title: [Clinical and pathologic identification of different forms of Creutzfeldt Jakob disease in Chile].
abstract: BACKGROUND
  The identification of clinical and pathological forms of Creutzfeldt Jakob Disease (CJD) started with the first cases of the disease. Genetic and biomolecular prion status assessment are allowing now a better classification.
  
  
  OBJECTIVE
  To identify the clinical forms of the disease that exist in Chile, based on clinical and neuropathological data.
  
  
  METHODS
  Review of records of 40 patients with CJD in whom a complete history, clinical details and neuropathological studies were available. Clinical aspects were grouped into five categories: behavioral and cognitive changes, sleep and alertness, visual impairment, motor disturbances, myoclonus and epilepsy. The neuropathological examination in each case allowed us to evaluate the damage of 13 areas of the central nervous system.
  
  
  RESULTS
  Five forms of CJD were identified. The classic form was present in 28 patients (70%), the Heidenhain form was present in five (12.5%), the ataxic form in four (10%), the form with Kuru plaques in two (5%) and the Vacuolar was present in one patient (2.5%).
  
  
  CONCLUSIONS
  The variety and forms of CJD in Chile do not differ substantially from those found abroad.
authors: Departamento de Ciencias Neurológicas Oriente, Facultad de Medicina, Universidad de Chile, Chile. lcartier@med.uchile.cl
authors: Luis R
authors: LR
authors: Cartier
authors: Gonzalo  Z
authors: G
authors: Quiroz
authors: Marcelo H
authors: MH
authors: Leiva
authors: Carmen R
authors: CR
authors: Vergara
mesh_terms: D000328:Adult; D000368:Aged; D001921:Brain / Q000473:pathology; D002677:Chile; D007562:Creutzfeldt-Jakob Syndrome / Q000145:classification / Q000473:pathology*; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged