ArticleJournal of neurology2018
Adult leukoencephalopathies with prominent infratentorial involvement can be caused by Erdheim-Chester disease.
Article in Journal of neurology, 2018. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 5 papers.
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5 citing papers in PubMed, 19 citations in OpenAlex.
- Novel paraneoplastic mechanism for cerebellar ataxia in Erdheim-Chester disease.BMJ case reports · 2023Article
- Neurological Erdheim-Chester Disease Manifesting with Subacute or Progressive Cerebellar Ataxia: Novel Case Series and Review of the Literature.Brain sciences · 2022Article
- Neurologic and oncologic features of Erdheim-Chester disease: a 30-patient series.Neuro-oncology · 2020Article
- Treating Heart Inflammation With Interleukin-1 Blockade in a Case of Erdheim-Chester Disease.Frontiers in immunology · 2018Article
- Anakinra Therapy for Non-cancer Inflammatory Diseases.Frontiers in pharmacology · 2018Review
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15 authors at 5 institutions in 1 country.
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Abstract
backgroundLeukoencephalopathies with prominent involvement of cerebellum and brainstem, henceforward called prominent infratentorial leukoencephalopathies (PILs), encompass a variety of inherited and acquired white matter diseases. Erdheim-Chester disease (ECD) is a rare non-Langerhans cell histiocytosis likely under-diagnosed as cause of adult PIL.
methodsWe reviewed the clinical and laboratory information of ten consecutive sporadic adult patients with PIL of unknown origin, who were investigated for ECD.
resultsThere were seven males and three females; mean age at clinical onset was 49.6 years (range 38-59); cerebellar ataxia with or without other neurological symptoms was the only or the main clinical manifestation; diabetes insipidus was present in three individuals. Eight patients had white matter focal supratentorial abnormalities, in addition to the infratentorial white matter changes. Six out of eight patients had spinal cord lesions. Thoraco-abdominal CT showed periaortic sheathing in two patients, whole-body FDG-PET revealed increased glucose uptake in the long bones of the legs in five patients, brain FDG-PET showed overt infratentorial hypermetabolism in one patient. In eight patients, ECD was confirmed by bone scintigraphy, pathological data, or both. Two ECD patients treated with vemurafenib showed a marked improvement of neurological symptoms and brain MRI abnormalities at 1 year follow-up.
conclusionsSymptoms of PIL can be the only clinical manifestation of ECD. Adult patients with PIL of unknown origin should undergo investigations aimed at unveiling ECD, including bone scintigraphy and whole-body FDG-PET. The early diagnosis allows starting disease-modifying therapies of an otherwise life-threatening disease.
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