NEW ROENTGENOLOGIC FINDINGS IN MYOTONIC DYSTROPHY

AN ANALYSIS OF 18 PATIENTS

¹ Professor of Radiology. Presently: Assistant Professor of Radiology, Graduate Hospital of University of Pennsylvania and Presbyterian-University of Pennsylvania Medical Center, Philadelphia, Pennsylvania.
² Formerly: Fellow in Neuroradiology.
³ Formerly: Professor of Radiology and Chairman of the Department of Radiology. Presently: Professor of Radiology and Chairman of the Department of Radiology, Jackson Memorial Hospital, University of Miami, Miami, Florida.

1. The roentgenographic findings of 18 patients with myotonic dystrophy, from 5 families, are presented. The previously described roentgen findings of the skull such as thickening of the calvarium, large frontal sinus, small sella and elongated mandible were observed in the majority of our series.

2. The previously undescribed roentgenologic findings are: (a) small basal angle (craniokyphosis); (b) small nasal angle; (c) small interorbital distance (hypotelorism); (d) small volumetric measurement of the pituitary fossa; and (e) pectus excavatum

3. Those patients who developed clinical symptoms before puberty tended to show a more severe form of myotonic dystrophy and generally there was a good correlation between the degree of clinical symptoms and the roentgenologic changes observed.

4. The skull changes are not necessarily characteristic of myotonic dystrophy due to wide normal variations of the skull. However, the diagnosis maybe suggested if the above findings are present.

CiteULike    Complore    Connotea    Del.icio.us    Digg    Reddit    Technorati    What's this?