[Frontotemporal dementias].

Area of Science:

• Neurology
• Neuroscience
• Genetics

Context:

• Frontotemporal dementias (FTD) represent a significant cause of dementia in individuals aged 45-65.
• FTD encompasses rare genetic variants, behavioral variant FTD, primary progressive aphasias, and FTD with motor neuron symptoms.
• TDP-43 protein accumulation is a common pathological hallmark in several FTD subtypes.

Purpose:

• To review the clinical classification, diagnostic criteria, and pathological underpinnings of Frontotemporal Dementias.
• To outline current therapeutic strategies, including pharmacological and non-pharmacological interventions for FTD.
• To highlight the challenges in FTD treatment due to a lack of causal therapies and limited evidence for medical interventions.

Summary:

• FTD is clinically categorized into behavioral variant, primary progressive aphasias, and FTD with motor neuron symptoms.
• Revised diagnostic criteria for behavioral variant FTD focus on specific behavioral and cognitive features.
• Primary progressive aphasias are subtyped based on linguistic and imaging characteristics, with potential overlap with movement disorders like Parkinsonism.

Impact:

• Current treatments for FTD are largely symptomatic, lacking causal therapies and possessing low levels of evidence.
• Pharmacological interventions include antidepressants, antipsychotics, anticonvulsants, and cholinesterase inhibitors, targeting specific symptoms.
• Non-pharmacological approaches such as physical, occupational, and speech therapy, alongside patient and family education, are crucial for managing FTD and ensuring safety.