The Follow-up Of A Patient With Progressive Supranuclear Palsy Having a 6-Year Survival

Abstract

Progressive supranuclear palsy (PSP) is a rare disorder classified clinically among parkinsonism-plus syndromes and pathologically among taupathies. Compared to Parkinson's disease, neurodegenerative changes in PSP are widespread through the brain, which reflect its rich symptomatology. The median survival in PSP varies between 5-7 years. In this paper, we presented a patient with clinically probable PSP followed-up for 5 years in our institution, who passed away at the 6th year of symptom-onset. He initially experienced frequent falls at the age of 60, and with a rapid progression, he became bed confined and required tracheotomy and gastrostomy during the last year of his life. During the last months, although he was conscious, his cooperation was very limited to global limb movements. He had marked facial dystonia and axial rigidity, which caused severe hiperextension of the neck. His eyes were fixed to the upright position with impairment of both vertical and horizontal gaze movements. Oculocephalic reflexes were last, which were preserved during the first years of the disease. Bradykinesia and rigidity were bilaterally present with left predominance as well as grasping. Pyramidal signs consisted of hyperreflexia and pathological reflexes. Since magnetic resonance imaging was contraindicated, only computerized axial tomography scanning was performed and revealed severe atrophy more marked on the mesencephalon, brain stem, basal ganglia and thalamus as well as cerebrum with dilatation of the ventricles. In our opinion, the follow-up of this patient and his videotape recordings archived far 5 years were very didactic in understanding the course of PSP.