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Juvenile Huntington Disease Exacerbated by Methylphenidate: Case Report

Department of Pediatrics, Children's Medical Center of Dallas, Texas

Van S. Miller, MD, PhD

Texas Child Neurology, LLP, Plano, Texas

Robert S. Chudnow, MD

Texas Child Neurology, LLP, Plano, Texas

Michael Morgan Dowling, MD, PhD

University of Texas Southwestern Medical Center, Dallas, Texas, michael.dowling{at}utsouthwestern.edu

The authors describe the case of an 8-year-old boy, otherwise healthy, who presented with symptoms consistent with attention-deficit hyperactivity disorder (ADHD) and was started on a trial of methylphenidate. Within 4 weeks, he experienced a rapid decline in fine motor skills, with dysarthria, intention tremor, motor impersistence, and diffusely increased tone. Symptoms persisted despite cessation of methylphenidate. At that time, a paternal history of Huntington disease was disclosed. Molecular analysis revealed an expansion in CAG repeats to 75 copies, within the range characteristic of juvenile Huntington disease. This report raises the possibility that use of dopaminergic agonists in patients with a family history of Huntington disease may lead to clinical exacerbation of motor symptoms and/or unwitting diagnosis in an unprepared family.

Key Words: basal ganglia • attention deficit • Huntington disease

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