"this is known as neurodegenerative Langerhans cell histiocytosis (ND-LCH)"
"GFAP: elevated"
"Treatment is complex, but often involves prednisolone and chemotherapy 4. However this treatment is not effective in improving the diabetes insipidus or neurodegenerative symptoms 1,7,8. Hence, many patients additionally require desmopressin for diabetes insipidus 1. Treatment of neurodegeneration in LCH may include immunochemotherapy (e.g. BRAF V600E inhibitor therapy) 7-9."
Expected headings
"Diabetes insipidus"
"Neurodegenerative changes"
"Mass lesions"
"There are generally three distinct clinical presentations that may or may not all be present in any given patient:"
"There is proliferation of CD1a and CD207 Langerhans cells with an abundance of eosinophils, lymphocytes and neutrophils, typically affecting the hypothalamic-pituitary axis, cerebellum, and basal ganglia, accounting for the clinical presentation and radiographic features 2,4."
"There is proliferation of CD1a and CD207 Langerhans cells with an abundance of eosinophils, lymphocytes and neutrophils, typically affecting the hypothalamic-pituitary axis, cerebellum, and basal ganglia, accounting for the clinical presentation and radiographic features 2,4."
"There is proliferation of CD1a and CD207 Langerhans cells with an abundance of eosinophils, lymphocytes and neutrophils, typically affecting the hypothalamic-pituitary axis, cerebellum, and basal ganglia, accounting for the clinical presentation and radiographic features 2,4."
"Mass lesions are the least common CNS manifestation of LCH 2,3. Masses have a variable MR appearance and can arise from the meninges (especially the dura mater), pineal gland, choroid plexus, hypothalamus, ependyma, or can rarely be intraparenchymal 2,3. These lesions, depending on size and location, may cause obstructive hydrocephalus 2,3."