What is the management approach for Primary Progressive Aphasia (PPA)?

Management Approach for Primary Progressive Aphasia (PPA)

The management of Primary Progressive Aphasia (PPA) should focus on targeted speech and language therapy interventions that address five core outcome constructs: participating in conversations with family and friends, getting words out, improving fluency, conveying messages by any means, and understanding what others are saying. 1

Understanding PPA Variants

• PPA encompasses three major variants, each requiring different management approaches:
  • Semantic variant (svPPA): Associated with frontotemporal lobar degeneration (TDP-43 type C), characterized by word retrieval difficulties and loss of word meaning 1, 2
  • Nonfluent/agrammatic variant (nfvPPA): Associated with frontotemporal lobar degeneration (tauopathy), characterized by apraxia of speech and grammatical difficulties 1, 2
  • Logopenic variant (lvPPA): Associated with Alzheimer's disease pathology, characterized by word retrieval difficulties and impaired phonological working memory 1, 2

Assessment Approach

• Use a combination of formal and informal assessment tools to evaluate speech and language function 3:
  • Comprehensive language evaluation across multiple domains (expression, comprehension, reading, writing) 4
  • Progressive Aphasia Severity Scale (PASS) to monitor symptom progression across speech, language, and functional communication domains 5
  • Informal interviews with patients and care partners to understand daily communication challenges 3

Intervention Strategy

• Communication partner training should be prioritized as the primary intervention approach 6, 3:

  • Train communication partners (family members, caregivers) in supportive communication strategies 6
  • Practice these strategies in real-life communication contexts 6

• Functional communication interventions focusing on:

  • Building on communication strategies the person already uses 6
  • Practicing these strategies with communication partners 6
  • Setting personalized communication goals based on daily needs 3

• Impairment-focused interventions tailored to specific PPA variant:

  • For naming deficits (common in svPPA): Word retrieval exercises 7
  • For sentence production difficulties (common in nfvPPA): Sentence construction practice 7
  • For speech apraxia (common in nfvPPA): Speech motor programming exercises 7
  • For phonological deficits (common in lvPPA): Phonological training 7

Goal Setting and Outcome Measurement

• Set personalized goals focusing on:

  • Communication aid use 3
  • Conversation skills 3
  • Functional communication in daily activities 3
  • Specific strategy implementation 3
  • Communication partner skills 3

• Measure outcomes using:

  • Goal attainment scaling to track progress toward personalized goals 3
  • Regular reassessment with the PASS to monitor disease progression 5

Future Planning and Additional Considerations

• Address anticipated future communication decline through:

  • Early discussion of communication aids and alternative strategies 3, 8
  • Advance care planning related to communication needs 8
  • Decision-making and mental capacity considerations 3

• Provide support for psychosocial impact:

  • Counseling for patients and families on coping with progressive communication loss 8
  • Connection to support groups and resources 8

Common Pitfalls to Avoid

• Relying solely on standardized aphasia tests designed for stroke patients rather than PPA-specific assessments 3
• Focusing exclusively on impairment-based therapy without addressing functional communication needs 6, 8
• Failing to involve communication partners in therapy 6, 8
• Not planning for disease progression and changing communication needs 8