A case of Duchenne's progressive muscular dystrophy with cerebral embolism after the use of estrogen

Abstract

This report contains a 13 year old boy of Duchenne's progressive muscular dystrophy (DMP) who suffered from cerebral embolism of possible cardiac origin.
The patient had muscular symptoms of DMP since the age of 2-3, together with mental retardation. He had been in National Nishitaga Hospital and educated there since the age of 6. During a month prior to onset of stroke, he was on 100 mg/day of diethylstilbestrol diphosphate in order to supress frequent and openly-performed masturbation.
On March 7th 1980, abruptly appeared loss of consciousness with right hemiplegia, conjugated deviation and vomiting. Motor aphasia was also noted next day when his consciousness became almost clear. 5 days later, on admission, right hemiplegia, motor aphasia and gynecomastia were noted in addition to apparent physical signs of DMP.
His brain CT taken 4 days after the onset showed a low density area in the left hemisphere, involving the internal capsule, lenticular nuclei and neighboring structures.
Angiograms taken 7 days after onset revealed an occlusion of the left carotid artery at its bifurcation to the anterior and middle cerebral arteries. Right retrograde carotid-vertebral angiograms showed completely normal arterial visualization including the left anterior cerebral artery. There were no findings suggestive of arteriosclerosis or inflammatory changes of the arterial wall. Angiograms taken 25 days after onset demonstrated recanalization of the occluded part.
His chest roentgenogram and ECG showed a marked cardiomegalia (CTR 63%) and myocardial damages frequently seen in DMP. Moreover, the mitral valve prolapse was strongly suggested by echocardiograms.
Laboratory examinations were within normal ranges except moderate leucocytosis, elevated CPK, slightly shortened bleeding time and elevated serum estrone for his age.
The possible roles of the mitral valve prolapse and estrogen intaken in production of cerebrovascular accident in the present case were discussed.