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Primary dysautonomia: cerebral blood flow and hemodynamic findings. Case report.

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Abstract

In a man with orthostatic and effort syncopes due to primary dysautonomia, we measured cerebral blood flow (CBF) — by the 133-Xenon inhalation method — in supine and in sitting positions, and after the i.v. administration of Acetazolamide, a potent cereral vasodilator.

Heart rate (HR) and blood pressure (BP) were monitored over the 24 hours by a non-invasive device. The CBF was normal in supine position and significantly reduced when the patient was sitting. Despite che sympathetic denervation, good response to acetazolamide infusion was seen.

BP changed with the position of the subject according to gravity, and HR was unresponsive to orthostatic and effort stimuli.

Sommario

Abbiamo misurato il Flusso Ematico Cerebrale (FEC) — con il metodo inalatorio allo Xenon-133 — in clinostatismo, in posizione seduta e dopo la somministrazione e.v. di Acetazolamide, un potente vasodilatatore cerebrale, in un uomo affetto da Sindrome Disautonomica Primitiva con ipotensione severa, ortostatica e da sforzo.

Inoltre, abbiamo monitorato per 24 ore la Pressione Arteriosa (PA) e la Frequenza Cardiaca (FC) per mezzo di metodica non-invasiva.

I nostri risultati dimostrano che il FEC è ridotto in posizione seduta, ma è normale in clinostatismo, e che mantiene la capacità di reagire a stimoli vasodilatatori, nonostante la denervazione simpatica. La PA è risultata dipendente dalla forza gravitazionale, mentre la FC è “fissa”, cioè non risponde agli stimoli ortostatici e allo sforzo.

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Nobili, F., Francione, S., Marenco, S. et al. Primary dysautonomia: cerebral blood flow and hemodynamic findings. Case report.. Ital J Neuro Sci 11, 281–288 (1990). https://doi.org/10.1007/BF02333859

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  • DOI: https://doi.org/10.1007/BF02333859

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