Progressive supranuclear palsy (PSP): Kader Khan died of PSP
Dr KK Aggarwal
1. is characterized by progressive supranuclear ophthalmoplegia, gait disorder and postural instability, dysarthria ( difficulty spcceh), dysphagia (difficulty eating), rigidity, and frontal cognitive disturbance.
2. an estimated prevalence of 3 to 7 per 100,000
3. The two most common are Richardson syndrome (the classic form of PSP) and PSP-parkinsonism.
4. PSP is the most common degenerative form of atypical parkinsonism.
5. The mean age of onset for PSP is approximately 65 years. No cases of PSP have been reported in patients younger than age 40 years.
6. Richardson syndrome (PSP-RS): the most frequent initial feature is a disturbance of gait resulting in falls. Supranuclear ophthalmoparesis or plegia is the hallmark of PSP but may come on later. Dysarthria, dysphagia, rigidity, frontal cognitive abnormalities, and sleep disturbances are additional common clinical features.
7. Patients with classic PSP-RS have a stiff and broad-based gait, with a tendency to have their knees and trunk extended (as opposed to the flexed posture of idiopathic Parkinson disease), and arms slightly abducted. They demonstrate impulsivity, probably from the frontal lobe involvement, and hence tend to lurch and stagger.
8. PSP with predominant parkinsonism (PSP-P) is characterized by asymmetric onset of limb symptoms, tremor, and a moderate initial therapeutic response to levodopa. It may be confused with idiopathic Parkinson disease.
9. Radiologic hummingbird sign (also called the penguin silhouette sign) results from the prominent midbrain atrophy in PSP with a relatively preserved pons, resembling a hummingbird or penguin in silhouette on midsagittal MRI of the brain
10. The diagnosis of PSP during life is based upon the clinical features. Suspicion for PSP is raised when new-onset neurologic, cognitive, or behavioral deficits progress in absence of other identifiable cause in a patient ≥40 years of age. The core clinical features include postural instability, oculomotor deficits, especially vertical gaze palsy, akinesia/parkinsonism,