Which disorder is a juvenile-onset form of Huntington disease presenting before age 21 with rigidity, dystonia, and akinesia?

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Multiple Choice

Which disorder is a juvenile-onset form of Huntington disease presenting before age 21 with rigidity, dystonia, and akinesia?

Juvenile-onset Huntington disease is the Huntington disease variant that begins before age 21. In contrast to the classic adult-onset form that is often choreiform, the juvenile presentation more commonly shows rigidity, dystonia, and akinesia, and can include seizures. This parkinsonian-dystonic motor pattern reflects the Westphal variant of HD, caused by the same HTT gene CAG-repeat expansion and often shows anticipation with earlier onset in subsequent generations. Other options describe distinct movement disorders with different etiologies and clinical pictures (Sydenham chorea is post-streptococcal chorea; hemiballismus is unilateral flinging movements from subthalamic damage; Wilson disease involves copper metabolism with hepatic and neuro signs). Thus, the juvenile-onset form of Huntington disease best fits the described presentation.

Juvenile-onset Huntington disease is the Huntington disease variant that begins before age 21. In contrast to the classic adult-onset form that is often choreiform, the juvenile presentation more commonly shows rigidity, dystonia, and akinesia, and can include seizures. This parkinsonian-dystonic motor pattern reflects the Westphal variant of HD, caused by the same HTT gene CAG-repeat expansion and often shows anticipation with earlier onset in subsequent generations. Other options describe distinct movement disorders with different etiologies and clinical pictures (Sydenham chorea is post-streptococcal chorea; hemiballismus is unilateral flinging movements from subthalamic damage; Wilson disease involves copper metabolism with hepatic and neuro signs). Thus, the juvenile-onset form of Huntington disease best fits the described presentation.

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