Immune-mediated extrapyramidal movement disorders, including Sydenham chorea

Area of Science:

• Neurology
• Immunology
• Pediatrics

Background:

• Immune-mediated movement disorders often affect previously healthy children.
• These disorders can manifest post-infectiously, paraneoplastically, or idiopatically.
• Conditions include Sydenham chorea, PANDAS, Encephalitis lethargica, and NMDA-R encephalitis.

Purpose of the Study:

• To review immune-mediated extrapyramidal movement disorders in children.
• To discuss the differential diagnosis and investigation of acute-onset movement disorders.
• To highlight the role of autoimmune processes in these conditions.

Main Methods:

• Literature review of immune-mediated movement disorders.
• Discussion of clinical presentations and suspected mechanisms.
• Outline of diagnostic and investigative approaches.

Main Results:

• Sydenham chorea is a post-streptococcal disorder with typically good outcomes.
• PANDAS links streptococcal infections to tics and OCD, though controversial.
• NMDA-R encephalitis involves autoantibodies and warrants immune therapies.
• Movement disorders are associated with lupus and antiphospholipid syndrome.

Conclusions:

• Autoimmune processes are central to various pediatric movement disorders.
• Accurate diagnosis and investigation are crucial for effective management.
• Emerging entities like NMDA-R encephalitis require specific immune-based interventions.