Abstract

Background: Huntington’s disease (HD) is a neurodegenerative disorder characterized by progressive chorea, cognitive decline, and psychiatric symptoms. Although typically diagnosed in mid-adulthood, early-onset Huntington’s disease (EOHD), or juvenile chorea, presents with unique clinical features and challenges.

Objective: This review aims to explore the manifestations and management strategies of juvenile chorea associated with early-onset Huntington’s disease, focusing on the distinct clinical presentation, diagnostic challenges, and therapeutic approaches.

Methods: A comprehensive review of current literature was conducted, examining case studies, clinical trials, and treatment guidelines related to juvenile chorea. Emphasis was placed on the age of onset, symptom progression, and the impact on functional abilities and quality of life.

Results: Juvenile chorea often presents with a more rapid progression and severe symptoms compared to adult-onset HD. Common manifestations include motor impairments, significant cognitive decline, and psychiatric disturbances. Diagnosis is frequently delayed due to the rarity of early-onset cases and overlapping symptoms with other disorders. Management strategies involve a multidisciplinary approach, incorporating pharmacological treatments, physical therapy, and psychological support. Emerging therapies and genetic research are promising but require further validation.

Conclusion: Juvenile chorea presents distinct challenges in the management of early-onset Huntington’s disease. Early diagnosis and tailored therapeutic interventions are crucial for improving patient outcomes and quality of life. Ongoing research is needed to better understand the disease mechanisms and develop effective treatments for this rare and debilitating condition.