Muscular dystrophy — A new approach

Muscular dystrophy — A new approach

S101 execution of cat locomotory movements, i n d i v i d u a l motoneurons in each pool are recruited in a reproducible and orderly way, from small ...

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S101

execution of cat locomotory movements, i n d i v i d u a l motoneurons in each pool are recruited in a reproducible and orderly way, from small to large. I . Hoffer et a l . , Science 213:466, 1981 2. Hoffer et a l . , J.Neurosci.Methods 4:211, 1981

F138 MUSCULARDYSTROPHY - A NEW APPROACH HOFMANN,W.W., Dept. of Neurology, Stanford U n i v e r s i t y School of Medicine and VA Medical Center, 38oi Miranda Avenue, Palo A l t o , C a l i f o r n i a 943o5, USA Increased protein turnover rates and transmembrane "leaks" of proteins are well documented features of muscle c e l l s in murine dystrophy. The muscle atrophy may r e s u l t from i n a b i l i t y to fuel the c e l l s with e i t h e r amino acids or s u f f i c i e n t energy from glucose uptake. A potent regulator of transport f o r both protein precursors and glucose is i n s u l i n , which acts by combining with a surface l i gand or receptor. A question in these experiments was whether dystrophic muscles bind normal amounts of the hormone and whether amino acid and glucose transport could be stimulated in the normal way. Soleus muscles from 129/ l e ~ d y mice and matched controls were tested in v i t r o for 125 i n s u l i n binding, C -2 deoxyglucose (2-DG) uptake, and Cl4 aminoisobutyric acid (AIB) uptake. The findings indicate that the dystrophic muscles have about 2/3 the i n s u l i n displaceable binding of controls and that amino acid transport is increased at rest but not f u r t h e r enhanced by i n s u l i n . Stimulation of (2-DG) uptake by i n s u l i n was about I / 4 c o n t r o l , but baseline transport appeared normal. These results suggest that membrane regulation of metabolites may be defective in dystrophy. The long known membrane abnormalities in the disorder may thus i n clude changes or decreases in i n s u l i n binding s i t e s .

F139 CARPALTUNNEL SYNDROME: A NEUROPHYSIOLOGICALSTUDY OF EFFECTS OF TWO TYPES OF OPERATION HOLMGRENH, RABOWL, LESNIEWSKI W, LINDEN UC, THORLING Jl~ Departments of Neurophysiology, Neurosurgery and Orthopedic Surgery, U n i v e r s i t y Hospital, S-581 85 Link~ping, Sweden In 48 patients with c l i n i c a l and neurophysiological signs of carpal tunnel syndrome were c l i n i c a l l y tested sensory disturbances, atrophy and motor weakness. Subjective estimation of d i s a b i l i t y was also noted. Objectively were tested v i b r a t i o n and touch thresholds, signs of denervation, changes of motor u n i t , conduction of middle f i n g e r sensory f i b e r s and motor conduction. The patients were randomized into two groups. One group was operated with transsection of the carpal ligament only, and the other with the same procedure combinded with an internal neurolysis under the microscope. Follow-up studies were carried out four weeks and six months a f t e r operation. Six months a f t e r operation approximately 85% of the patients in each group considered themselves almost completely r e s t i t u t e d . In the ligament d i v i s i o n (L)group d i s t a l motor latency improved from 5.1 ± 1.7 ms preoperatively, to 3.6 + 0.6 ms and from 4.9 ± 1.5 ms to 3.9 ± 1.2 ms in the neurolysis (N)-group. In the L-group the sensory terminal latency improved from 3.8 ± 0.9 ms to 3.2 + 0.7 ms, and from 3.8 + 0.9 ms to 3.2 ± 0.7 ms in the N-group. In the L-gro-up the threshold for ITght touch was increased in 58% and was a f t e r six months s t i l l abnormal in 26% of the patients. In the N-group the respective