Differential Diagnosis

Neuroimaging can evaluate for other conditions that mimic frontotemporal dementia (FTD) (e.g., white matter diseases, frontotemporal focal lesions, frontal lobe tumors, and cerebrovascular disease). The clinical manifestations of GRN-FTD significantly overlap with those of other conditions including FTD with or without parkinsonism associated with pathogenic variants in MAPT, Parkinson disease, Alzheimer disease, Pick disease (OMIM 172700), other inherited FTD disorders, corticobasal degeneration, progressive supranuclear palsy, and Creutzfeldt-Jacob disease (OMIM 123400). This clinical overlap makes it difficult to predict which family has a GRN pathogenic variant by clinical presentation alone. Up to 50% of individuals with FTD have a positive family history of dementia, usually with autosomal dominant inheritance. below lists the most common genes associated with familial FTD. Table 2. Genes in the Differential Diagnosis of GRN Frontotemporal Dementia

Source: GeneReviews — "GRN Frontotemporal Dementia"

Management Guidelines

Evaluations Following Initial Diagnosis

To establish the extent of disease and needs in an individual diagnosed with GRN frontotemporal dementia (GRN-FTD), the following evaluations (if not performed as part of the evaluation that led to the diagnosis) are recommended:

• Detailed general, neurologic, and family history

• Physical examination

• Neurologic examination

• Cognitive examination. When clinical cognitive assessments are not informative enough, a neuropsychological assessment may be performed to provide a more comprehensive and objective view of a patient's cognitive function. Formal neuropsychological assessment requires comparison of the patient's raw score on a specific test to a large general population normative sample which is usually drawn from a population comparable to that of the person being examined. This allows for the patient's performance to be compared to a suitable control group, adjusted for age, sex, level of education, and/or ethnicity. While much more sensitive than bedside clinical cognitive examination, such assessment is resource intensive and time consuming.

• Discussion of capabilities for job and for driving

• Discussion of advanced care planning

• Consultation with a clinical geneticist and/or genetic counselor

Treatment of Manifestations

There is currently no known treatment for GRN-FTD or FTD in general. Psychosocial support is essential in the management of FTD and should include occupational therapy and environmental and physical interventions.

Source: GeneReviews — "GRN Frontotemporal Dementia"

Therapies Under Investigation

Search ClinicalTrials.gov in the US and EU Clinical Trials Register in Europe for access to information on clinical studies for a wide range of diseases and conditions. Note: There may not be clinical trials for this disorder.

Source: GeneReviews — "GRN Frontotemporal Dementia"