What is the management of frontotemporal dementia?

Management of Frontotemporal Dementia

The management of frontotemporal dementia (FTD) requires a structured diagnostic approach followed by targeted interventions for behavioral, cognitive, and communication symptoms, with genetic testing for C9orf72 mutation strongly recommended in all possible/probable behavioral variant FTD (bvFTD) cases, especially those with psychiatric features. 1

Diagnostic Approach

• Obtain a detailed history with caregiver input to establish symptom timeline, as impaired insight is almost always present in bvFTD 2
• Assess for key differentiating features between bvFTD and primary psychiatric disorders (PPD):
  • Age of onset (typically 40-70 years for bvFTD) 2
  • Insidious onset with progressive course in bvFTD versus abrupt onset or fluctuating course in PPD 2
  • Family history of FTD, ALS, or other early-onset neurodegenerative diseases 2

Essential Diagnostic Testing

• Brain MRI with 3D T1 sequence and FLAIR is required for all patients with suspected bvFTD 2, 1
• FDG-PET should be considered in diagnostically ambiguous cases 2, 1
• Structured testing of social cognition (e.g., Ekman 60 faces test) 2
• Genetic testing for C9orf72 mutation in all possible/probable bvFTD cases, especially those with strong psychiatric features 2, 1
• Consider testing for other genetic mutations (MAPT, GRN) if family history is positive 3

Behavioral Symptom Management

• Specialized psychiatric assessment to identify treatable psychiatric conditions that may mimic or coexist with FTD 2, 1
• Selective serotonergic antidepressants may help manage behavioral symptoms 4
• Caution with antipsychotic agents due to motor, cardiovascular, and mortality risks 4
• Avoid cholinesterase inhibitors and memantine as they have no consistent positive effects in FTD and may worsen neuropsychiatric symptoms 5, 4

Communication and Swallowing Management

• Speech and language therapy focusing on:
  • Regaining voluntary control over speech and phonation through structured interventions 1
  • Progression from automatic activities to functionally relevant tasks 1
  • For swallowing difficulties: positive practice between old and new movement patterns 1

Cognitive Symptom Management

• Implement structured routines to compensate for executive dysfunction 1
• Create environmental adaptations to support daily functioning 1
• Use goal-setting approaches that accommodate the fluctuating nature of symptoms 1

Occupational Therapy Interventions

• Provide education about FTD as a real, disabling condition with symptoms outside the person's control 1
• Teach self-management strategies:
  • Redirecting attention to reduce symptom focus 1
  • Understanding how stress responses influence nervous system functioning 1
  • Implementing rehabilitation strategies throughout daily routines 1
• Consider vocational rehabilitation for work/study accommodations when appropriate 1

Common Pitfalls and Caveats

• Be aware that some patients may present with non-progressive "phenocopy" FTD, requiring different management approaches 2, 1
• Recognize that FTD symptoms often follow a pattern of remission and exacerbation 1
• Avoid overuse of adaptive equipment in early phases, as this may reinforce maladaptive movement patterns 1
• If aids are necessary, consider them short-term solutions with a plan to progress toward independence 1
• Be vigilant for emerging signs of bvFTD in patients with chronic mental illnesses 2
• Remember that C9orf72 repeat expansion carriers can present with psychiatric symptoms and form a diagnostic challenge given their slow progressive course 2

Multidisciplinary Care

• Implement a multi-disciplinary approach with both psychiatric and neurologic diagnostic expertise in FTD 2, 5
• Include speech therapy, occupational therapy, and physical therapy as part of comprehensive management 1, 5
• Provide caregiver support and education, as FTD management heavily relies on caregiver involvement 3, 5