primary progressive aphasia and current speech pathology services in nsw n.
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Primary Progressive Aphasia and Current Speech Pathology Services in NSW

Primary Progressive Aphasia and Current Speech Pathology Services in NSW

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Primary Progressive Aphasia and Current Speech Pathology Services in NSW

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  1. Primary Progressive Aphasia and Current Speech Pathology Services in NSW Cathleen Taylor and Rachel Miles Speech Pathology Department War Memorial Hospital, Waverley NSW

  2. Today’s presentation: • PPA information • WMH review of NSW Speech Pathology intervention for individuals with Primary Progressive Aphasia • Results of review to date • Discussion points and plans for the future

  3. Primary Progressive Aphasia • “Primary Progressive Aphasia (PPA) is a clinical dementia syndrome characterized by the gradual dissolution of language without impairment of other cognitive domains for at least the first two years of illness.” (M.M. Mesulam, 1982, 2001)

  4. What do we know about PPA?Incidence: • “We know of no studies of incidence or prevalence of PPA.” (Weintraub & Mesulum, 2005) • Evidence that frontotemporal dementia constitutes the second most common form of neurodegenerative dementia (Ratnavalli et al, Neurology 2002). • One quarter of dementias are atypical and some of these will be PPAs (McNeil and Duffy, 2001) • Hundreds of patients with PPA have been described (Mesulum, 2003) • Australia? NSW?

  5. Demographics:(Duffy & Petersen (1992), Westbury & Bub ( 1997) and Rogers & Alarcon (1999) • 2:1 male to female ratio • Average age of onset: 60.5 years (Range 17-81 years) • Duration of isolated language signs and symptoms = 5.1 years (Range 1.5-20 yrs)

  6. Clinical presentation : • “patients with PPA come to medical attention because of the onset of word finding difficulties, abnormal speech patterns and prominent spelling errors”(Mesulam, 2003) • Fluent (SD) and non-fluent (PNFA) varieties, but clinical picture varies depending on distribution of the disease process. • Depression and frustration common • No defining clinical test. Post-mortem examination not definitive.

  7. Management/Treatment: • Small number of available studies regarding treatment. • Single case studies describing direct treatment (McNeil et al, 1995, Schneider et al, 1996, Murray, 1998, Graham et al, 1999, Laurence et al, 2002, Louis et al, 2001.) • Proactive Management (Rogers et al, 2000): • Minimising activity limitation and participation restrictions • Maximising communication competence through development and training of AAC and strategies • Duffy & McNeil (2001) support combination of all possible interventions

  8. Impetus For WMH Study • Significant increase in referrals to WMH Speech Pathology • Is our experience atypical? • Is speech pathology accessible for all PPA pts? • What’s happening elsewhere? • What the clinical pathway? Best practise for treatment? • Literature review re: PPA • Need for a review of current local services

  9. Developed surveys for both Speech Pathologists and Neurologists / Geriatricians Distributed SP survey to 30 sites across NSW Based on July 2004 – June 2005 Questions based on referral patterns, demographics and intervention provided Method

  10. Results • Data collection ongoing until end of November 2005 • 26 completed surveys returned thus far. • 12 completed surveys provided information on 20 cases of queried or confirmed PPA referred to their service. • Respondents spread throughout metropolitan areas and rural areas. • Received from acute hospital, inpatient, outpatient rehabilitation, domiciliary and private services

  11. Demographics of PPA clients • 13 females, 7 males • 10 were aware of Dx of PPA • 10 unaware of Dx of PPA • 60 % of cases Sp Path findings used to assist with Dx • Referred by LMO 10% Neurologist 50% Geriatrician 35% Self 5%

  12. Semantic Rx Naming therapy Word finding strategies Fluency Rx Non verbal language Total communication techniques AAC Life books/personal portfolios Communication books Drawing Facilitated conversation Education to carers Types of Intervention

  13. Other SP comments • “Dementia/Alzheimer’s area is a huge new area for us to move in to” • “ In the acute setting inconclusive diagnosis of PPA would be challenging for discharge planning.” • “..we can be a great resource to this client population and their carers.” • “…need for more research, case studies” • “…need more services, awareness and information” • “..a little understood area. Lack of support groups “

  14. Discussion points • All Speech Pathologists referred PPA clients provided some form of intervention • Uniformity of intervention strategies across respondents • Support groups? • The need to promote the role of the SP with this population to referring agencies • Referral numbers not reflecting probable incidence

  15. Factors to consider… • “The hiatus in the diagnosis can delay aphasia management and general life planning” • Added strain and impact of delay in formal diagnosis • Life planning issues…when should intervention begin to deal with this? • How can SPs give education when a diagnosis is not confirmed? • How can a care plan be developed?

  16. Where to from here….? • Awaiting further responses from rural SP services • Distribute survey to neurologists and geriatricians • Communicate analysed collated information to Speech Pathologists • Generate discussion amongst profession re: optimal management for individuals with PPA • Explore opportunities to provide more treatment and support for this group

  17. References and Bibliography • Croot, K. Communication Disruptions in Dementia of the Alzheimer Type and Primary Progressive Aphasia: Impairment-, and Activity/Participation-based Interventions. Speech Pathology Australia Neurology Focus day, Sydney, November 09, 2002. • Duffy, J.R. & Petersen, R.C. (1992). Primary progressive aphasia. Aphasiology, 6(1) 1-15. • Graham,K.S., Patterson, K.H & Hodges, J.R. (1999). Relearning and subsequent forgetting of semantic category exemplars in a case of semantic dementia. Neuropsychology, 13(3), 359-380. • Louis M. Espesser R. Rey V. Daffaure V. Di Cristo A. Habib M. 2001 Intensive training of phonological skills in progressive aphasia: a model of brain plasticity in neurodegenerative disease. • Laurence, F, Manning, M & Croot, K (2002) Impairment-based interventions in primary progressive aphasia: Theoretical and clinical issues. Brain Impairment, 3, 157-158. • McNeil, M.R., Small, S.L., Masterton, R.J. & Fossett, T.R.D. (1995). Behavioural and pharmacological treatment of lexical-semantic defictis in a single patient with primary progressive aphasia. American Journal of Speech-Language Pathology, 4, 76-87

  18. References and Bibliography • Mesulam,M.M,, Current Concepts: Primary Progressive Aphasia – A Language-Based Dementia. New England Journal of Medicine. • Murray, L (1998). Longitudinal treatment of primary progressive aphasia: A case study. [Peer Reviewed Journal] Aphasiology. Vol 12(7-8) Jul-Aug 1998, 651-672. Taylor & Francis, United Kingdom • Ratnavalli E. Brayne C. Dawson K. Hodges JR. The prevalence of frontotemporal dementia.[see comment]. [Journal Article] Neurology. 58(11):1615-21, 2002 Jun 11 • Rogers & Alarcon (1999). Characteristics and management of primary progressive aphasia. ASHA Special Interest Division Neurophysiology and Neurogenic Speech and Language Disorders, 9(4), 12-26 • Schneider, S.L., Thompson, C.K., & Luring, B. (1996). Effects of verbal plus gestural matrix on sentence production in a patient with primary progressive aphasia. Aphasiology, 10(3), 297-317. • Westbury & Bub (1997) Primary Progressive Aphasia: A review of 112 cases. Brain and Language, 60(3) 381-406