A Retrospective Study in Clinical Presentation and Imaging Patterns in Progressive Supranuclear Palsy

Abstract

First described in 1964 by Steele, Richardson, and Olszewski, PSP is the second most common form of atypical parkinsonism but it is still a relatively rare disease, with prevalence estimates ranging from 1.39 to 6.4 per 100,000. Methodology: 18 patients, diagnosed as probable Progressive supranuclear palsy based on MDS criteria, were studied. Age of onset, symptomatology, disease progression, signs of cognitive dysfunction, occulomotor dysfunction, procerus sign, rocket sign, applause sign, rigidity and other extra pyramidal signs were analysed. Patients were subjected to neuroimaging to look for radiological features. Results: Common age of onset was in 5th-6th decade, but as late as 9th decade was noted. Most patients developed recurrent falls after one year of disease onset. Earliest was within 6 months of disease onset. 60% patients experienced dysarthria, usually at 3rd year of the disease. In our study only 3 had dysphagia, happening at 3rd year of the disease. 22% had frontal lobe involvement in form of disinhibition and dysexecution. All patients except 2 had vertical gaze palsy. 52% patients had procerus sign.3 patients had rocket sign and applause sign. Only 11 patients underwent neuro imaging, all 11 patients showed midbrain to pons ratio less than 0.5 and mid brain to pons area ratio less than 0.15. 2 patients in whom MRPI was measured showed a value more than 13.5. Discussion: The MDS criteria 2017 identified four functional domains oculomotor dysfunction, postural instability, akinesia, and cognitive dysfunction. The most disabling symptom of PSP usually relates to balance impairment. Occulo motor abnormalities include, vertical gaze palsy, impairment of saccades, optokinetic nystagmus, and the presence of square wave jerks, blepharospasm and involuntary eye closure. Pseudobulbar symptoms in PSP patients are characterized by dysarthria, dysphagia and pseudobulbar affect MRI of patients with PSP include generalized and brain-stem, particularly midbrain atrophy.