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Familial dysautonomia is a hereditary sensory and autonomic neuropathy (HSAN type
III), expressed at birth, that is associated with reduced or absent pain and temperature
sensibilities, postural hypotension, absent baroreflex function and labile blood pressure
that increases markedly during emotional excitement (Norcliffe-Kaufmann et al., 2010).
Given the absent baroreflex function we tested the hypothesis that cardiac-locked
bursts of muscle sympathetic nerve activity (MSNA) are absent in familial dysautonomia.
Tungsten microelectrodes were inserted percutaneously into muscle or cutaneous fascicles
of the common peroneal nerve in 10 patients with FD. Spontaneous bursts of MSNA were
absent, but we found evidence of tonically firing sympathetic neurones that increased
during emotional arousal. Conversely, skin sympathetic nerve activity (SSNA) appeared
normal. We conclude that the loss of phasic bursts of MSNA and the loss of baroreflex
modulation of muscle vasoconstrictor drive contribute to the poor control of blood
pressure in familial dysautonomia.
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