title: Nonanaplastic pleomorphic xanthoastrocytoma with meningeal dissemination presenting with bilateral visual loss.
abstract: Pleomorphic xanthoastrocytoma (PXA) is a brain neoplasm included in the astrocytic group, exceptionally manifesting with meningeal dissemination. We described a 27-year-old patient presented with acute bilateral visual loss and papilledema with normal brain computed tomography scan, initially mimicking idiopathic intracranial hypertension (IIH). Brain and spinal cord magnetic resonance imaging (MRI) study revealed a subtle area of hyperintensity of the gyri surrounding the left central sulcus, and contrast enhancement of the thoracic leptomeninges. Brain biopsy of the parietal lesion revealed nonanaplastic PXA. Treatment with temozolomide was given. Yearly control MRI demonstrated new brain lesions and marked progression of leptomeningeal spinal enhancement. In spite of this, the patient has remained stable with no new symptoms. Nonanaplastic PXA may present with widespread meningeal dissemination with acute visual loss and papilledema mimicking IIH, and no clinical progression at 3 years.
authors.affiliation: Services of Neurology, University Hospital "Marqués de Valdecilla (IFIMAV)", University of Cantabria and "Centro de Investigación Biomédica en Red de Enfermedades Neurodegenerativas (CIBERNED)", Santander, Spain.
authors.forename: Manuel
authors.initials: M
authors.lastname: Delgado-Alvarado
authors.forename: Javier
authors.initials: J
authors.lastname: Gómez-Román
authors.forename: Elena
authors.initials: E
authors.lastname: Sánchez-Salmón
authors.forename: Eloy
authors.initials: E
authors.lastname: Rodríguez-Rodríguez
authors.forename: José M
authors.initials: JM
authors.lastname: Polo
authors.forename: Almudena
authors.initials: A
authors.lastname: García-Castaño
authors.forename: José
authors.initials: J
authors.lastname: Berciano
mesh_terms: D000328:Adult; D001254:Astrocytoma / Q000150:complications / Q000473:pathology* / Q000628:therapy*; D001766:Blindness / Q000175:diagnosis / Q000209:etiology* / Q000517:prevention & control; D001932:Brain Neoplasms / Q000150:complications / Q000473:pathology* / Q000628:therapy*; D003131:Combined Modality Therapy; D003606:Dacarbazine / Q000031:analogs & derivatives / Q000627:therapeutic use; D006261:Headache / Q000175:diagnosis / Q000209:etiology* / Q000517:prevention & control; D006801:Humans; D008297:Male; D000077204:Temozolomide; D016896:Treatment Outcome; D017287:Ventriculoperitoneal Shunt
chemical_list: D003606:Dacarbazine; D000077204:Temozolomide
keywords: Idiopathic intracranial hypertension; magnetic resonance imaging; meningeal neoplasm; pleomorphic xanthoastrocytoma; temozolamide