TASTE AND SMELL IN FAMILIAL DYSAUTONOMIA

Familial dysautonomia (FD) is one of the classic diseases characterize d by taste and smell abnormalities, However, these typical features ar e based on data obtained from two separate crude studies published in 1964, In the present study psychophysical-cognitive and reflex-like fa cial-behavioral responses to taste and smell, in nine patients with FD and 15 healthy controls, were recorded, Five taste stimulants were pr esented to both study groups, while a selection of common household od ors was used for FD patients only. The patients with PD showed a marke dly higher incidence of recognition failures for salty, bitter, sweet, and water stimuli than the controls, but rate of recognition of sour stimuli was almost identical in the two groups. Estimates by the subje cts on a hedonic scale of 0 to 10 and facial display in FD indicated a relatively normal sensitivity to sour stimuli and to a lesser extent to bitter stimuli. Water, sweet, and salty stimuli evoked non-discrimi natory responses, These findings indicate specific dysgeusia rather th an general ageusia. Smell was found to be normal. In children with tas te and smell impairment, a systematic evaluative approach may help in planning palatable diets for adequate and comfortable nutrition.