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Neurological Manifestations of Histiocytic Disorders

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Abstract

Purpose of Review

Histiocytic disorders, including Erdheim-Chester disease (ECD), Langerhans cell histiocytosis (LCH), and Rosai-Dorfman disease (RDD), are rare neoplasms that may present with a spectrum of neurologic involvement. Diagnostic delay is common due to heterogeneity in presentation and challenging pathology.

Recent Findings

Recent advances in the treatment of these diseases targeted towards mutations in the MAP kinase pathway have led to an improved prognosis in these patients with neurologic involvement.

Summary

It is critical for clinicians to have a high index of suspicion to allow for early targeted treatment and optimize neurologic outcomes. A systematic approach to diagnosis is presented in this article to allow for accurate diagnosis of these rare diseases.

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Authors and Affiliations

  1. Department of Neurology, Mayo Clinic Rochester, 200 First St SW, Rochester, MN, 55905, USA

    Samantha A Banks & W Oliver Tobin

  2. Department of Dermatology, Mayo Clinic Rochester, Rochester, MN, USA

    Julio C Sartori Valinotti

  3. Division of Hematology, Mayo Clinic Rochester, Rochester, MN, USA

    Ronald S Go, Jithma P Abeykoon, Jay H Ryu, N Nora Bennani & Mithun V Shah

  4. Division of Hematology-Oncology, The University of Alabama at Birmingham, Birmingham, AL, USA

    Gaurav Goyal

  5. Department of Radiology, Mayo Clinic in Jacksonville, Jacksonville, FL, USA

    Jason R Young

  6. Division of Rheumatology, Mayo Clinic Rochester, Rochester, MN, USA

    Matthew J Koster

  7. Division of Pulmonary and Critical Care Medicine, Mayo Clinic Rochester, Rochester, MN, USA

    Robert Vassallo

  8. Division of Endocrinology, Mayo Clinic Rochester, Rochester, MN, USA

    Caroline J Davidge-Pitts

  9. Division of Laboratory Medicine-Hematopathology, Department of Pathology, The University of Alabama at Birmingham, Birmingham, AL, USA

    Aishwarya Ravindran

  10. Division of Hematopathology, Mayo Clinic Rochester, Rochester, MN, USA

    Karen L Rech

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on behalf of the Mayo Clinic-University of Alabama at Birmingham Histiocytosis Working Group and Mayo Clinic Center for Multiple Sclerosis and Autoimmune Neurology

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Correspondence to W Oliver Tobin.

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Conflict of Interest

Samantha A Banks, Julio C Sartori Valinotti, Ronald S Go, Jithma P Abeykoon, Jason R Young, Matthew J Koster, Jay H Ryu, Caroline J Davidge-Pitts, Aishwarya Ravindran, N Nora Bennani, and Karen L Rech each declare no potential conflicts of interest.

N Bennani serves as an unpaid Advisory Board member of Daichii Sankyo, Inc.; Kyowa Kirin; Vividion Therapeutics, Kymera, Secura Bio; Affimed, GmbH; Astellas Pharma, and Acrotech Biopharma, LLC. WO Tobin is an unpaid member of the Mayo Clinic Center for Multiple Sclerosis and Autoimmune Neurology.

Gaurav Goyal is on the advisory board of Opna Bio LLC and has received royalties from UpToDate. Robert Vassallo has received grants from Pfizer, Bristol Myers Squibb, and Sun Pharma. M Shah receives research support from AbbVie, MRKR Therapeutics, Celgene, and Astellas. W. Oliver Tobin has received grants from the National Institutes of Health, Mallinckrodt Inc, personal fees from DKBMed and book royalties from the publication of Mayo Clinic Cases in Neuroimmunology (Mayo Clinic Scientific Press) 2022. Mithun V Shah has received grants from ABBVIE, MRKR THERAPEUTICS, CELGENE, and ASTELLAS.

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Banks, S.A., Sartori Valinotti, J.C., Go, R.S. et al. Neurological Manifestations of Histiocytic Disorders. Curr Neurol Neurosci Rep 23, 277–286 (2023). https://doi.org/10.1007/s11910-023-01272-4

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