| Nelson's syndrome -- 46 years later: clinical experience with 37 patients. | |
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MedLine Citation:
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PMID: 16518746 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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OBJECTIVE: Pituitary tumours occurring after bilateral adrenalectomy for Cushing's disease (Nelson's syndrome) are frequently aggressive, so an early diagnosis and careful management are of prime importance. For a new insight into this entity it is necessary to analyse the factors predisposing to its development and the course of the disease, as well as the methods of diagnosis and modalities of treatment. PATIENTS AND METHODS: Thirty-seven patients with Nelson's syndrome were observed, 32 women and 5 men, aged 16 to 61 years at the time of pituitary tumour detection (at present, 27 to 82 years old). The diagnostic methods included clinical observation, imaging examinations (X-ray studies, CT, MRI), hormonal evaluation (especially ACTH and cortisol levels during replacement therapy) and ophthalmologic investigations. Neurosurgery was the main method of treatment. RESULTS: The clinical analysis indicated that young age at the time of adrenalectomy, pregnancy, insufficient replacement therapy and fulminant course of Cushing's disease were the main factors predisposing to Nelson's syndrome. MRI appeared to be the most valuable imaging method, as this detected Nelson's tumours in the microadenoma stage in 7 patients. Plasma ACTH levels varied between 32.6 pmol/l in an early phase to 2 000 pmol/l in the full-blown syndrome. Absolute temporal scotomas found in ophthalmologic examinations were an early abnormality. The best results after therapy were obtained in patients treated by neurosurgery using a transsphenoidal approach in an early stage. CONCLUSIONS: MRI, ophthalmologic examination and plasma ACTH determination were the most valuable investigations for early diagnosis of Nelson's syndrome. Early neurosurgery offered the best outcome in our group of patients. |
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Authors:
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A A Kasperlik-Załuska; W Bonicki; W Jeske; J Janik; W Zgliczyński; Z Czernicki |
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Publication Detail:
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Type: Journal Article; Research Support, Non-U.S. Gov't |
Journal Detail:
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Title: Zentralblatt für Neurochirurgie Volume: 67 ISSN: 0044-4251 ISO Abbreviation: Zentralbl. Neurochir. Publication Date: 2006 Feb |
Date Detail:
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Created Date: 2006-03-06 Completed Date: 2006-04-04 Revised Date: 2006-11-15 |
Medline Journal Info:
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Nlm Unique ID: 0413646 Medline TA: Zentralbl Neurochir Country: Germany |
Other Details:
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Languages: eng Pagination: 14-20 Citation Subset: IM |
Affiliation:
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Department of Endocrinology, Centre for Postgraduate Medical Education, Warsaw, Poland. anna@ultima.neostrada.pl |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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Adolescent Adrenalectomy / adverse effects Adrenocorticotropic Hormone / blood Adult Aged Aged, 80 and over Cushing Syndrome / complications, surgery Eye Diseases / diagnosis, etiology Female Follow-Up Studies Hormone Replacement Therapy Humans Hydrocortisone / blood Magnetic Resonance Imaging Male Middle Aged Nelson Syndrome / radiography, surgery* Neurosurgical Procedures Pituitary Neoplasms / radiography, surgery* Tomography, X-Ray Computed |
| Chemical | |
Reg. No./Substance:
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50-23-7/Hydrocortisone; 9002-60-2/Adrenocorticotropic Hormone |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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