title: Idiopathic very late-onset cerebellar ataxia: a Brazilian case series.
abstract: The authors present a Brazilian case series of eight patients with idiopathic very-late onset (mean 75.5 years old) cerebellar ataxia, featuring predominantly gait ataxia, associated with cerebellar atrophy.
  
  
  METHODS
  26 adult patients with a diagnosis of idiopathic late onset cerebellar ataxia were analyzed in a Brazilian ataxia outpatient clinic and followed regularly over 20 years. Among them, 8 elderly patients were diagnosed as probable very late onset cerebellar ataxia. These patients were evaluated with neurological, ophthalmologic and Mini-Mental Status examinations, brain MRI, and EMG.
  
  
  RESULTS
  62.5% of patients were males, mean age was 81.9 years-old, and mean age of onset was 75.5 years. Gait cerebellar ataxia was observed in all patients, as well as, cerebellar atrophy on brain MRI. Mild cognitive impairment and visual loss, due to macular degeneration, were observed in 50% of cases. Chorea was concomitantly found in 3 patients.
  
  
  CONCLUSIONS
  We believe that this condition is similar the one described by Marie-Foix-Alajouanine presenting with mild dysarthria, associated with gait ataxia, and some patients had cognitive dysfunction and chorea.
authors.affiliation: Departamento de Medicina Interna, Hospital de Clínicas, Universidade Federal do Paraná, Curitiba, PR, Brazil.
authors.forename: Hélio A G
authors.initials: HA
authors.lastname: Teive
authors.affiliation: Departamento de Medicina Interna, Hospital de Clínicas, Universidade Federal do Paraná, Curitiba, PR, Brazil.
authors.forename: Mariana
authors.initials: M
authors.lastname: Moscovich
authors.affiliation: Departamento de Medicina Interna, Hospital de Clínicas, Universidade Federal do Paraná, Curitiba, PR, Brazil.
authors.forename: Adriana
authors.initials: A
authors.lastname: Moro
authors.affiliation: Departamento de Medicina Interna, Hospital de Clínicas, Universidade Federal do Paraná, Curitiba, PR, Brazil.
authors.forename: Marina
authors.initials: M
authors.lastname: Farah
authors.affiliation: Departamento de Medicina Interna, Hospital de Clínicas, Universidade Federal do Paraná, Curitiba, PR, Brazil.
authors.forename: Walter O
authors.initials: WO
authors.lastname: Arruda
authors.affiliation: Movement Disorders Centre, Toronto Western Hospital, University of Toronto, Toronto, ON, Canada.
authors.forename: Renato P
authors.initials: RP
authors.lastname: Munhoz
mesh_terms: D017668:Age of Onset; D000368:Aged; D000369:Aged, 80 and over; D001284:Atrophy; D001938:Brazil; D002531:Cerebellum / Q000473:pathology; D002819:Chorea / Q000473:pathology / Q000503:physiopathology; D060825:Cognitive Dysfunction / Q000473:pathology / Q000503:physiopathology; D004576:Electromyography; D005260:Female; D020234:Gait Ataxia / Q000473:pathology / Q000503:physiopathology*; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D008609:Mental Status Schedule; D013132:Spinocerebellar Degenerations / Q000473:pathology / Q000503:physiopathology*