Primary progressive aphasia (PPA) is a blanket term for a group of neurodegenerative disorders whose main feature is speech and language dysfunction. There are semantic variants, nonfluent or agrammatic variants, and logopenic variants. There are no curative treatments yet. Speech and language pathologists have developed impairment-based interventions and compensatory strategies, however, though certain institutional barriers to their implementation remain to be addressed.
Word retrieval interventions are one of the impairment-based approaches to therapy. Studies show that they can be of benefit. It is unclear how generalisable the gains are, and how long they are maintained. Generalisation appears more likely in the nonfluent and logopenic variants, though they degrade quickly in the absence of continuing practice. Individually-tailored training sets, with pictures of participants’ own items, promote relearning and maintenance the best. It may be that the provision of a verb or noun facilitates successful sentence production, and it may also help to supplement spoken word retrieval treatment with written naming. Script training is another technique that may improve fluency. Preliminary research suggests that the core symptoms of agrammatism and apraxia of speech can be successfully addressed. Gains have been observed for treated verb tenses, and so has generalised improvement on untrained verbs. There has been some observed stability in speech production over the year following treatment. Findings in nonfluent PPA so far have also shown significant improvement in accurate production of scripted content and general intelligibility and grammaticality. Intelligibility for untrained topics and gains in accurate production of trained scripts have also been maintained up to 1 year post-treatment.
Very little research exists on compensatory-based approaches in PPA. What studies exist focus on communication skills training or augmentative and alternative communication development. It is common for SLP’s to prioritise communication skills training above impairment-based interventions. It is a practical approach, after all, targeting everyday conversation, underpinned by strategies to facilitate communication (such as gesture) and remedy barriers to communication (such as interruptions or abrupt topic changes). Recent study of facilitative techniques in semantic PPA are encouraging, and more systematic, randomized, trials will be underway shortly.
There are as well books and smart phone apps that can support activities of daily living, such as shopping and cooking. Augmentative techniques like these can help meet the complex communication needs of people with PPA beyond compensatory strategies.
Group education and support, for people with PPA and their families, is another general avenue for help, providing opportunities for practice and problem-solving with other people. Research shows that people with PPA and their families do feel more confident after participating in such groups.
Therapeutic models are evolving, generally toward a person-centered approach. ‘Staging’ offers impairment-based interventions focusing on remediation and rehabilitation in the early stages of PPA, and then moving to compensatory strategies if restoration has failed and language skills have been lost. This can be adapted to individual needs, and dynamic interaction with clinicians. The Life Participation Approach for Aphasia and the CARE Pathway model are two such examples, models that invert the traditional intervention that begins with standardized diagnostic assessment, and starts instead by identifying individuals’ goals and expectations and addressing the self-reported barriers along the way.
There remain some impediments to widespread SLP intervention in PPA. These range through professional skepticism about the efficacy of speech-language interventions, spotty availability of speech and language therapy services for PPA, and still fairly limited speech and language therapy research in PPA. In the US, provision is also made difficult sometimes by the complexities of Medicare and insurance benefit arrangements.
It remains to educate healthcare providers on the potential benefit of speech and language therapy for people with PPA, to train speech and language therapists across graduate school programmes about PPA, to develop a set of evidence-based speech and language therapy clinical practice guidelines for assessment and management of PPA, and to advocate for more widely available services and insurance reimbursement for speech and language therapy for PPA. For individual patients, it is also important to identify the variables that impact the potential benefits of their treatment, which may include such things as the presence of an engaged care partner in treatment sessions. Identifying suitable candidates for different approaches at each stage of disease severity is also fundamental. Some interventions are difficult to test with conventional trial methods.
MyoNews from BreatheWorksTM is a report on trends and developments in oromyofunctional disorder and therapy. These updates are not intended as diagnosis, treatment, cure or prevention of any disease or syndrome.