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Paraneoplastic neurologic disorders in children
Elizabeth M Wells1, Josep Dalmau
1Department of Neurology, Children's National Medical Center, 111 Michigan Avenue, NW, Washington, DC, 20010, USA. ewells@cnmc.org
Abstract:
Paraneoplastic neurologic syndromes are rare disorders that have potentially devastating effects on the developing brain. Recently, there has been increased interest in possible immunotherapy for these disorders. Recognition of paraneoplastic syndromes in children may lead to early detection and treatment of the pediatric cancer and may diminish the neurologic damage that is the major source of morbidity in children with successfully treated tumors. This article reviews the presenting symptoms, immunology, long-term sequelae, and management options for paraneoplastic neurologic syndromes, focusing on those most commonly reported in children: opsoclonus-myoclonus ataxia, limbic encephalitis, and anti-NMDAR encephalitis. The child neurologist plays an important role in recognizing these disorders, initiating a tumor search, and directing ongoing treatment and management of neurologic symptoms after oncologic treatment is complete. Given the rarity of these conditions, multisite collaborative efforts are needed to develop standardized approaches to characterization and treatment.
Insights
Paraneoplastic neurologic syndromes (PNS) affect children
Area of Science:
- Pediatric Neurology
- Neuro-oncology
- Immunology
Background:
- Paraneoplastic neurologic syndromes (PNS) are rare, severe neurological disorders impacting the developing brain.
- Increased interest in immunotherapy for PNS highlights potential treatment avenues.
- Early recognition of PNS in children aids in pediatric cancer detection and reduces neurological damage.
Purpose of the Study:
- To review the clinical presentation, immunology, long-term outcomes, and management of pediatric PNS.
- To focus on commonly occurring pediatric PNS: opsoclonus-myoclonus ataxia, limbic encephalitis, and anti-NMDAR encephalitis.
Main Methods:
- Review of presenting symptoms.
- Analysis of immunological markers.
- Evaluation of management strategies.
- Focus on specific pediatric PNS subtypes.
Main Results:
- PNS can lead to significant neurological damage, a major cause of morbidity in pediatric cancer survivors.
- Opsoclonus-myoclonus ataxia, limbic encephalitis, and anti-NMDAR encephalitis are frequently observed in children.
- Child neurologists are crucial for diagnosis, tumor detection, and managing neurological sequelae.
Conclusions:
- Prompt recognition and diagnosis of PNS in children are vital for timely cancer treatment and minimizing neurological impact.
- Multisite collaborative efforts are essential for standardizing the characterization and treatment of rare pediatric PNS.
- Effective management requires a coordinated approach involving neurologists and oncologists throughout the treatment continuum.
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