DYSAUTONOMIA (Familial Dysauto- nomia)

Dysfunction of the autonomic nervous system, believed to result from an obscure brain defect. The condition tends to run in families and is sometimes confused with childhood schizophrenia and mental retardation.The dysautonomic child is tense, irritable, cannot adapt to change, and has difficulty performing complex motor or perceptual tasks. He is also likely to be emotionally unstable and to manifest a variety of physical disorders such as inability to shed tears, fluctuation of temperature, relative insensitivity to pain, and hyperhidrosis (excessive sweating). Barbiturate sedatives are effective in alleviating the physical symptoms in some cases, especially those in which the sympathetic portion of the autonomic system is overactive.In a recent study by Sak et al. (1967) the performance of twenty-five dysauto- nomic children referred by the Dys- autonomia Association was compared with that of a control group of ten normal children on four tests: the Wechsler Scales (five verbal, five nonverbal subtests), the Bender-Gestalt (copying nine figures and reproducing them from memory), the Goodenough Draw-A-Person Test (drawing a figure, then another of the opposite sex, and telling a story about them), and the Benton Left-Right Discrimination Test (performing some simple tasks that indicate a preference for one eye, hand, or leg).On the full scale Wechsler, the dys- autonomic children scored in the low average range (averaging 86.4 versus 118 for the controls), with the most pronounced retardation in nonverbal skills. They usually (particularly the older children) scored best on the similarities subtest, indicating an ability to think abstractly, and worst on the object assembly human figure test, indicating a distorted self-image. The Bender- Gestalt figures were copied and recalled with great difficulty and many distortions (Fig. 15), with twenty-four of the twenty-five subjects falling below their age level, suggesting organic brain disorder.Performance on the Draw-A-Person- Test was considerably below the I.Q. on the Wechsler test, probably reflecting the generally poor scores on nonverbal subtests. Moreover, from a clinical point of view, the drawings were not only extremely immature but bizarre, and suggested a disturbance in self-image typical of emotionally disturbed and socially isolated individuals (Fig. 16). This result tied up with their performance on the face assembly subtest of the Wechsler and was also demonstrated on the Left-Right Discrimination Test. They were usually unable to identify right and left on the examiner, could not identify parts of their own bodies, and 65 per cent showed mixed dominance as compared with about 5 per cent among unselected boys and girls. In addition, a school survey of nineteen of the twenty-five dysautonomic children revealed that only two were able to maintain a normal position; the rest were either in special classes or had repeated at least one grade.In interpreting these findings, the authors suggest that the widespread sensory defects characteristic of this disease, as well as the highly emotional environment in which the children develop, both contribute to their poor performance. They believe there are indications of a central (brain) defect as well as a peripheral defect, and point out that recent evidence suggests that both types of deficit may be due in part at least to deficiencies in neurotransmission.