TY - JOUR
T1 - Lipid Storage Myopathy Responsive to Prednisone
AU - Engel, Andrew G.
AU - Siekert, Robert G.
PY - 1972/8
Y1 - 1972/8
N2 - 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.
AB - 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.
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U2 - 10.1001/archneur.1972.00490140078011
DO - 10.1001/archneur.1972.00490140078011
M3 - Article
C2 - 5040634
AN - SCOPUS:0015384205
SN - 0003-9942
VL - 27
SP - 174
EP - 181
JO - Archives of neurology
JF - Archives of neurology
IS - 2
ER -