ArticleAnnals of clinical and translational neurology2020
Neurological manifestations of Erdheim-Chester Disease.
Article in Annals of clinical and translational neurology, 2020. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 27 papers.
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Who cites it
27 citing papers in PubMed, 43 citations in OpenAlex.
- Histiocytosis development and clinical variation through the lens of genomics.The Journal of pathology · 2026Review
- From suspected brain malignancy to Erdheim-Chester disease: the role of PET imaging and bone marrow biopsy in diagnosis.Oxford medical case reports · 2026Article
- Neuroimaging Spectrum of Erdheim-Chester Disease: An Image-Based Review.AJNR. American journal of neuroradiology · 2025Review
- Article
- Anxiety and depression in patients with histiocytic neoplasms and their associated clinical features.Blood advances · 2025Observational
- Neurological manifestations of Erdheim-Chester disease and their management: A scoping review.Medicine · 2025Article
- Article
- Erdheim-Chester with central nervous system involvement: Complex histopathological diagnosis and neurosurgical challenges in a rare histiocytic disorder.Surgical neurology international · 2025Article
- Utility of MR spectroscopy and MR perfusion in characterizing intracranial pathology in Erdheim-Chester disease: A case report.Radiology case reports · 2024Article
- Atypical case of Erdheim-Chester Disease involving bilateral orbits.American journal of ophthalmology case reports · 2024Article
- A microglia clonal inflammatory disorder in Alzheimer's Disease.bioRxiv : the preprint server for biology · 2024Article
- Diagnosing an unusual case of myelitis and diabetes insipidus through skin biopsies.Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology · 2024Article
- Mechanism of neurodegeneration mediated by clonal inflammatory microglia.bioRxiv : the preprint server for biology · 2024Article
- IgG4-related disease with biopsy confirmed inflammatory polyneuropathy.Rheumatology advances in practice · 2024Article
- Neurological Manifestations of Histiocytic Disorders.Current neurology and neuroscience reports · 2023Review
- Neurological Erdheim-Chester Disease Manifesting with Subacute or Progressive Cerebellar Ataxia: Novel Case Series and Review of the Literature.Brain sciences · 2022Article
- Preceding polydipsia/polyuria, ataxia, and dysarthria in an adult with mixed histiocytosis (Erdheim Chester disease/Langerhans cell histiocytosis).Annals of hematology · 2022Article
- Neuroimaging in Pediatric Patients with Juvenile Xanthogranuloma of the CNS.AJNR. American journal of neuroradiology · 2022Article
- Erdheim-Chester Disease with Isolated CNS Involvement: A Systematic Review of the Literature.Neurology international · 2022Review
- Erdheim-Chester Disease Involving the Central Nervous System with Latent Toxoplasmosis.Internal medicine (Tokyo, Japan) · 2022Article
Corrections and comments
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Authors and funding
12 authors at 5 institutions in 1 country.
Funding
Abstract
objectiveTo characterize the spectrum of neurologic involvement in Erdheim-Chester Disease (ECD), a treatable inflammatory neoplasm of histiocytes.
methodsSixty-two patients with ECD were prospectively enrolled in a natural history study that facilitated collection of clinical, imaging, laboratory, neurophysiologic, and pathologic data.
resultsNinety-four percent of the patients had neurologic abnormalities on examination or imaging, and 22% had neurologic symptoms as the initial presentation of ECD. The most common neurologic findings were cognitive impairment, peripheral neuropathy, pyramidal tract signs, cranial nerve involvement, and cerebellar ataxia. Imaging revealed atrophy and demyelination along with focal lesions that were located throughout the nervous system, dura, and extra-axial structures. The BRAF V600E variant correlated with cerebral atrophy. Brain pathology revealed lipid-laden, phagocytic macrophages (histiocytes) accompanied by demyelination and axonal degeneration.
interpretationIn patients with ECD, neurologic morbidity is common and contributes significantly to disability. Since neurologic symptoms can be the presenting feature of ECD and, given the mean delay in ECD diagnosis is 4.2 years, it is critical that neurologists consider of ECD and other histiocytosis in patients with inflammatory, infectious, or neoplastic-appearing white matter. Furthermore, given the broad spectrum of neurologic involvement, neurologists have an important role in a team of specialists treating ECD patients.
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