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What Every SLP Should Know About Progressive Supranuclear Palsy

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What Every SLP Should Know About Progressive Supranuclear Palsy • Strained voice, slow rate of speech, emotional lability and palilalia (the Mission – compulsive repetition of utterances in context of increasing rate and decreasing Increase awareness of loudness) progressive supranuclear palsy, • Language and cognitive deficits have been observed and can limit therapy efforts corticobasal degeneration, and related brain diseases; Speaker with PSP Strategies: • Use compensatory strategies to enhance fund research toward cure and intelligibility PSP prevention; educate healthcare • Speech must become a conscious effort • Breathe first, and speak loudly and slowly professionals; and provide support, • Keep sentences short information and hope for affected What Every • Repeat entire sentence when necessary vs. persons and their families. isolated word • Say one sentence at a time without SLP immediate repetition • Establish the context Should Know About • Use gestures • LSVT® techniques emphasizing increased Progressive Supranuclear phonatory effort may be of benefit • Investigate assistive forms of communication considering visual, cognitive, and motor Palsy limitations such as communication board or SGD Laura Purcell Verdun, M.A., CCC/SLP • Consider personal portable amplifier Sandra McWilliams, M.A., CCC/SLP Listener Strategies: • Keep comments & questions brief • Stick with familiar topics, and one topic at a time • Use “yes / no” question format Executive Plaza III • Ask for clarification, “Did you say...?” 11350 McCormick Road, Suite 906 Hunt Valley, MD 21031 • Provide choices to ease decision making www.curepsp.org Phone: 410-785-7004 Toll Free: 800-457-4777 Canada: 866-457-4777 Fax: 410-785-7009 respiratory infections and respiratory deaths • VFSS if conducted, needs to replicate the WHAT IS PROGRESSIVE more common in PSP than PD home eating environment, and rule out non- SUPRANUCLEAR PALSY (PSP)? • Early swallowing evaluation and frequent neurogenic contributions monitoring of swallowing function allow for • Decompensation and aspiration may not be • Originally described as Steele-Richardson- problem anticipation and use of supportive observed in a controlled VFSS environment, Olzewski syndrome (1964) measures thus severity of dysphagia may not be truly • A rare, aggressive, neurodegenerative disease • Goal of swallowing therapy is to minimize appreciated which is the most common parkinsonian complications such as aspiration pneumonia • Discussions regarding feeding tube options disorder after Parkinson disease (PD) and malnutrition through early symptomatic should take place sooner rather than later, • Often misdiagnosed as PD treatment and repeated frequently • Presents with early postural instability, • No efficacious approach to speech therapy • Person w/PSP and family should agree in supranuclear gaze palsy (paralysis of voluntary has been documented for this patient advance with MD about what is hoped to be vertical gaze with preserved reflexive eye population accomplished with a feeding tube movements), and levadopa-non-responsive • Therapy program should be simple and parkinsonism enhance functional communication as Treatment Strategies: quickly and efficiently as possible vs. just • Optimize oral hygiene • Onset of symptoms is typically symmetric speech outcomes alone • Supervision at mealtimes • Pathologically classified as a tauopathy • Moist, soft, tender foods; avoid dry, (abnormal accumulation in the brain of the particulate, textured foods protein tau) SWALLOWING IN PSP • Keep the plate in the line of vision • There are currently 3 described variations Feeding & Swallowing Symptoms: • Maintain head in a chin-tucked position of PSP: Richardson’s syndrome; PSP- Parkinsonism (PSP-P); and PSP-Pure akinesia • Difficulty looking down at the plate • Restrict liquid and food bolus volumes w/gait freezing (PSP-PAGF) • Mouth stuffing and rapid drinking • Make sure food is swallowed before taking more • Prevalence is 6 per 100,000 people; men > • Tremor or stiffness interfere with self-feeding women • Restricted head & neck posture or • Put cup and utensils down between bites hyperextension and sips • Onset usually in the early 60’s but may begin in • Look for mealtime adaptive devices the 40’s • Delayed pharyngeal swallow onset • Poor cough including cups, plates and utensils to assist • Life expectancy is 7 years following symptom with self-feeding • Occasional difficulty opening the mouth onset • Medications with a puree consistency • Person w/PSP may lack awareness of Speech Pathology Considerations: swallowing difficulties • Ask the neurologist about anti-cholinergic drugs or botulinum toxin for management of • Management of swallowing & speech in PSP Swallowing Management: