Myotonic dystrophy - type 1

Case contributed by Eugen Lungu
Diagnosis almost certain

Presentation

Trauma, rule out intracranial hemorrhage. History of myotonic dystrophy type 1.

Patient Data

Age: 55
Gender: Male
ct

No acute findings.

Mild cortical atrophy. Bilateral diffuse subcortical hypodensities including frontal and parietal regions, as well as bilateral anterior temporal lobes. No cortical lesions.

Annotated image

Bilateral diffuse subcortical hypodensities including bilateral anterior temporal lobes (blue arrows). 

Case Discussion

This case nicely demonstrates expected head CT findings in myotonic dystrophy type 1 (Steinert disease). MRI findings include diffuse bilateral subcortical white matter lesions in the fronto-parietal and anterior temporal regions, the latter being highly specific for this condition, as well as mild cortical atrophy and ventricular dilatation. Unfortunately, no MR study was available for this patient.

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