title: Moyamoya disease and sagittal sinus thrombosis in a child with Down's syndrome.
abstract: A girl with Down's syndrome, moyamoya disease and sagittal sinus thrombosis is described. She was diagnosed after acute neurological deterioration by MRI and angiography. Recombinant tissue plasminogen activator (r-TPA) was injected locally to recanalise the thrombus. The patient's condition significantly improved and she was discharged. After 2 years of follow-up the child remains asymptomatic. Moyamoya syndrome and cerebral venous thrombosis should not be overlooked as a cause of acute neurological deterioration in a child with Down's syndrome. MRA appears to be a safe and accurate alternative to traditional angiography for the diagnosis of moyamoya disease. Local fibrinolysis with r-TPA is the treatment of choice for cerebral venous thrombosis due to its safety and efficacy.
authors.affiliation: Department of Paediatrics, Fundación Jiménez Diaz, Madrid, Spain. rpediatria@fjd.es
authors.forename: G
authors.initials: G
authors.lastname: Del-Rio Camacho
authors.forename: A L
authors.initials: AL
authors.lastname: Orozco
authors.forename: A
authors.initials: A
authors.lastname: Pérez-Higueras
authors.forename: M
authors.initials: M
authors.lastname: Camino López
authors.forename: I
authors.initials: I
authors.lastname: Al-Assir
authors.forename: M
authors.initials: M
authors.lastname: Ruiz-Moreno
mesh_terms: D002675:Child, Preschool; D004314:Down Syndrome / Q000150:complications*; D005260:Female; D005343:Fibrinolytic Agents / Q000627:therapeutic use; D006801:Humans; D018810:Magnetic Resonance Angiography; D008279:Magnetic Resonance Imaging; D009072:Moyamoya Disease / Q000175:diagnosis / Q000209:etiology*; D012851:Sinus Thrombosis, Intracranial / Q000175:diagnosis* / Q000188:drug therapy / Q000209:etiology*; D010959:Tissue Plasminogen Activator / Q000627:therapeutic use
chemical_list: D005343:Fibrinolytic Agents; D010959:Tissue Plasminogen Activator