title: [A retrospective study of Creutzfeldt-Jakob disease in North of Portugal 1993-2002: demographic, clinical and neuropathological features].
abstract: OBJECTIVE
  Description of the demographic, clinical and neuropathological features of 11 cases of Creutzfeldt-Jakob disease (CJD).
  
  
  METHODS
  Review of the clinical and neuropathological features of patients with CJD diagnosed in hospitals in the North of Portugal between 1993 and 2002.
  
  
  RESULTS
  Eleven patients were identified, 4 females: mean age of onset of symptoms--64 years, mean duration of disease--8 months. All presented with a syndrome of progressive dementia with myoclonus, with four patients presenting with cerebellar signs. Neuropathological examination of brain at autopsy showed spongiosis and reactive gliosis associated with neuronal loss. In eight cases immunocytochemistry for prion protein (PrP) was carried out and was positive.
  
  
  CONCLUSIONS
  The group of patients described represents the heterogeneity of the clinical phenotypes possible in CJD. Neuropathological examination is still indispensable to make the definitive diagnosis of the disease.
authors.affiliation: Serviço de Neurologia, Hospital Geral de Santo António, Porto, Portugal. anadmsilva@yahoo.com
authors.forename: Ana Martins
authors.initials: AM
authors.lastname: Silva
authors.forename: Manuel Melo
authors.initials: MM
authors.lastname: Pires
authors.forename: Antonio J
authors.initials: AJ
authors.lastname: Bastos Leite
authors.forename: Mrinalini
authors.initials: M
authors.lastname: Honavar
authors.forename: Alexandre
authors.initials: A
authors.lastname: Mendes
authors.forename: Manuel
authors.initials: M
authors.lastname: Correia
authors.forename: Manuel
authors.initials: M
authors.lastname: Nora
authors.forename: Mário Rui
authors.initials: MR
authors.lastname: Silva
authors.forename: Manuela
authors.initials: M
authors.lastname: Costa
authors.forename: Antonio
authors.initials: A
authors.lastname: Guimarães
authors.forename: Luís
authors.initials: L
authors.lastname: Monteiro
mesh_terms: D017668:Age of Onset; D000368:Aged; D001284:Atrophy; D001921:Brain / Q000473:pathology*; D007562:Creutzfeldt-Jakob Syndrome / Q000235:genetics / Q000473:pathology*; D003937:Diagnosis, Differential; D004569:Electroencephalography; D005260:Female; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D011174:Portugal; D011328:Prions / Q000032:analysis; D012189:Retrospective Studies; D014057:Tomography, X-Ray Computed
chemical_list: D011328:Prions