Neurobehavioral profiles in individuals with hyperimmunoglobulin e syndrome (HIES) and brain white matter hyperintensities

Staci Martin, Pamela Wolters, Nia Billings, Mary Anne Toledo-Tamula, Dima A. Hammoud, Pamela Welch, Dirk Darnell, Steven M. Holland, Alexandra F. Freeman

Research output: Contribution to journalArticlepeer-review


Purpose: Individuals with hyperimmunoglobulin E Syndrome (HIES) have central nervous system abnormalities, including focal white matter hyperintensities (WMH), or unidentified bright objects. This cross-sectional study aimed to describe the cognitive and emotional functioning and quality of life of people with HIES. We also sought to explore the relationship between cognitive functioning and WMHs in this population. Methods: Twenty-nine individuals (13 males) with autosomal-dominant HIES (mean age = 35.1 years, range 16-55) were administered a comprehensive psychological assessment as part of a natural history protocol. The assessment included measures of global cognitive functioning (Wechsler Adult Intelligence Scale-III), memory (California Verbal Learning Test-II, Wechsler Memory Scale-III), executive skills (Delis Kaplan Executive Function System), and attention (Test of Everyday Attention). Emotional symptoms and quality of life also were assessed. Results: All mean cognitive scores were within normal limits. Mean scores on memory and executive functioning measures were significantly lower than Full Scale IQ scores (ps <.05). Substantial percentages of patients self-reported executive skills to be in the clinical range. Patients with fewer (1-20) versus more (21+) WMHs scored significantly better on measures of global cognitive skills, visual-perceptual skills, and working memory. Mean scores on emotional symptom and quality of life measures were in the average range and unrelated to WMHs. Conclusions: Global cognitive functioning was average to high average in our sample of individuals with HIES. However, focal brain lesions were associated with lower scores in specific domains. Emotional functioning and quality of life are within normal limits in this sample.

Original languageEnglish (US)
Pages (from-to)1175-1184
Number of pages10
JournalJournal of Clinical Immunology
Issue number7
StatePublished - Oct 2013
Externally publishedYes


  • Hyperimmunoglobulin E Syndrome
  • Job's Syndrome
  • cognitive functioning
  • psychological functioning
  • quality of life

ASJC Scopus subject areas

  • Immunology and Allergy
  • Immunology


Dive into the research topics of 'Neurobehavioral profiles in individuals with hyperimmunoglobulin e syndrome (HIES) and brain white matter hyperintensities'. Together they form a unique fingerprint.

Cite this