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May 1989

High-Resolution Magnetic Resonance Imaging Findings in Juvenile-Onset Myotonic Dystrophy

Author Affiliations

Neurology Service Department of Medicine Letterman Army Medical Center Presidio of San Francisco, CA 94129-6700

Arch Neurol. 1989;46(5):481-482. doi:10.1001/archneur.1989.00520410015010

To the Editor.  —In a recent article, Glantz et al1 reported frequent changes in the magnetic resonance imaging (MRI) findings for patients with myotonic dystrophy (MD). These MRI changes were ventriculomegaly and patchy areas of increased T2 signal in the white matter. These findings were only noted in patients with congenital or adult-onset MD.We describe a series of five patients from two unrelated families, all with juvenile-onset MD. Although the literature is vague in delineating congenital from juvenile and adult forms of MD, we define juvenile MD as having an onset in the first to second decade, and an absence of hypotonia, significant respiratory distress, facial diplegia with poor suck, or contractures at birth.2-4 None of these patients show ventriculomegaly, and all have abnormal areas of increased T2 signal consisting of thin periventricular rimming and multiple focal areas in the white matter deep to the gray-white

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