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.
