title: Erdheim-Chester Disease: An Unusual Cause of Intracranial Vasculitis and Progressive Leukoencephalopathy.
abstract: Erdheim-Chester disease (ECD) is a non-Langerhans histiocytosis affecting multiple organs. Stroke as symptom onset of ECD with intracranial vasculitis is unusual. We report the case of a 64-year-old man who presented with an acute ischemic stroke associated with a moderate leukoencephalopathy and intracranial arteries stenosis. Four years later, he developed movement disorders with dysarthria and cognitive impairment. Neuroradiological findings demonstrated a rapidly progressive and diffuse leukoencephalopathy associated with brain atrophy and infiltration of the intracranial vertebral artery wall. Brain postmortem evaluation confirmed the diagnosis of ECD. This diagnosis should be evoked in patients with cryptogenic stroke, progressive leukoencephalopathy, and infiltration of the arterial wall.
authors.affiliation: Unité Neuro-vasculaire, CHU de Bordeaux, Bordeaux, France; Université Bordeaux Segalen, CHU de Bordeaux, Bordeaux, France.
authors.forename: Sharmila
authors.initials: S
authors.lastname: Sagnier
authors.affiliation: Unité Neuro-vasculaire, CHU de Bordeaux, Bordeaux, France.
authors.forename: Sabrina
authors.initials: S
authors.lastname: Debruxelles
authors.affiliation: Université Bordeaux Segalen, CHU de Bordeaux, Bordeaux, France; Laboratoire d'Anatomo-Pathologie, CHU de Bordeaux, Bordeaux, France.
authors.forename: Sébastien
authors.initials: S
authors.lastname: Lepreux
authors.affiliation: Unité Neuro-vasculaire, CHU de Bordeaux, Bordeaux, France; Université Bordeaux Segalen, CHU de Bordeaux, Bordeaux, France. Electronic address: igor.sibon@chu-bordeaux.fr.
authors.forename: Igor
authors.initials: I
authors.lastname: Sibon
mesh_terms: D001284:Atrophy; D001344:Autopsy; D001706:Biopsy; D001921:Brain / Q000473:pathology; D002423:Cause of Death; D038524:Diffusion Magnetic Resonance Imaging; D018450:Disease Progression; D031249:Erdheim-Chester Disease / Q000150:complications* / Q000000981:diagnostic imaging / Q000473:pathology; D017809:Fatal Outcome; D006801:Humans; D007150:Immunohistochemistry; D020244:Infarction, Middle Cerebral Artery / Q000000981:diagnostic imaging / Q000209:etiology* / Q000473:pathology; D007968:Leukoencephalopathy, Progressive Multifocal / Q000000981:diagnostic imaging / Q000209:etiology* / Q000473:pathology; D008297:Male; D008875:Middle Aged; D049268:Positron-Emission Tomography; D020293:Vasculitis, Central Nervous System / Q000000981:diagnostic imaging / Q000209:etiology* / Q000473:pathology
keywords: Erdheim–Chester disease; brain atrophy; diffuse progressive leukoencephalopathy; stroke; vasculitis