What is the best management approach for a patient with primary progressive aphasia?

Management of Primary Progressive Aphasia

The best management approach for primary progressive aphasia centers on person-centered speech-language therapy that prioritizes functional communication goals—specifically participating in conversations with family and friends, getting words out, being more fluent, conveying messages by any means, and understanding what others say—rather than traditional impairment-based approaches alone. 1, 2

Core Management Principles

Shift from Impairment-Based to Functional Communication Goals

Traditional clinical priorities that emphasize impairment-based approaches should be replaced with or preceded by compensatory strategies that address what matters most to patients and their families. 1 The 2025 international consensus identified five priority outcome constructs that should guide all therapeutic interventions:

1. Participating in conversations with family and friends 1, 2
2. Getting words out 1, 2
3. Being more fluent 1, 2
4. Conveying a message by any means 1, 2
5. Understanding what others are saying 1

These patient-centered outcomes represent a fundamental departure from focusing solely on word accuracy or linguistic precision. 1

Speech-Language Therapy as Primary Intervention

Speech-language therapy is the cornerstone of PPA management, as there are currently no curative treatments or effective pharmacological therapies to reverse or halt disease progression. 3, 4 All treatment must therefore be palliative, designed to manage symptoms and improve quality of life. 5

Specific Therapeutic Approaches:

• Personalized word retrieval training tailored to the individual's specific communication needs and goals 3, 6
• Communication partner training to optimize interactions with family members and caregivers 7, 3
• Compensatory strategy training that emphasizes alternative methods of conveying messages beyond verbal speech 3
• Technology integration to facilitate functional communication goals, including computerized treatment as a supplement 7, 3

Variant-Specific Considerations

The therapeutic approach should be tailored to the specific PPA variant, though functional communication goals remain paramount across all types:

Semantic variant (svPPA): Characterized by difficulties in word retrieval and loss of word meaning, associated with frontotemporal lobar degeneration (TDP-43 type C). 1, 2 Focus on compensatory strategies for conveying meaning when specific words are lost. 8

Non-fluent/agrammatic variant (nfvPPA): Characterized by apraxia of speech and/or grammatical difficulties, associated with frontotemporal lobar degeneration (typically primary tauopathy). 1, 2, 7 Address motor speech planning deficits alongside grammatical impairments. 7

Logopenic variant (lvPPA): Characterized by word-finding difficulties with preserved word meaning but impaired phonological working memory, commonly associated with Alzheimer's disease pathology. 1, 2, 7 Target phonological processing and working memory support strategies. 8

Multicomponent Care Model

The Communication Bridge Approach

A comprehensive care model should include person-centered care with dyadic instruction (involving both patient and care partner) for disease education, counseling, and tailored levels of both impairment-based and compensatory communication strategy training. 3 This approach incorporates:

• Client-directed assessment that identifies personally meaningful communication goals 3
• Interventions aimed at maximizing functional communication in desired life activities 3
• Direct and indirect technology integration to facilitate goal achievement 3
• Shared decision-making with a holistic, dementia-inclusive approach encompassing physical, mental, emotional, psychosocial, and spiritual dimensions 5

Multidisciplinary Team Involvement

Optimal care requires a multidisciplinary medical team from the onset of language difficulties, not just at diagnosis. 5 The majority of those affected receive little or no follow-up care after diagnosis, particularly in the early stage of disease—this represents a critical gap in care. 5

Emerging Therapeutic Modalities

Transcranial Direct Current Stimulation (tDCS)

Home-based tDCS combined with personalized word retrieval training is feasible and may enhance speech-language therapy effects, though larger controlled studies are needed to confirm effectiveness. 6 The protocol involves:

• 20 daily sessions over 1 month, each with 45 minutes of personalized word retrieval training 6
• tDCS over the left inferior frontal gyrus (anode F7, cathode O1) at 2.0 mA during the first 30 minutes 6
• Remote supervision is feasible for home-based delivery 6

This approach is well-tolerated with no treatment-limiting adverse events and shows improved naming for trained items and confrontation naming. 6 However, this should be considered investigational until larger trials confirm clinical benefit.

Treatment Delivery Considerations

Intensity and Format

Intensive treatment is probably indicated for aphasia, though the optimal intensity for PPA specifically requires further study. 7 Treatment can be delivered through:

• Individual therapy sessions 7
• Group therapy (shows no significant difference in outcomes compared to individual therapy) 7
• Home-based and remotely supervised interventions 6

Pharmacotherapy

Pharmacotherapy may be considered on a case-by-case basis in conjunction with speech therapy, though no specific regimen is recommended for routine use. 7 There are currently no disease-modifying therapies available. 3, 4

Critical Pitfalls to Avoid

Do not delay referral to speech-language therapy until later disease stages. Early intervention from the onset of language difficulties is essential. 5 Multiple barriers currently limit access to care, including low awareness among referring clinicians and insufficient training of speech-language therapists in PPA-specific interventions. 4

Do not focus exclusively on impairment-based measures at the expense of functional communication goals. When patients are unable to identify their own goals because they are unclear about the role of the speech-language therapist, offering them a range of possible intervention goals based on the five core outcome constructs facilitates the goal-setting process. 1

Do not use a one-size-fits-all approach. The core outcome set should be used carefully and complement measures that are more proximal to the specific intervention being provided. 1 For example, a peer support intervention may impact participation in conversation but not necessarily understanding what others say. 1

Prognosis and Long-Term Management

The course of PPA is typically progressive, requiring ongoing adjustment of therapeutic strategies throughout the disease trajectory. 2, 8 Treatment must evolve from early-stage interventions focused on maintaining verbal communication to later-stage compensatory approaches that maximize any means of conveying messages. 1