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A Syndrome of the Neural Crest
JASON W. BROWN, MD;
ROBERT PODOSIN, MD
Arch Neurol. 1966;15(3):294-301.
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| Since this article does not have an abstract, we have provided the first 150 words of the full text PDF and any section headings. |
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IN THE past few years a number of cases have been described under the general heading of congenital insensitivity to pain with anhidrosis. In the present article an additional case is reported and a defect in neural crest differentiation proposed as the common underlying embryonic abnormality. The syndrome consists of the following: (1) loss of deep and/or superficial pain sensibility; (2) autonomic dysfunction manifested by (a) pupillary abnormalities ranging from a partial to complete bilateral Horner's syndrome, (b) neurogenic anhidrosis with otherwise normal sweat glands, (c) vasomotor instability with abnormal vanillylmandelic and homovanillic acid urine assays; (3) aplasia of dental enamel; (4) meningeal thickening and cystic change; (5) mild mental retardation; (6) hyporeflexia; (7) blond hair, blue or blue-green eyes, and fair complexion.
Report of a Case
A five-year-old, white boy of Scandinavian extraction was first hospitalized at Childrens Hospital, Los Angeles, on Aug 10, 1965, with a presenting complaint
. . . [Full Text PDF of this Article]
Author Affiliations
LOS ANGELES
From the Division of Neurology, UCLA Medical Center (Dr. Brown), and the Division of Neurology, University of Southern California School of Medicine Childrens Hospital (Dr. Podosin), Los Angeles.
Footnotes
Submitted for publication April 21, 1966; accepted May 19.
Reprint requests to Department of Neurology, UCLA Medical Center, Los Angeles 90024 (Dr. Brown).
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