65 years of age or older. For a person older than 79 years, AGED, 80 AND OVER is available. Elderly
D057180 Frontotemporal Dementia The most common clinical form of FRONTOTEMPORAL LOBAR DEGENERATION, this dementia presents with personality and behavioral changes often associated with disinhibition, apathy, and lack of insight. DDPAC,Dementia, Frontotemporal,Dementia, Frontotemporal, with Parkinsonism,Dementia, Hereditary Dysphasic Disinhibition,Disinhibition-Dementia-Parkinsonism-Amyotrophy Complex,Disinhibition-Dementia-Parkinsonism-Amytrophy Complex,FTD-GRN,FTD-PGRN,FTDP-17,FTLD with TDP-43 Pathology,FTLD-17 GRN,FTLD-TDP,Familial Pick's Disease,Frontotemporal Dementia with Parkinsonism,Frontotemporal Dementia with Parkinsonism-17,Frontotemporal Dementia, Ubiquitin-Positive,Frontotemporal Lobar Degeneration With Ubiquitin-Positive Inclusions,Frontotemporal Lobe Dementia,Frontotemporal Lobe Dementia (FLDEM),GRN-Related Frontotemporal Dementia,HDDD1,HDDD2,Hereditary Dysphasic Disinhibition Dementia,Multiple System Tauopathy with Presenile Dementia,Semantic Dementia,Wilhelmsen-Lynch Disease,Complex, Disinhibition-Dementia-Parkinsonism-Amyotrophy,Complex, Disinhibition-Dementia-Parkinsonism-Amytrophy,Complices, Disinhibition-Dementia-Parkinsonism-Amyotrophy,Complices, Disinhibition-Dementia-Parkinsonism-Amytrophy,Dementia, Frontotemporal Lobe,Dementia, Frontotemporal Lobe (FLDEM),Dementia, GRN-Related Frontotemporal,Dementia, Semantic,Dementia, Ubiquitin-Positive Frontotemporal,Dementias, Frontotemporal,Dementias, Frontotemporal Lobe,Dementias, Frontotemporal Lobe (FLDEM),Dementias, GRN-Related Frontotemporal,Dementias, Semantic,Dementias, Ubiquitin-Positive Frontotemporal,Disease, Familial Pick's,Disease, Wilhelmsen-Lynch,Diseases, Familial Pick's,Diseases, Wilhelmsen-Lynch,Disinhibition Dementia Parkinsonism Amyotrophy Complex,Disinhibition Dementia Parkinsonism Amytrophy Complex,Disinhibition-Dementia-Parkinsonism-Amyotrophy Complices,Disinhibition-Dementia-Parkinsonism-Amytrophy Complices,FTLD with TDP 43 Pathology,Familial Pick Disease,Familial Pick's Diseases,Familial Picks Disease,Frontotemporal Dementia with Parkinsonism 17,Frontotemporal Dementia, GRN-Related,Frontotemporal Dementia, Ubiquitin Positive,Frontotemporal Dementias,Frontotemporal Dementias, GRN-Related,Frontotemporal Dementias, Ubiquitin-Positive,Frontotemporal Lobar Degeneration With Ubiquitin Positive Inclusions,Frontotemporal Lobe Dementias,Frontotemporal Lobe Dementias (FLDEM),GRN Related Frontotemporal Dementia,GRN-Related Frontotemporal Dementias,Lobe Dementia, Frontotemporal,Lobe Dementias, Frontotemporal,Pick's Disease, Familial,Pick's Diseases, Familial,Semantic Dementias,Ubiquitin-Positive Frontotemporal Dementia,Ubiquitin-Positive Frontotemporal Dementias,Wilhelmsen Lynch Disease,Wilhelmsen-Lynch Diseases
D020187 REM Sleep Behavior Disorder A disorder characterized by episodes of vigorous and often violent motor activity during REM sleep (SLEEP, REM). The affected individual may inflict self injury or harm others, and is difficult to awaken from this condition. Episodes are usually followed by a vivid recollection of a dream that is consistent with the aggressive behavior. This condition primarily affects adult males. (From Adams et al., Principles of Neurology, 6th ed, p393) Rapid Eye Movement Sleep Behavior Disorder,Behavior Disorder, REM,Behavior Disorder, Rapid Eye Movement Sleep,REM Behavior Disorder,Behavior Disorders, REM,REM Behavior Disorders

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