DISEASE SUMMARIES at a glance
DISEASE SUMMARIES at a glance
Progressive Supranuclear Palsy( PSP)
• Rare neurodegenerative brain disease, the most common parkinsonian disorder after Parkinson’ s disease( PD)
• Originally described and named in 1964 by Steele, Richardson and Olszewski of the University of Toronto
• Often mistakenly diagnosed as PD due to the similar early symptoms
• Symptoms include early postural instability, supranuclear gaze palsy( paralysis of voluntary vertical gaze with preserved reflexive eye movements), and levodopa-nonresponsive parkinsonism
• Onset of symptoms is typically symmetric
• Pathologically classified as a tauopathy( abnormal accumulation in the brain of the protein tau)
• Five to seven cases per 100,000 people
• Average age of onset is 60 – 65 years, but can occur as early as age 40
• Life expectancy averages 7.4 years after symptom onset
• No cure or effective medication management
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Signs and Symptoms
• Early onset gait and balance problems
• Clumsy, slow or shuffling gait
• Lack of coordination
• Slowed or absent balance reactions and postural instability
• Frequent falls( primarily backward)
• Slowed movements
• Muscle stiffness, mostly at neck
• Reduced range and speed of up and down eye movements
• Difficulty opening or closing the eyelids
• Decreased blinking with“ staring” look
• Blepharospasms( involuntary eyelid spasms)
• Double vision
• Dystonia, commonly at neck and hands into flexion, but can also be into extension at neck
• Cognitive loss emphasizing difficulty creating and organizing one’ s thoughts
• Strained, loud, slurred speech
• Reduced swallowing control, starting with liquids
• Behavioral changes, typically social withdrawal and reduced impulse control