Hypothalamic dysfunction and neuroendocrine and metabolic alterations in Huntington's disease: clinical consequences and therapeutic implications.
Huntington's disease (HD) is a hereditary neurodegenerative disorder characterized by cognitive, psychiatric, behavioural and motor disturbances. Although the course of HD is also frequently complicated by unintended weight loss, sleep disturbances and autonomic nervous system dysfunction, the aetiology of these signs and symptoms remains largely unknown. In recent years, many novel findings from both animal and human studies have emerged that indicate considerable hypothalamic, endocrine and metabolic alterations in HD. However, a comprehensive overview of these findings is lacking and their precise clinical significance is far from clear. Therefore, in this review we attempt to put these recent developments in the field into perspective by integrating them with previous findings in a comprehensible manner, and by discussing their clinical relevance, with a special focus on body weight, sleep and autonomic functions in HD, which will also allow for the identification of future lines of research in this area.
Aziz
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NA
Department of Neurology, Leiden University Medical Centre, Leiden, The Netherlands. N.A.Aziz.Neur@lumc.nl
Swaab
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DF
Pijl
H
H
Roos
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RA
D000818:Animals; D001342:Autonomic Nervous System Diseases / Q000209:etiology / Q000378:metabolism / Q000503:physiopathology*; D004700:Endocrine System Diseases / Q000209:etiology / Q000378:metabolism / Q000503:physiopathology*; D006801:Humans; D006816:Huntington Disease / Q000150:complications / Q000378:metabolism / Q000503:physiopathology*; D007027:Hypothalamic Diseases / Q000209:etiology / Q000378:metabolism / Q000503:physiopathology*; D007030:Hypothalamo-Hypophyseal System / Q000378:metabolism / Q000503:physiopathology; D008659:Metabolic Diseases / Q000209:etiology / Q000378:metabolism / Q000503:physiopathology; D009490:Neurosecretory Systems / Q000378:metabolism / Q000503:physiopathology; D012893:Sleep Wake Disorders / Q000209:etiology / Q000378:metabolism / Q000503:physiopathology