title: Systemic blood pressure profile in cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy.
abstract: OBJECTIVE
  Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is a genetic form of subcortical ischemic vascular dementia (SIVD). The most common vascular risk factors are unremarkable in CADASIL; however, studies on systemic blood pressure (BP) changes over time are substantially lacking. Because BP instability is a relevant risk factor for developing or worsening white matter changes in sporadic SIVD, we aimed to study the BP profile of CADASIL to investigate its relationship with cognitive decline and white matter injury.
  
  
  METHODS
  Twenty-four-hour ambulatory BP monitoring was performed in a group of 14 CADASIL patients (12 males and 2 females) and in a group of 15 healthy age-matched control subjects. The following BP variables were compared between the 2 groups: mean daytime and nighttime systolic, diastolic, and mean arterial BP (SABPday, DABPday, and MABPday, and SABPnight, DABPnight, and MABPnight) and nocturnal percentage decline in arterial BP (%MABP reduction). Cognitive performances were tested by mini mental status examination (MMSE), and brain MRI was performed to extrapolate the T2-weighted lesion volume (LV) in each CADASIL patient. The 24-hour arterial BP variables were compared between CADASIL and controls. In addition, for CADASIL patients only, MMSE, LV, and age were compared with each pressure variable.
  
  
  RESULTS
  Patients with CADASIL showed a significant reduction (P<0.05) of SABPday, DABPday, MABPday and %MABP decline with respect to controls. In addition, MMSE of CADASIL subjects correlated significantly (P<0.0001) with daytime MABP.
  
  
  CONCLUSIONS
  The low systemic BP profile observed in CADASIL patients was specifically attributable to reduced diurnal BP values. This may further affect cerebral hemodynamics and increase the risk of cognitive impairment in these patients. The pathogenesis of abnormal BP profile in CADASIL remains to be clarified. It is likely that central and peripheral mechanisms controlling BP variations are involved.
authors.affiliation: Dipartimento di Scienze Neurologiche e del Comportamento, Università di Siena, Viale Bracci, 53100 Siena, Italy.
authors.forename: Alessandra
authors.initials: A
authors.lastname: Rufa
authors.forename: Maria Teresa
authors.initials: MT
authors.lastname: Dotti
authors.forename: Massimo
authors.initials: M
authors.lastname: Franchi
authors.forename: Maria Laura
authors.initials: ML
authors.lastname: Stromillo
authors.forename: Gabriele
authors.initials: G
authors.lastname: Cevenini
authors.forename: Silvia
authors.initials: S
authors.lastname: Bianchi
authors.forename: Nicola
authors.initials: N
authors.lastname: De Stefano
authors.forename: Antonio
authors.initials: A
authors.lastname: Federico
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D001794:Blood Pressure; D018660:Blood Pressure Monitoring, Ambulatory; D046589:CADASIL / Q000453:epidemiology / Q000503:physiopathology*; D002544:Cerebral Infarction / Q000453:epidemiology / Q000235:genetics / Q000503:physiopathology*; D002940:Circadian Rhythm; D015897:Comorbidity; D003863:Depression / Q000453:epidemiology; D005260:Female; D006801:Humans; D006937:Hypercholesterolemia / Q000453:epidemiology; D020138:Hyperhomocysteinemia / Q000453:epidemiology; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D008881:Migraine Disorders / Q000453:epidemiology; D012307:Risk Factors