What is the treatment plan for frontotemporal dementia?

Treatment Plan for Frontotemporal Dementia

No FDA-Approved Disease-Modifying Therapies Available

There are currently no FDA-approved medications for frontotemporal dementia, and treatment focuses on symptomatic management through non-pharmacological interventions, behavioral support, and selective use of medications for specific symptoms. 1, 2, 3

Core Management Framework

Multidisciplinary Team Approach

• 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. 4
• Caregiver support and education should be prioritized, as families experience severe burden associated with caregiving. 4, 5
• Focus on alleviating caregiver burden through legal and financial planning advice early in the disease course. 5

Non-Pharmacological Interventions (First-Line Treatment)

Occupational Therapy Interventions

• Provide education about FTD as a real, disabling condition with symptoms outside the person's control, explaining that symptoms result from potentially reversible miscommunication between brain and body. 4
• Teach 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. 4
• Focus on structured routines to compensate for executive dysfunction. 4
• Implement environmental adaptations to support daily functioning and goal-setting approaches that accommodate the fluctuating nature of symptoms. 4
• Consider vocational rehabilitation to support work/study accommodations when appropriate. 4

Speech and Language Therapy

• Speech therapy should focus on regaining voluntary control over speech and phonation through structured interventions, progressing from automatic activities to functionally relevant tasks. 4
• 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. 4

Pharmacological Management (Symptomatic Only)

Behavioral Symptoms

• Selective serotonin reuptake inhibitors (SSRIs) are the most evidence-supported pharmacological option for behavioral symptoms in FTD, with multiple studies reporting therapeutic benefits. 1, 3, 6, 5
• Stimulants may help with disinhibition, apathy, and risk-taking behavior. 3
• Second-generation antipsychotics may help individual patients with agitation and psychosis, but should be used with extreme caution due to motor, cardiovascular, and mortality risks. 6, 5

Medications to AVOID

• Cholinesterase inhibitors and memantine should be avoided as they have been ineffective in FTD and may worsen symptoms. 3, 6, 5
• Anti-dementia drugs approved for Alzheimer's disease have demonstrated no efficacy in FTD. 1, 5

Critical Pitfalls to Avoid

Equipment and Adaptive Devices

• Avoid overuse of adaptive equipment in early/acute phases, as this may reinforce maladaptive movement patterns. 4
• If aids are necessary, consider them short-term solutions with a plan to progress toward independence. 4
• Avoid use of splints or devices that immobilize joints. 7

Environmental Modifications

• Careful consideration of environmental changes to cope with abnormal behaviors is essential rather than relying on medications. 5
• Complete a relapse prevention and ongoing self-management plan as part of treatment. 7

Special Considerations

Genetic Forms of FTD

• Genetic testing for C9orf72 mutation should be strongly considered in all possible/probable bvFTD cases, especially those with strong psychiatric features. 4
• New therapies for genetic forms of FTD (mutations in MAPT, GRN, and C9orf72) are moving into clinical trials but remain years away from clinical availability. 5

Non-Progressive "Phenocopy" FTD

• Be aware that some patients may present with non-progressive "phenocopy" FTD, which requires different management approaches. 4
• Recognize that FTD symptoms often follow a pattern of remission and exacerbation, requiring flexible approaches to goal-setting. 4

Disease-Modifying Therapies Under Investigation

• Treatments targeting tau protein aggregation or TDP-43 clearance are in development, but recent Phase III trials (such as LMTM for behavioral variant FTD) have been negative. 1, 5
• Disease-modifying treatments remain years away from clinical availability. 1