Abstract
Various neurodegenerative diseases in childhood may mimic acquired demyelinating conditions, and a thorough work-up may be required to reach a definite diagnosis. Both categories of conditions can present with multifocal central nervous system involvement and can have a variable course to include relapsing or progressive disease. Details of the clinical history, CSF results, and neuroimaging findings can help to distinguish between conditions, although more extensive investigations can be required when a clear diagnosis cannot be determined. Neurodegenerative Langerhans cell histiocytosis (LCH) may occur with a history of known systemic disease, but occasional patients may present without a previous diagnosis and can pose a diagnostic dilemma to the clinician with features that might be suggestive of ADEM, MS, or even NMO among other diseases. We present the clinical course of a 13-year-old girl with a known diagnosis of LCH who presented with new neurological features concerning for neurodegenerative disease.
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Agarwal, S., Lotze, T.E. (2017). Neurodegenerative Langerhans Cell Histiocytosis. In: Waubant, E., Lotze, T. (eds) Pediatric Demyelinating Diseases of the Central Nervous System and Their Mimics. Springer, Cham. https://doi.org/10.1007/978-3-319-61407-6_18
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DOI: https://doi.org/10.1007/978-3-319-61407-6_18
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