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Bloch-Sulzberger Syndrome (Incontinentia Pigmenti)Report of a Case with Prominent Neurological Features
ARCHIE McPHERSON, M.D.;
THOMAS L. AUTH, M.D.
Arch Neurol. 1963;8(3):332-339.
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The Bloch-Sulzberger syndrome is a manyfaceted syndrome which has received attention primarily for its dermatological manifestations. The manifestations are in 3 phases: an initial papular rash, a verrucous stage, and a final pigmented lesion.
However, it appears that in addition to the well-described skin pathology the syndrome exhibits widespread lesions in many organs and systems. It may present neurologically in many forms, from mild lifelong mental retardation to severe life-threatening seizures in the nursery. In addition, ocular, dental, integumental, hematological, and orthopedic defects have been described.
This disorder has received scant attention in the neurological literature. We, there-fore, feel that this should be brought more directly to neurologists' attention, since it can be recognized at different stages in early life and needs to be included in the differential diagnosis of neonatal convulsions. The recognition of the disorder may prevent the performance of many unnecessary diagnostic and therapeutic maneuvers.
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Author Affiliations
WASHINGTON, D.C.
Footnotes
Received for publication Oct. 31, 1962.
Presented at the meeting of the Section on Neurology, Neurosurgery and'Psychiatry, Medical Society of the District of Columbia, June 6, 1962.
Resident in Neurology (Dr. McPherson), Georgetown University Medical Center; Assistant Professor of Neurology, Department of Neurology, Georgetown University School of Medicine (Dr. Auth).
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