Which syndrome is characterized by atypical eye and movement disturbances in children?

Prepare for USMLE Step 1 Pathology Exam with comprehensive quizzes, flashcards, and detailed explanations. Enhance your understanding and be exam-ready!

Opsoclonus-myoclonus ataxia syndrome is characterized by a combination of rapid, involuntary eye movements (opsoclonus), myoclonic jerks (sudden muscle spasms), and ataxia (lack of voluntary coordination of muscle movements). This condition is often seen in children and is frequently associated with neuroblastoma, a type of tumor that develops from immature nerve cells.

In this syndrome, the atypical eye movements manifest as erratic and uncontrolled movements, which can be distressing for both the patients and their caregivers. The myoclonic jerks can affect various muscle groups, leading to difficulties in movement and coordination, while ataxia can result in balance issues and an unsteady gait.

This condition highlights the significance of the immune response in pediatric neurology, where autoimmunity may play a role in the pathogenesis. It typically presents in young children, making early recognition and intervention critical for management and potential treatment of associated malignancies.

The other options, while they describe various neuromuscular or autoimmune conditions, do not specifically present with the combination of eye and movement disturbances as prominently as opsoclonus-myoclonus ataxia syndrome does, which distinguishes it as the

Subscribe

Get the latest from Examzify

You can unsubscribe at any time. Read our privacy policy