Cardiac sympathetic hypo-innervation in familial dysautonomia
نویسندگان
چکیده
منابع مشابه
Familial dysautonomia.
Familial dysautonomia is a rare syndrome of childhood affecting the nervous system. As the name suggests, dysfunction of the autonomic system is a prominent feature. It was first recognized as a separate entity by Riley, Day, Greeley and Langford (1949), the first large series being described by Riley (1952). So far, about 70 patients have been recorded. Though the fully developed syndrome is u...
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BACKGROUND In postural tachycardia syndrome (POTS) and repeated neurocardiogenic presyncope (NCS), orthostatic intolerance occurs without persistent sympathetic neurocirculatory failure. Whether these conditions involve abnormal cardiac sympathetic innervation or function has been unclear. METHODS AND RESULTS Patients with POTS or NCS underwent measurements of neurochemical indices of cardiac...
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In familial dysautonomia (FD), i.e., Riley-Day syndrome, parasympathetic dysfunction has not been sufficiently evaluated. The cold face test is a noninvasive method of activating trigeminal brain stem cardiovagal and sympathetic pathways and can be performed in patients with limited cooperation. We performed cold face tests in 11 FD patients and 15 controls. For 60 s, cold compresses (0-1 degre...
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Metaiodobenzylguanidine (MIBG), a noradrenaline analogue,labelled with radioactive iodine I-123 is used for assessing pre-synaptic activity of the myocardial adrenergic system. The paper describes the mechanism of myocardial I-123-MIBG uptake and the relevant examination methods. It also refers to the necessity of standardising acquisition methods and assessment criteria in order to improve com...
متن کاملFamilial Dysautonomia (FD)
Familial dysautonomia (FD) is an autosomal recessive congenital neuropathy which results from poor development and progressive degeneration of the nervous system. The mutation responsible for FD was found at the 5�ss of intron 20 of the IKBKAP gene, encoding the I?B kinase complex-associated protein (IKAP...
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ژورنال
عنوان ژورنال: Clinical Autonomic Research
سال: 2008
ISSN: 0959-9851,1619-1560
DOI: 10.1007/s10286-008-0464-1