Progressive supranuclear palsy Flashcards
PSP
Due to degeneration of the brainstem reticular formation.
* Early stages present with impairment of vertical saccades.
* Initial slowing of vertical saccadic velocity, usually affecting down-gaze first.
* Followed by complete vertical saccadic paralysis in due course.
* Horizontal gaze disorders appear later, potentially leading to complete ophthalmoplegia.
Eyelid Apraxia, retraction and blepharospasm
* Frequent square-wave jerks on motility and apraxia of lid opening (difficulty in voluntarily opening eyelids).
* Associated neurological signs: axial rigidity, speech and swallowing difficulty, balance issues.
* History of falls is a common finding. (Fall backwards whereas Parkinsons fall forward)
* Patients may have trouble seeing food on the plate or walking downstairs due to vertical gaze palsy and axial rigidity.
* Progressive dementia develops, with death usually occurring 10 years after disease onset.
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In progressive supranuclear palsy a down-gaze deficit results in difficulty with reading and carrying out even simple tasks such as eating a meal from a plate. Base-down deviating prisms can help the patient when eating their food.
Parkinson’s Disease
May be associated with impaired up-gaze, while down-gaze is usually unaffected.
* Saccades may be hypometric but have normal velocity.
* Convergence insufficiency may occur.
* Prolonged tonic elevation of the eyes (oculogyric crisis) has been described in postencephalitic Parkinsonism.