INCREASED MAJOR SALIVARY-GLAND SECRETION IN FAMILIAL DYSAUTONOMIA

Familial dysautonomia (FD) an autosomal recessive peripheral nervous s ystem disorder, affects almost exclusively children of Jewish Ashkenaz i origin and causes profound generalized autonomic dysfunction. Excess ive drooling is frequent and is traditionally attributed to swallowing difficulties. Although true hypersalivation has bren postulated, no q uantitative assessment of the salivary secretion rate has yet been rep orted. The authors determined this rate in 13 children with FD and 28 healthy controls. Resting parotid, submandibular/sublingual and unstim ulated whole salivary secretion rates were significantly elevated in c hildren with FB. The known relation of salivary function with age was found in controls only. This apparently major contribution of salivary hyperfunction to excessive drooling in FD may be attributable ts sali vary gland denervation supersensitivity, as this mechanism is present in the cardiovascular system and the pupil in FD.