Medicine

July 26th, 2020

Progressive Supranuclear Palsy

Clinicals - History

Introduction

Progressive supranuclear palsy (PSP) is an adult-onset, rapidly progressive neurodegenerative disease characterized by intracerebral aggregation of tau protein. A definite diagnosis of PSP requires a neuropathological examination.


PSP occurs in 5-6% of patients with parkinsonism. Most patients are between 63 and 66 years old and have a mean survival of 5 to 9 years. Males are more commonly affected than females.


PSP is the most common “Parkinson-plus” syndrome. The cause of PSP is not known.

History of falls

Progressive supranuclear palsy is characterized by early frequent falls, particularly falling backwards.


Falls are multifactorial and may result from axial rigidity, bradykinesia, freezing, reduced/absent postural reflex, visual-vestibular deficits, or decreased insight due to frontal lobe disturbance.

Unsteady gait

Patients may show a clumsy, slow and unsteady gait ("drunken sailor gait").

Visual symptoms

Visual symptoms include blurred vision, photosensitivity, diplopia, and difficulty reading. These result from the inability to look down and from convergence insufficiency.

Behavioral changes

Behavioral changes include emotional withdrawal, apathy, depression, disinhibition, dysphoria, anxiety, irritability, and agitation. Pseudobulbar symptoms (laughing or crying incongruous with mood) can also occur.

Parkinsonism

Parkinsonism features associated with progressive supranuclear palsy include asymmetric tremor, bradykinesia, axial rigidity, and early gait and postural instability.

Dysphagia/dysarthria

Dysarthria and dysphagia are present early in the disease course. These are due to pseudobulbar palsy.

Cognitive decline

Patients show progressive cognitive decline, marked by slowed processing, reduced verbal fluency, and executive dysfunction. This results from progressive frontal subcortical dementia.

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