RESUMO
Overestimation of blood pressure (BP) resulting from medial artery calcification (MAC) is a cause of pseudoresistant hypertension. In this condition, there is no noninvasive way to reliably assess the BP. We report the case of a 62-year-old man who had a four-limb MAC, hypertension despite five antihypertensive molecules, and significant orthostatic hypotension following the addition of the fifth drug. Hypotension symptoms resolved after discontinuation of two treatments. Subsequently, measurement of finger SBP (FSBP) by laser Doppler flowmeter (LDF) was used to estimate upper-limb SBP. While the brachial SBP was greater than 170âmmHg, the FSBP was always less than 140âmmHg. In the absence of upper-limb artery disease, by evidencing the discrepancy between FSBP and brachial SBP, LDF-obtained FSBP could be a reliable tool to ascertain pseudohypertension and monitor the antihypertensive treatment.
RESUMO
BACKGROUND AND PURPOSE: Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is one of the most devastating cerebral small vessel diseases. However, despite its progression with aging, some patients remain neurologically intact (Nint) even when they get older. Their main characteristics are poorly known. We aimed to delineate their clinical, imaging, and molecular features. METHODS: Individuals aged over 65 years were selected from a cohort of 472 CADASIL patients. Subjects who had no focal deficit, cognitive impairment, or disability were considered Nint. Their demographic, genetic, clinical, and imaging features were compared to those with permanent neurological symptoms (Nps). RESULTS: Among 129 patients, 23 (17.8%) individuals were considered Nint. The frequency of vascular risk factors and NOTCH3 cysteine mutations in epidermal growth factor-like repeat (EGFr) domains 7-34 did not differ between Nint and Nps patients but Nint patients had less stroke events and were more likely to have migraine with aura. The number of lacunes and microbleeds and degree of brain atrophy were lower in the Nint group, but the volume of white matter hyperintensities did not differ between the two groups. CONCLUSIONS: Nearly one in five CADASIL patients can remain Nint after the age of 65 years. Their clinical and imaging profile differed from that of other age-matched CADASIL patients. The location of NOTCH3 mutation inside or outside EGFr domains 1-6 cannot fully explain this discrepancy. The factors involved in their relative preservation of brain tissue from severe damage despite aging remain to be determined.