Muscle computed tomography in adult-onset acid maltase deficiency.
Nine patients with adult-onset acid maltase deficiency (Pompe's disease) were examined clinically and with computed tomography (CT). The CT scan showed early and severe involvement of the muscles of trunk and thighs, with selective sparing of the tensor fasciae latae, short head of biceps femoris, gracilis, and sartorius muscles. Shoulder and leg muscles were less affected. The disease spread over the years from trunk to extremities. Muscle strength and CT findings were positively correlated.
de Jager
A E
AE
Department of Neurology, University Hospital Groningen, The Netherlands.
van der Vliet
T M
TM
van der Ree
T C
TC
Oosterink
B J
BJ
Loonen
M C
MC
D000328:Adult; D017668:Age of Onset; D018450:Disease Progression; D005260:Female; D006009:Glycogen Storage Disease Type II / Q000000981:diagnostic imaging / Q000503:physiopathology*; D006801:Humans; D008297:Male; D008875:Middle Aged; D018482:Muscle, Skeletal / Q000000981:diagnostic imaging* / Q000503:physiopathology; D014057:Tomography, X-Ray Computed; D000520:alpha-Glucosidases / Q000172:deficiency*
D000520:alpha-Glucosidases