Congenital muscular dystrophy (Fukuyama type)--changes in the white matter low density on CT.
Sixty-two computed tomographic (CT) scans of 36 patients with congenital muscular dystrophy of Fukuyama type (FCMD) were analysed. A low density area in the cerebral white matter was characteristic of FCMD and a special reference was made to the changes in the white matter low density. It was present in 15 patients out of 36 (42%) and frequently seen in the scans obtained on the younger patients; among 11 scans taken of patients between 1 and 2 years of age, 10 scans (91%) showed the white matter low density. Repeated CT scans were carried out on 26 of the 36 cases. Follow-up study revealed that the white matter low density areas were most apparent around the age of one year and decreased or disappeared at 2 or 3 years of age. From these observations, delayed myelination was suspected for the pathogenesis of the low density area found in FCMD.
Yoshioka
M
M
Department of Pediatrics, Kobe General Hospital, Japan.
Saiwai
S
S
D000293:Adolescent; D000328:Adult; D000367:Age Factors; D001921:Brain / Q000000981:diagnostic imaging*; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D009136:Muscular Dystrophies / Q000000981:diagnostic imaging*; D014057:Tomography, X-Ray Computed