{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,20]],"date-time":"2026-05-20T07:11:50Z","timestamp":1779261110748,"version":"3.51.4"},"reference-count":20,"publisher":"American Academy of Pediatrics (AAP)","issue":"5","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2007,5,1]]},"abstract":"<jats:p>OBJECTIVES. Hyperimmunoglobulin E syndrome is a multisystem disorder with abnormalities of the immunologic, connective tissue, and skeletal tissue systems. Central nervous system abnormalities have not been considered a feature of hyperimmunoglobulin E syndrome. We aimed to determine whether central nervous system abnormalities detected on brain MRI exist in hyperimmunoglobulin E syndrome and to characterize any identified abnormalities.<\/jats:p>\n               <jats:p>PATIENTS AND METHODS. Fifty patients aged from 3 to 52 years (mean: 24 years) with established diagnoses of hyperimmunoglobulin E syndrome had MRI of the brain as part of an hyperimmunoglobulin E syndrome natural history protocol. Abnormalities were described, measured, counted, and mapped. Patient charts were reviewed for neurologic findings and blood pressure measurements.<\/jats:p>\n               <jats:p>RESULTS. Focal brain lesions exhibiting high signal intensities on flow-attenuated inversion recovery and on T2-weighted techniques were found in 35 of the 50 patients. The focal hyperintensities were predominantly in the white matter of the cerebral hemispheres, and the number ranged from 2 to &amp;gt;50. The hyperintensities occurred more frequently in adults than in children, and no association with elevated blood pressure was found. Five patients had lacunar infarctions. Chiari type 1 malformations were found in 9 of 50 patients. Two patients had infectious complications presenting on MRI as cerebritis in 1 patient and as a hemorrhagic infarct in the other; both were found on autopsy to be fungal. Neurologic abnormalities were present in 1 patient with a lacunar infarction, the 2 patients with infectious complications, and in 1 patient with a subarachnoid hemorrhage secondary to a berry aneurysm.<\/jats:p>\n               <jats:p>CONCLUSIONS. Central nervous system abnormalities are common in hyperimmunoglobulin E syndrome. Focal T2 hyperintensities, not appreciated previously, represent a prominent feature of this rare disease that may assist in diagnosis. The etiology and clinical implications of these abnormalities remain to be investigated.<\/jats:p>","DOI":"10.1542\/peds.2006-2649","type":"journal-article","created":{"date-parts":[[2007,4,17]],"date-time":"2007-04-17T00:39:20Z","timestamp":1176770360000},"page":"e1121-e1125","source":"Crossref","is-referenced-by-count":68,"title":["Brain Abnormalities in Patients With Hyperimmunoglobulin E Syndrome"],"prefix":"10.1542","volume":"119","author":[{"given":"Alexandra F.","family":"Freeman","sequence":"first","affiliation":[{"name":"National Institute of Allergy and Infectious Diseases"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Christina J.","family":"Collura-Burke","sequence":"additional","affiliation":[{"name":"Walter Reed Army Medical Center, Washington, DC"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Nicholas J.","family":"Patronas","sequence":"additional","affiliation":[{"name":"Clinical Center"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Lidia Stana","family":"Ilcus","sequence":"additional","affiliation":[{"name":"Clinical Center"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Dirk","family":"Darnell","sequence":"additional","affiliation":[{"name":"National Institute of Allergy and Infectious Diseases"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Joie","family":"Davis","sequence":"additional","affiliation":[{"name":"National Human Genome Research Institute, National Institutes of Health, Bethesda, Maryland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jennifer M.","family":"Puck","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, University of California, San Francisco, California"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Steven M.","family":"Holland","sequence":"additional","affiliation":[{"name":"National Institute of Allergy and Infectious Diseases"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"417","published-online":{"date-parts":[[2007,5,1]]},"reference":[{"key":"2021120823332433700_R1","doi-asserted-by":"crossref","unstructured":"Buckley RH, Wray BB, Belmaker EZ. 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