title: A case of atypical progressive supranuclear palsy.
abstract: BACKGROUND
  Progressive supranuclear palsy (PSP) is a neurodegenerative extrapyramidal syndrome. Studies have demonstrated that PSP can present clinically as an atypical dementing syndrome dominated by a progressive apraxia of speech (AOS) and aphasia.
  
  
  OBJECTIVE
  We aimed to investigate the clinical presentation of PSP, using a comprehensive multidimensional evaluation, and the disease response to various pharmacological treatments.
  
  
  METHODS
  A 72-year-old right-handed male, with 17 years education, who first presented with aphasia, AOS, depression, apathy, and postural instability at 69 years; a complete neuropsychological evaluation, tapping the different cognitive domains, was performed.
  
  
  RESULTS
  Testing revealed a moderate global cognitive deficit (Mini-Mental State Examination test score =20), low memory test scores (story recall, Rey's 15-word Immediate and Delayed Recall), and poor phonemic and semantic fluency. The patient's language was characterized by AOS, with slow speech rate, prolonged intervals between syllables and words, decreased articulatory accuracy, sound distortions, and anomia. Behavioral changes, such as depression, anxiety, apathy, and irritability, were reported. The neurological examination revealed supranuclear vertical gaze palsy, poor face miming, and a mild balance deficit. Magnetic resonance imaging showed only widespread cortical atrophy. Single photon emission computed tomography demonstrated left > right frontotemporal cortical abnormalities. After 6 months, a further neuropsychological assessment showed a progression in cognitive deficits, with additional attention deficits. The patient reported frequent falls, but the neurological deficits remained unchanged. Neuroimaging tests showed the same brain involvement.
  
  
  CONCLUSIONS
  Our case highlights the heterogeneity of the clinical features in this syndrome, demonstrating that atypical PSP can present as AOS and aphasia, without the classical features or involvement of the subcortical gray and brainstem region, commonly affected in typical PSP.
authors.affiliation: IRCCS Salvatore Maugeri Foundation, Cassano Murge, Bari, Italy.
authors.forename: Simona
authors.initials: S
authors.lastname: Spaccavento
authors.affiliation: IRCCS Salvatore Maugeri Foundation, Cassano Murge, Bari, Italy.
authors.forename: Marina
authors.initials: M
authors.lastname: Del Prete
authors.affiliation: IRCCS Salvatore Maugeri Foundation, Cassano Murge, Bari, Italy.
authors.forename: Angela
authors.initials: A
authors.lastname: Craca
authors.affiliation: IRCCS Salvatore Maugeri Foundation, Cassano Murge, Bari, Italy.
authors.forename: Anna
authors.initials: A
authors.lastname: Loverre
mesh_terms: D000368:Aged; D001037:Aphasia / Q000175:diagnosis / Q000628:therapy; D056344:Executive Function; D006801:Humans; D008297:Male; D009483:Neuropsychological Tests; D010300:Parkinson Disease / Q000175:diagnosis* / Q000628:therapy; D011569:Psychiatric Status Rating Scales; D013494:Supranuclear Palsy, Progressive / Q000175:diagnosis* / Q000628:therapy
keywords: neuropsychological deficits; pharmacological treatments