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)
Definition by Dr M. Marcel Mesulam , Northwestern University, Feinberg School of Medicine, Chicago.

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 years)
Duration of isolated language signs and symptoms = 5.1 years (Range 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.
People have described subtypes
Fluent
Semantic
Pure progressive anomia
Primary progressive conduction aphasia
Pure progressive aphemia, progressive anarthria
Primary progressive apraxia of speech
Progressive aphasia
Rapidly progressive aphasia
Nonfamilial dysphasic dementia
Familial aphasia
Hereditary dysphasia dementia

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
Duffy and McNeil:
Treatment of deficits
Compensatory strategies
Psychological concomitants of the disorder
Environmental influences
AAC where it is accepted by the patient and trained before it is needed.

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
Significant increase in referrals to WMH of patients with queried or confirmed PPA. Small percentage of caseload and limited clinical history for service provision. Led to seeking out current treatment options. Literature review. Benchmarking service delivery options. Information from USA centres. Led to decision to review local information and services.
Led to department researching current treatment and service delivery options for PPA.

9. Method
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

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%
The reviews by Duffy and Petersen (19192), Westbury and Bub ( 1997) and Rogers and Alarcon (1999) show a 2:1 male to female ratio.

12. All 20 cases had speech pathology assessment
All 20 cases had speech pathology assessment. 11 had less than 5 individual treatment sessions. 6 cases had greater than 5 sessions of individual intervention (we don’t know if that is direct therapy or other types of intervention). 6 also had up to 5 review sessions. 4 had greater than 5 review sessions. 1 had participated in a conversation group.
17/20 of the clients with PPA received education about their . But half of these people didn’t know they had PPA, so looks like it was aphasia education and strategies rather than specific information about the condition. 15 of the carers received education. Some did not have carers, so showed a high level of the SP involving the carer in intervention. 6 were referred on to other agencies and 3 were referred on to other speech pathologists (incl 1 to the community SP).
None of the speech paths responding to the survey seemed to have a service that they could refer the individual on to that was a specific support group or self-help group. Very knowingly, they referred to how the individuals were not appropriate to be referred on to stroke groups or dementia care groups. That confirmed what we at WMH were concerned about. We know that these individuals are at risk of isolation and depression, yet there is no support network that they can tap into if they wish….

13. Types of Intervention 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

14. 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 “
Comments by respondents reflect that this is a expanding area of practice,
There are many issues around timing of and the disclosing of the Dx to the patient and the carer.
That there are important implications for people presenting to acute hospital settings
There is need for easily accessible information for sp paths to provide to clients and their carers because it is a less common area of practice. But one where information is key.
There is a need for more research.

15. 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
Sp Paths seem to feel it is an area where they have something positive to offer because they all provide some type of intervention beyond Ax
Uniformity…although only referring to 20 cases and 12 Sp Path there was a certain amount of uniformity of intervention.
Support groups….these individuals don’t fit into post stroke aphasia type groups and Sp Paths do not send them in that direction, although their initial impairment may be uncomplicated aphasia. But they don’t fit into dementia specific groups, as they have an absence of memory and cognitive difficulties, but they and their carers will be dealing with issues around progressive disease. Sp Paths were asking for support info or networks. Maybe there is a need for an info pack or an Australian specific website for Sp Paths and individuals with PPA and their carers.
Obviously large number of people not being referred, referral numbers not reflecting likely incidence in the community. So a large number are not being diagnosed or referred or both.

16. 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?
Regardless of the eventual outcome, the early prominence of aphasia and its relative isolation for long periods of time justify its distinction from dementing illnesses. People affected by the disease must cope with a different set of problems than generalized dementia for an extended period of time. Quote from M.M. Mesulam.

17. 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

18. 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),
Louis M. Espesser R. Rey V. Daffaure V. Di Cristo A. Habib M 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,
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

19. 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, Taylor & Francis, United Kingdom
Ratnavalli E. Brayne C. Dawson K. Hodges JR. The prevalence of frontotemporal dementia.[see comment]. [Journal Article] Neurology. 58(11): , 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),
Westbury & Bub (1997) Primary Progressive Aphasia: A review of 112 cases. Brain and Language, 60(3)