PEDIATRICS Vol. 14 No. 5 November 1954, pp. 475-480
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FURTHER OBSERVATIONS ON FAMILIAL DYSAUTONOMIA

CONRAD M. RILEY M.D.1, ALFRED M. FREEDMAN M.D.1, and WILLIAM S. LANGFORD M.D.1

1 The Babies Hospital and the Departments of Pediatrics and Psychiatry, College of Physicians and Surgeons, Columbia University, New York.

Studies of familial dysautonomia by a team made up of a pediatrician, a neurologist, speech therapists, psychiatrists, psychologists and a psychiatric social worker have been presented in summary form.

1. Facts supporting the theory that this is a condition of genetic origin are enumerated. They are compatible with its being transmitted by an autosomal recessive gene.

2. Evidence of disturbance of statural growth, with frequent significant dwarfing, is presented.

3. Postural hypotension as a regular part of the syndrome is demonstrated.

4. Data showing the variable neurological abnormalities are included.

5. The frequency of disturbance of speech, but not of language organization, is reported.

6. Psychological testing suggests a deterioration with advancing years in dealing with concrete tests, but no significant disturbance in abstract thinking.

7. At the psychosocial level, inadequate adjustment to daily life is seen in all patients. This difficulty is reflected in varying degree by emotional disturbance in the parents. A schematic concept of the dynamics is offered.

8. Drug treatment of the acute crisis is discussed and a plan for daily management of such cases is projected.

9. Avenues for further investigation are briefly mentioned.

Submitted on July 14, 1954




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