Case of the Week
Section Editors: Matylda Machnowska1 and Anvita Pauranik2
1University of Toronto, Toronto, Ontario, Canada
2BC Children's Hospital, University of British Columbia, Vancouver, British Columbia, Canada
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July 5, 2010
Hand-Schüller-Christian Disease
- Hand-Schüller-Christian disease belongs to the spectrum of disorders, now known as "eosinophilic granulomatosis" or "Langerhans cell granulomatosis".
- It occurs in children and is characterized by the clinical triad of diabetes inspidus, exophthalmos, and lytic bone lesions.
- MRI is the imaging study of choice.
- Findings include expansile lytic bone lesions, thickening of the pituitary stalk, hypothalamic mass, meningeal enhancement, cystic appearing pituitary gland, white matter lesions, and prominent Virchow-Robin spaces.
- The neurodegenerative form of the disease may show changes in the basal ganglia and dentate nuclei.
- Diagnosis is established by biopsying the bone lesions.
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