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Dissociated Sensation in AmyloidosisCompound Action Potential, Quantitative Histologic and Teased-Fiber, and Electron Microscopic Studies of Sural Nerve Biopsies
Peter James Dyck, MD;
Edward H. Lambert, MD, PhD
Arch Neurol. 1969;20(5):490-507.
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IN A perspicuous report in 1952, Andrade1 described the natural history of dominantly inherited amyloidosis. The hallmarks of the condition were (1) dominant inheritance, (2) onset in the second or third decade, (3) unremitting course to death, due to cachexia and infection, an average of seven to ten years later, and (4) characteristic pattern of symptoms. Affected persons exhibited paresis, early impairment of thermal and pain sensibilities, and anhidrosis in the lower limbs. Concomitant symptoms were nausea, vomiting, abdominal pains, and sexual and sphincter disorders. A unique feature was the dissociated sensory loss. Andrade found that the various modalities of sensation were lost in the following order: temperature, pain, touch, and, finally, joint position. On postmortem examination, amyloid deposits were found in large amounts in kidneys and peripheral nerves and to a lesser extent in other organs. No adequate anatomic or physiologic explanation for the dissociated sensory loss
. . . [Full Text PDF of this Article]
Author Affiliations
Rochester, Minn
From the Mayo Clinic and Mayo Foundation: Section of Neurology (Dr. Dyck) and Physiology (Dr. Lambert).
Footnotes
Submitted for publication Sept 12, 1968; accepted Oct 16.
Reprint requests to Mayo Clinic, 200 First St, SW, Rochester, Minn 55901 (Dr. Dyck).
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