title: A case of late onset leukoencephalopathy with cerebral calcifications and cysts in a 59-year-old woman.
abstract: BACKGROUND
  Leukoencephalopathy with cerebral calcifications and cysts (LCC) is a recently described, very rare entity, clinically characterized by progressive neurological deficits such as cognitive decline, epileptic seizures, pyramidal, extrapyramidal and cerebellar symptoms/signs. With the exception of two patients with adult onset, in all previously described cases symptoms onset occurred between early infancy and adolescence.
  
  
  RESULTS
  We report a case of late onset LCC in a 59-year-old woman presenting with urinary and fecal incontinence and behavioural changes, then rapid progression with hemianopia, hemiparesis, ataxia and cognitive decline. Extensive work-up was performed, including brain magnetic resonance imaging, magnetic resonance spectroscopy, cyst fluid analysis and brain biopsy, confirming the final diagnosis of LCC.
  
  
  CONCLUSIONS
  Our case supports the existence of a late onset adult form of LCC.
authors.affiliation: Department of Neurology, University Hospital of Zurich, Zurich, Switzerland.
authors.forename: T
authors.initials: T
authors.lastname: Kaffenberger
authors.forename: P O
authors.initials: PO
authors.lastname: Valko
authors.forename: J
authors.initials: J
authors.lastname: von Meyenburg
authors.forename: K
authors.initials: K
authors.lastname: Baráth
authors.forename: E
authors.initials: E
authors.lastname: Hewer
authors.forename: F L
authors.initials: FL
authors.lastname: Heppner
authors.forename: C E
authors.initials: CE
authors.lastname: Jagella
authors.forename: A
authors.initials: A
authors.lastname: Horst
authors.forename: M M
authors.initials: MM
authors.lastname: Siccoli
mesh_terms: D017668:Age of Onset; D001927:Brain Diseases / Q000473:pathology* / Q000503:physiopathology; D002114:Calcinosis / Q000473:pathology* / Q000503:physiopathology; D003560:Cysts / Q000473:pathology* / Q000503:physiopathology; D005260:Female; D006801:Humans; D008279:Magnetic Resonance Imaging; D008875:Middle Aged