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Nelson syndrome: definition and management.
T M Barber1, E Adams2, J A H Wass2
1Division of Metabolic and Vascular Health, Warwick Medical School, University of Warwick, Coventry, UK.
Handbook of Clinical Neurology
|September 25, 2014
Summary
Nelson syndrome, a complication of Cushing's disease treatment, is now detected earlier due to advanced imaging and assays. Long-term screening is crucial for patients who underwent total bilateral adrenalectomy (TBA).
Area of Science:
- Endocrinology
- Neuro-oncology
- Oncology
Background:
- Nelson syndrome is a significant complication following total bilateral adrenalectomy (TBA) for refractory Cushing's disease.
- Modern detection methods like MRI and ACTH assays have altered the presentation and improved outcomes.
- Predictive factors for Nelson syndrome development require further clarification.
Purpose of the Study:
- To review the current understanding of Nelson syndrome, including its changing clinical presentation and diagnostic challenges.
- To discuss the proposed pathogenetic mechanisms of Nelson syndrome.
- To highlight the importance of long-term screening and management strategies for Nelson syndrome.
Main Methods:
- Literature review focusing on Nelson syndrome in Cushing's disease patients treated with TBA.
- Analysis of diagnostic advancements, including MRI and ACTH assays.
- Discussion of current hypotheses on pathogenesis and treatment modalities.
Main Results:
- Earlier detection has reduced morbidity and mortality associated with Nelson syndrome.
- Lack of neoadjuvant pituitary radiotherapy post-TBA may predict Nelson syndrome, but other factors are debated.
- The pathogenesis remains incompletely understood, with 'released negative feedback' and 'aggressive corticotropinoma' as leading hypotheses.
Conclusions:
- Long-term, close screening for Nelson syndrome is essential in all patients with a history of Cushing's disease and TBA.
- Management of Nelson syndrome, including pituitary surgery, radiotherapy, Gamma Knife surgery, and temozolomide, presents challenges and requires further research.
- Understanding the pathogenesis is critical for refining diagnostic and therapeutic approaches.
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