Hydrocephalus and cognitive decline in myotonic dystrophy: case report and literature review.

Myotonic dystrophy is a rare genetic neuromuscular disorder with multisystem involvement. Hydrocephalus and cognitive deterioration are not commonly considered part of adult-onset myotonic dystrophy. This report describes a case of progressive distal muscle weakness, cognitive decline, and longstanding ventricular enlargement. Review of the literature suggests that hydrocephalus may be associated with myotonic dystrophy while progressive cognitive decline is rare in the adult form of the disease.