Progressive Supranuclear Palsy

I've just started a community job and am having to learn more about progressive conditions. Here's some info on PSP.

PSP

• Neurodegenerative parkinsonian disorder.
• Typically presents in middle/late age with progressive unexplained postural instability, falls, supranuclear gaze palsy, pseudobular palsy and mild cognitive disturbances.
• Cause: progressive damage to the basal ganglia and brainstem.
• Begins slowly and leads to continuous decline.

Observations

• Personality changes (apathy/forgetfulness/irritability/lability)
• Blurring of vision, problems controlling eye movement e.g. problems looking down/ with eye contact.
• Slurred speech and dysphagia (often more severe than in Parkinsons).

Dysphagia

• Pneumonia = the most common cause of death
• People may live up to 10 years with proper interventions.

Leopold et al (1997) Dysphagia in progressive supranuclear palsy: radiologic features, dysphagia, 12(3), 140-3.

Key features

• Uncoordinated lingual movements
• Absent velar elevation/retraction
• Impaired lingual displacement
• copious pharyngeal secretions
• non cohesive lingual transfer
• oral bolus leakage into the pharynx
• significant vallecular residue
• reduced oral sensation

Key observations

• Difficulties chewing/ with the oral stage of swallowing mean that softer foods may be easier. 85% of the patients with PSP had oral stage difficulties. 
• Most of the patients had pharyngeal stage problems which may have been caused by the oral stage impairments e.g. pharyngeal overspill.

Example Recommendations

• Avoid mixed textures/ hard chewy foods.
• Decrease distractions
• Put the plate high up if the person cannot look down.
• Encourage a clearing cough post swallow
• Make the patient more aware of the oral stage e.g. write Step 1, 2, 3, 4.

Observations from a videofluoroscopy of a patient with PSP

• decreased coordination of lingual movements, especially the base of tongue. Pre-swallow loss of bolus'.
• +++ vallecular residue on soft food (banana + yogurt) but no aspiration.
• Small amount of aspiration on thin fluids when taken after eating.
• No aspiration on syrup thickened fluids taken after eating - this helped clear the vallecular residue.

Recommendations from the VF

1. Syrup thick fluids at mealtimes.
2. Sips of thin fluids throughout the day (for QOL)
3. SOft mashed diet.