Management of Frontotemporal Dementia

Communication and Swallowing Management

Speech and language therapy should focus on regaining voluntary control over speech and phonation through structured interventions, progressing from automatic activities to functionally relevant tasks, and addressing psychosocial factors that may affect communication and swallowing in patients with frontotemporal dementia 1
For swallowing difficulties, therapy should include positive practice between old and new patterns of movement, consolidation of normalized behaviors into wider social contexts, and addressing psychosocial factors that may affect communication and swallowing in patients with frontotemporal dementia 1

Cognitive rehabilitation should focus on structured routines to compensate for executive dysfunction in patients with frontotemporal dementia 2
Environmental adaptations to support daily functioning and goal-setting approaches that accommodate the fluctuating nature of symptoms are recommended for patients with frontotemporal dementia 3

Education about frontotemporal dementia as a real, disabling condition with symptoms outside the person's control, and explanation that symptoms result from potentially reversible miscommunication between brain and body, should be provided to patients and their families 3
Teaching self-management strategies, including redirecting attention to reduce symptom focus, understanding how stress responses influence nervous system functioning, and implementing rehabilitation strategies throughout daily routines, is recommended for patients with frontotemporal dementia 3
Vocational rehabilitation to support work/study accommodations when appropriate is recommended for patients with frontotemporal dementia 3

Genetic testing for C9orf72 mutation should be strongly considered in all possible/probable bvFTD cases, especially those with strong psychiatric features, according to the Brain journal 4, 5
Specialized psychiatric assessment is recommended to identify treatable psychiatric conditions that may mimic or coexist with frontotemporal dementia, as suggested by the Brain journal 4, 6
Brain MRI with T1 and FLAIR sequences is essential for diagnosis, with FDG-PET recommended in ambiguous cases, according to the Brain journal 5, 7

Avoid overuse of adaptive equipment in early/acute phases, as this may reinforce maladaptive movement patterns in patients with frontotemporal dementia 3
If aids are necessary, they should be considered short-term solutions with a plan to progress toward independence in patients with frontotemporal dementia 3
Be aware that some patients may present with non-progressive "phenocopy" frontotemporal dementia, which requires different management approaches, as suggested by the Brain journal 6
Recognize that frontotemporal dementia symptoms often follow a pattern of remission and exacerbation, requiring flexible approaches to goal-setting in patients with frontotemporal dementia 3

Obtain a detailed history with caregiver input to establish symptom timeline, as impaired insight is almost always present in behavioral variant frontotemporal dementia (bvFTD), typically affecting individuals between 40-70 years of age, with a progressive course, and often a family history of FTD, ALS, or other early-onset neurodegenerative diseases 8, 9, 10
Assess for key differentiating features between bvFTD and primary psychiatric disorders (PPD), including age of onset, insidious onset with progressive course, and family history of FTD, ALS, or other early-onset neurodegenerative diseases 9, 10

Brain MRI with 3D T1 sequence and FLAIR is required for all patients with suspected bvFTD, and FDG-PET should be considered in diagnostically ambiguous cases, with structured testing of social cognition, such as the Ekman 60 faces test 10
Genetic testing for C9orf72 mutation is recommended in all possible/probable bvFTD cases, especially those with strong psychiatric features, with a consideration for testing for other genetic mutations (MAPT, GRN) if family history is positive 10, 11

Be aware that some patients may present with non-progressive "phenocopy" FTD, requiring different management approaches, and recognize that FTD symptoms often follow a pattern of remission and exacerbation, with emerging signs of bvFTD in patients with chronic mental illnesses 9, 10
C9orf72 repeat expansion carriers can present with psychiatric symptoms and form a diagnostic challenge given their slow progressive course 12

Implement a multi-disciplinary approach with both psychiatric and neurologic diagnostic expertise in FTD, including speech therapy, occupational therapy, and physical therapy as part of comprehensive management, with a consideration for caregiver support and education 12