This Article  • References  • Full text PDF  • Reply to article  • Send to a friend  • Save in My Folder  • Save to citation manager  • Permissions  Citing Articles  • Citation map  • Citing articles on HighWire  • Citing articles on Web of Science (79)  • Contact me when this article is cited  Related Content  • Similar articles in this journal  Social Bookmarking   What's this?

A. N. RAHMAN, MD; R. LINDENBERG, MD

Arch Neurol. 1963;9(4):373-385.

	Since this article does not have an abstract, we have provided the first 150 words of the full text PDF and any section headings.

Introduction

Angiokeratoma corporis diffusum universale, formerly designated a dermatological condition, is now recognized as a lipid storage disease.^1-3 Recently the term hereditary dystopic lipidosis was introduced to denote the underlying metabolic abnormality.² The symptomatology of this disease has been reported elsewhere.^2,17-25 In brief, the cardinal manifestations of the disease are paroxysmal crises consisting of attacks of fever, burning pains and paresthesia in extremities, and fleeting proteinuria in childhood. A macular and papular erythematous skin eruption heralds the onset of these symptoms in males. In young adults there are few crises, but a mild diabetes insipidus, diminished sweating, and persistent proteinuria are characteristic. Later in life anhidrosis, hypertension, cardiomegaly, and progressive renal insufficiency with uremia are common.

Involvement of the nervous system in this disease by lipid storage in ganglion cells was first described by Scriba³ in 1951. He noted swelling of neurons of peripheral autonomic ganglia . . . [Full Text PDF of this Article]

Author Affiliations
BALTIMORE

Departments of Medicine, The Johns Hopkins University School of Medicine, Baltimore, and the Western Reserve University School of Medicine at Cleveland Metropolitan General Hospital, Cleveland, and Central Anatomic Laboratory of the Maryland State Department of Mental Hygiene.

Footnotes
Submitted for publication March 23, 1963; accepted May 10, 1963.

This work was carried out in part during the tenure of a Special Fellowship BT-591 (C2) from the National Institute of Neurological Diseases and Blindness, Public Health Service (Dr. Rahman).

Supported in part by USPHS Grant E1230.

CiteULike    Connotea    Del.icio.us    Digg    Reddit    Technorati    Twitter     What's this?