Document Detail

Diurnal blood pressure variation and hormonal correlates in fatal familial insomnia.
MedLine Citation:
PMID:  8175163     Owner:  NLM     Status:  MEDLINE    
Fatal familial insomnia is a prion disease in which a selective thalamic degeneration leads to total sleep deprivation, hypertension, dysautonomia, adrenal overactivity, and impaired motor functions. With patients under continuous recumbency and polysomnographic control, we assessed the changes in the 24-hour patterns of blood pressure, heart rate, plasma catecholamines, corticotropin, and serum cortisol in three patients at different stages of the disease. Six healthy volunteers were used as control subjects. A dominant 24-hour component was detected at rhythm analysis of all variables, both in patients and control subjects. In the patients, the amplitudes gradually decreased as the disease progressed, leading to the obliteration of any significant dirunal variation only in the preterminal stage. A shift in phase corresponded to the loss of the nocturnal fall in blood pressure in an early stage of the disease, when nocturnal bradycardia was still preserved. Plasma cortisol was high and became increasingly elevated, whereas corticotropin remained within normal levels; abnormal nocturnal peaks appeared in their circadian patterns. The disrupted patterns of cortisol and blood pressure preceded the development of hypertension and severe dysautonomia, which in turn were paralleled by increasing catecholamine and heart rate levels. Our data demonstrate that in patients with fatal familial insomnia the changes detectable in the rhythmic component of diurnal blood pressure variability result in a pattern of secondary hypertension. Disturbances in thalamic, pituitary-adrenal, and autonomic functions seem to be involved in mediating these changes.
F Portaluppi; P Cortelli; P Avoni; L Vergnani; M Contin; P Maltoni; A Pavani; E Sforza; E C degli Uberti; P Gambetti
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Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't    
Journal Detail:
Title:  Hypertension     Volume:  23     ISSN:  0194-911X     ISO Abbreviation:  Hypertension     Publication Date:  1994 May 
Date Detail:
Created Date:  1994-06-07     Completed Date:  1994-06-07     Revised Date:  2006-11-15    
Medline Journal Info:
Nlm Unique ID:  7906255     Medline TA:  Hypertension     Country:  UNITED STATES    
Other Details:
Languages:  eng     Pagination:  569-76     Citation Subset:  IM    
Hypertension Unit, University of Ferrara, Italy.
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MeSH Terms
Adrenocorticotropic Hormone / blood*
Blood Pressure*
Circadian Rhythm*
Heart Rate
Hydrocortisone / blood*
Middle Aged
Prion Diseases / physiopathology*
Reg. No./Substance:
50-23-7/Hydrocortisone; 9002-60-2/Adrenocorticotropic Hormone

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