secretions requires changing intervention strategies as the disease progresses • Clinical swallow evaluation should include mealtime observations and suggestions to • Changes in swallowing and speech often promote easier and safer swallowing COMMUNICATION IN PSP occur early in PSP and are typically more • Query the caregiver about swallowing severe and deteriorate more rapidly than in Motor Speech Symptoms: symptoms PD • Mixed dysarthria typically including • Family should maintain a journal of • Index of suspicion for dysphagia in PSP hypokinetic and spastic dysarthria, less observations to help define and adjust should be high as it is one of the most commonly ataxic common causes of mortality, with recurrent management strategies • Speech is hesitant and halting, with strained Mission – voice and slower speech production • Slowed rate of speech is a reliable marker Increase awareness of for acquired apraxia of speech progressive supranuclear palsy, • Initially may have intact written language • Yes—No reversal corticobasal degeneration, and related brain diseases; Speaker with CBD Strategies: fund research toward cure and • Use compensatory strategies to enhance CBD intelligibility prevention; educate healthcare • Speech must become a conscious effort professionals; and provide support, • Use short phrases and simpler language information and hope for affected because of increased errors with increased What Every rate of speech, number of syllables, and persons and their families. complexity of language SLP • Optimize use of written language • Use of gestures may be limited by apraxia Should Know About • Alert to “yes / no” confusion • Investigate assistive forms of communication Corticobasal Degeneration considering cognitive and motor limitations, such as communication board or SGD Listener Strategies: Laura Purcell Verdun, M.A., CCC/SLP • Eliminate distractions to reduce background noise • Face the speaker with CBD • Keep comments & questions brief • Stick with familiar topics, and one at a time • Use “yes / no” question format • Ask for clarification, “Did you say…?” Executive Plaza III 11350 McCormick Road, Suite 906 Hunt Valley, MD 21031 www.curepsp.org Phone: 410-785-7004 Toll Free: 800-457-4777 Canada: 866-457-4777 Fax: 410-785-7009 • Early swallowing evaluation and frequent thus severity of dysphagia may not truly be WHAT IS CORTICOBASAL monitoring of swallowing function allow for appreciated DEGENERATION? problem anticipation and use of supportive • Discussions regarding feeding tube options measures should take place sooner rather than later, • Originally described in 1968 by Rebeiz, • Goal of swallowing therapy is to minimize and repeated frequently Kolodny, and Richardson; with earliest complications such as aspiration pneumonia • Person w/CBD and family should agree in descriptions by Charcot (1888) and malnutrition through early symptomatic advance with MD about what is hoped to be • A rare neurodegenerative disease ­characterized treatment accomplished with a feeding tube by apraxia, non-fluent aphasia, frontal • No efficacious approach to speech therapy dementia, limb rigidity and dystonia, alien limb has been documented for this patient Treatment Strategies: phenomenon, and postural instability population • Optimize oral hygiene • Onset of symptoms is markedly asymmetric • Therapy program should be simple and • Supervision at mealtimes enhance functional communication as • Pathologically classified as a tauopathy • Use less-affected side for self-feeding quickly and efficiently as possible vs. just (abnormal accumulation in the brain of the • Maintain head in a chin-tucked position speech outcomes alone protein tau) • Avoid highly textured, particulate foods • Diagnosis is difficult because clinical features • Blend multiple consistency foods often over-lap with Parkinson disease (PD), SWALLOWING IN CBD • Proceed with caution with thin liquids progressive supranuclear palsy, Alzheimer’s, • Alternate food and liquid swallows primary progressive aphasia, and fronto- Feeding & Swallowing Symptoms: • Medications with a puree consistency temporal dementia • Impaired self-feeding • Look for mealtime adaptive devices • Onset between 60 – 80 y.o. • Slow or incomplete chewing including cups, plates, and utensils to assist • Prevalence of 5 – 7 per 100,00; women > men • Oral and swallowing apraxia with self-feeding • Slowed swallowing movements • Life expectancy is 7-10 years following • Consider smaller, more frequent meals due symptom onset • May be aware of swallowing difficulties to increased length of time when eating • Ask the neurologist about the role of Swallowing Management: Speech Pathology Considerations: anti- cholinergic drugs or botulinum toxin for • Management of swallowing & speech • Clinical swallow evaluation should include management of secretions assist with self-
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