ABSTRACT
Erdheim-Chester disease (ECD) is a rare form of non-Langerhans cell histiocytosis characterized by a xanthogranulomatous infiltration of tissues by spumous histiocytes. Neurological involvement is frequent, but ischemic strokes have been exceptionally described. We report the case of a 68-year-old woman who presented with an acute ischemic stroke associated with a multisystemic disorder including insipidus diabetes, infiltration of the aorta and the carotid arteries, perirenal infiltration, aortitis, and lytic bone lesions. The surgical biopsy of a lumbar vertebra revealed an infiltration of spumous macrophages consistent with ECD. Many ischemic symptoms can occur in ECD. Ischemic strokes, infrequently reported, might be caused by perivascular infiltration and adventitial fibrosis of the supra-aortic trunks or intracranial arteries.
Subject(s)
Erdheim-Chester Disease/complications , Infarction, Middle Cerebral Artery/etiology , Aged , Anti-Inflammatory Agents/therapeutic use , Cerebral Angiography/methods , Erdheim-Chester Disease/diagnosis , Erdheim-Chester Disease/drug therapy , Erdheim-Chester Disease/genetics , Fatal Outcome , Female , Genetic Predisposition to Disease , Humans , Infarction, Middle Cerebral Artery/diagnostic imaging , Interleukin 1 Receptor Antagonist Protein/therapeutic use , Magnetic Resonance Angiography , Mutation , Proto-Oncogene Proteins B-raf/genetics , Tomography, X-Ray Computed , Treatment OutcomeABSTRACT
BACKGROUND AND IMPORTANCE: Arterial aneurysms of the spinal cord are rare. Their pathogenesis is variable, and the therapeutic strategies remain controversial, because their natural history is unclear. We report a case of multiple dissecting aneurysms of radiculomedullary and radiculopial arteries presenting with spinal infarction and subarachnoid hemorrhage, which spontaneously resolved. CLINICAL PRESENTATION: A 67-year-old woman was hospitalized owing to sudden onset of severe back pain with discomfort in the lower extremities. Two days later, she again experienced sudden back pain accompanied by paraparesis and sphincter disturbance. Magnetic resonance imaging of the thoracic spinal cord showed 2 intradural masses of heterogeneous intensity at the levels of T-8 and T-10, spinal infarction, and subarachnoid hemorrhage. Spinal angiography revealed a "pearls and strings" aspect of a radiculomedullary artery arising from the left T-11 and a fusiform aneurysm of a radiculopial artery arising from the left T-8. Infectious and immunological evaluations failed to show any anomaly. Spontaneous dissections were suspected. Conservative treatment was proposed, and the patient's clinical course remained uneventful. Follow-up magnetic resonance imaging and spinal angiography performed 2 months after onset showed disappearance of both arterial lesions. CONCLUSION: Arterial dissections of spinal radicular arteries can resolve spontaneously and be managed conservatively. We propose a classification of the pathogenesis of spinal arterial aneurysm to clarify appropriate treatment strategies by a literature review.
