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Familial dysautonomia is a rare disease of childhood first described by Riley et al. in 1949 (8). It presents in blatant fashion symptoms generally thought of as physical and emotional. The disease itself with its vomiting attacks, self-destructive rages, vacillating hypertension, skin changes, lack of tears, and high mortality is a fascinating subject for study. Its therapy, in a truly pioneering stage, from both pediatric and psychodynamic points of view, is full of problems and unanswered questions. Research in dysautonomia raises dramatic possibilities for comprehensive investigation. To date there have been 48 cases of this disease seen at Babies' Hospital. A summary report of the findings related to these cases made by a team consisting of a pediatrician, a neurologist, speech therapists, psychiatrists, psychologists and psychiatric social worker is to be found in an article entitled, “Further Observations on Familial Dysautonomia” (9).
Dysautonomia is a disease of childhood occurring in children of Jewish extraction. Its chief signs and symptoms are lack of tears, labile blood pressure, vomiting attacks, retarded growth and development, and emotional outbursts. Postural hypotension, pulmonary infection, unexplained fever, urinary frequency, and breath-holding spells in infancy are other findings. Riley et al. (9) conclude in a recent report that the findings of dysautonomia are a product of “disturbed autonomic homeostasis” and that there is in addition “a diffuse involvement of the central nervous system” such as is manifested in difficulty in motor coordination with retarded motor development, absent or diminished deep tendon reflexes and relative indifference to pain. These symptoms are not necessarily evidence of disease outside of the autonomic nervous system, inasmuch as this system is represented at every level of the nervous system and is to be found overlapping sensory and somatic fields. The disease occurs in 25 to 35 percent of the siblings of patie