A case of atypical progressive supranuclear palsy
The patient was a 72-year-old, right-handed male, with 17 years of education and no family history of a neurodegenerative disorder. He was a retired university office manager. The patient only suffered from high blood pressure and was in good health until approximately 2 years before presentation, when he was affected by a depression of mood state.\nA neuropsychological evaluation performed at that time, in 2009, highlighted only an impairment in episodic memory and reduced phonemic fluency and a pathological performance in the Frontal Assessment Battery.\nAt the first visit in our clinic, in January 2011, a general physical examination did not reveal any known pathology. A neurological examination detected hypomimic facies; impaired balance; ataxic-type gait; pyramidal-extrapyramidal hypertonia in all four limbs; reduced strength, especially on the right side; wide-base standing position; and supranuclear upgaze paresis.\nMagnetic resonance imaging (MRI) showed the ventricular–cisternal system in its normal axis, normal morphology and size, and no areas of altered signal in the brain parenchyma. Diffuse cortical atrophy was present.\nSingle photon emission computed tomography (SPECT) showed widespread hypoperfusion of the cerebral cortex, with thinning of cortical thickness. The hypoperfusion of both hemispheres appeared more pronounced in the posterior temporal cortex, with a prevalence in the left hemisphere. Perfusion of the basal ganglia and cerebellar hemispheres was preserved.\nThe patient was assessed by the same neuropsychologist for all follow-ups, using a wide battery of neuropsychological tests to investigate all cognitive functions.