Objective - To analyse the regenerating capability of the peripheral nerve
fibers and the capability of the muscle fibers to accept the regenerating a
nd new nerve sprouts in myotonic dystrophy (MD). Material and methods - One
male, aged 58 years, diagnosed of MD at the age or 30 years, suffered neur
algic amyotrophy in the right shoulder girdle 4 weeks before admission. Nee
dle EMG and nerve conduction studies were performed on admission and 6, 12,
and 18 months later. Results - Oil admission there were atrophy and absenc
e of voluntary contraction in deltoids, supra- and infraspinatus muscles. E
MG showed abundant fibrillations, positive sharp waves and myotonic bursts
in these Muscles without voluntary activity, consistent with axonal neuropa
thy of both axillary and suprascapular nerves. The follow-up showed signs o
f reinnervation 6 months later and slight loss of long duration and high am
plitude MUPs at 18 months of evolution, with good clinical recovery. This i
s compatible with chronic neurogenic atrophy, presumably as ail expression
of type grouping. Conclusions - The reinnervation capability of the nerve f
ibers and the capability of muscle fibers membrane to accept regenerating a
nd new nerve sprout remain in MD. Myotonic bursts persist after total dener
vation.