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  • Contact me when this article is cited  Related Content  • Similar articles in this journal  Social Bookmarking   What's this?

Andrew G. Engel, MD; Robert G. Siekert, MD

Arch Neurol. 1972;27(2):174-181.

Abstract
A 19-year-old girl had had slight muscle weakness before age 18. In her 19th year, she became progressively weaker and eventually bedfast; she then needed respiratory support. Results of liver function and blood coagulation studies were abnormal when the patient was at her weakest. An almost complete remission occurred after seven weeks of prednisone therapy (60 to 27.5 mg/ day); there was relapse 21 months later with lower dosages (10 to 20 mg/day). With higher dosages, the patient again improved but did not regain her strength fully. In three muscle biopsy specimens, there were myriads of abnormal spaces, especially in type I fibers, containing neutral fat and fatty acids. The histologic abnormalities were less marked during remission than just before the start of therapy or during relapse. The metabolic defect in the present case remains unidentified.

Author Affiliations
Rochester, Minn

From the Mayo Clinic and Mayo Foundation, Department of Neurology and Neuromuscular Research Laboratory, Rochester, Minn.

Footnotes
Accepted for publication Feb 24, 1972.

Reprint requests to Mayo Clinic, 200 First St SW, Rochester, Minn 55901 (Dr. Engel).

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

THIS ARTICLE HAS BEEN CITED BY OTHER ARTICLES

Lipid Storage Myopathy in Familial Hyperlipoproteinemia
Kinoshita and Wakata
Arch Neurol 1984;41:551-554.
ABSTRACT  

Familial Systemic Carnitine Deficiency
Cruse et al.
Arch Neurol 1984;41:301-305.
ABSTRACT  

Carnitine Metabolism and Its Application in Parenteral Nutrition
Tao and Yoshimura
JPEN J Parenter Enteral Nutr 1980;4:469-486.
 

Hepatitis-Associated Lipid Storage Myopathy
PATTEN et al.
ANN INTERN MED 1977;87:417-421.
ABSTRACT  

Reversible Vacuolar Myopathy of Type II Fibers
Hanson and Balint
Arch Neurol 1975;32:830-833.
ABSTRACT  

Muscle Carnitine Deficiency: Association With Lipid Myopathy, Vacuolar Neuropathy, and Vacuolated Leukocytes
Markesbery et al.
Arch Neurol 1974;31:320-324.
ABSTRACT  

Carnitine Deficiency Myopathy
S.V.
JAMA 1973;225:165-166.
ABSTRACT  

Carnitine Deficiency of Human Skeletal Muscle with Associated Lipid Storage Myopathy: A New Syndrome
Engel and Angelini
Science 1973;179:899-902.
ABSTRACT