This is a consecutive study on 28 patients who have been
diagnosed as having congenital muscular dystrophy at Jordan
University Hospital in the period from January 1990 to February
1997. Of 75 patients diagnosed as having muscle disease, 55
(73.3%) had muscular dystrophy. Of 55 muscular dystrophy
patients, 28 (50.9%) had congenital muscular dystrophy, 11 (20%)
had Duchenne muscular dystrophy, 9 (16.4%) had Becker muscular
dystrophy, 4 (7.3%) had myotonic dystrophy, 2 (3.6%) had
limb-girdle dystrophy, and 1 (1.8%) patient had
facioscapulohumeral dystrophy. Age of onset of symptoms of
congenital muscular dystrophy (hypotonia and weakness) was
documented antenatally or in the first few months in the
majority (92.9%) of patients. Parental consanguinity was
documented in 21 (75%) of congenital muscular dystrophy cases,
and family history of possible similar cases in 15 (53.6%).
Congenital muscular dystrophy patients with normal cognitive
milestones (n = 16; 57.1%) were slightly more common than
patients with cognitive delay. In contrast to previous reports,
congenital muscular dystrophy is probably more common in
communities with high rates of parental consanguinity than other
dystrophies. Our study adds significant support to the most
recent literature on this finding.
Institution
Department of Pediatrics, Jordan University Hospital, Amman.
|