| Central hypothyroidism and Sturge-Weber syndrome. | |
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MedLine Citation:
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PMID: 18555176 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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Sturge-Weber syndrome is a rare disorder manifesting with a facial port-wine birthmark and a vascular malformation of the brain. Infants and children present with seizures and stroke-like episodes with focal neurologic deficits. Our previous investigations revealed that growth-hormone deficiency occurs with an increased prevalence in Sturge-Weber syndrome, presumably secondary to involvement of the hypothalamic-pituitary axis. We have continued to screen for hormonal abnormalities in patients with Sturge-Weber syndrome, specifically those from our multidisciplinary center for patients with this condition. We describe 2 children out of 83 (2.4%) with Sturge-Weber syndrome and brain involvement who were evaluated at our center and diagnosed with central hypothyroidism, based on clinical signs and laboratory findings. This prevalence is much higher than that of central hypothyroidism in the general population. Although it is well-known that anticonvulsants can lead to abnormalities in thyroid function tests, including central hypothyroidism, patients with Sturge-Weber syndrome carry the additional risk of developing hypothalamic-pituitary dysfunction, secondary to their central nervous system dysfunction. Therefore, it is important that patients with Sturge-Weber syndrome undergo routine thyroid-function testing, especially in the face of any clinical manifestations. |
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Authors:
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Anne M Comi; Sridevi Bellamkonda; Lisa M Ferenc; Bernard A Cohen; Emily L Germain-Lee |
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Publication Detail:
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Type: Case Reports; Journal Article; Research Support, Non-U.S. Gov't |
Journal Detail:
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Title: Pediatric neurology Volume: 39 ISSN: 0887-8994 ISO Abbreviation: Pediatr. Neurol. Publication Date: 2008 Jul |
Date Detail:
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Created Date: 2008-06-16 Completed Date: 2008-08-25 Revised Date: - |
Medline Journal Info:
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Nlm Unique ID: 8508183 Medline TA: Pediatr Neurol Country: United States |
Other Details:
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Languages: eng Pagination: 58-62 Citation Subset: IM |
Affiliation:
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Department of Neurology and Developmental Medicine, Kennedy Krieger Institute, Baltimore, Maryland 21205, USA. comi@kennedykrieger.org |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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Anticonvulsants
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adverse effects,
therapeutic use Child Female Glaucoma / complications Humans Hypothyroidism / complications*, drug therapy Magnetic Resonance Imaging Male Paresis / complications Port-Wine Stain / complications Seizures / etiology Stroke / complications Sturge-Weber Syndrome / complications* Thyroid Function Tests Thyroxine / therapeutic use |
| Chemical | |
Reg. No./Substance:
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0/Anticonvulsants; 7488-70-2/Thyroxine |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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