Clinical Guidance

GRN frontotemporal dementia (GRN-FTD) generally affects the frontal and temporal cortex leading to behavioral changes, executive dysfunction, and language disturbances. In GRN-FTD, the parietal cortex and basal ganglia may be affected as well, resulting in parkinsonism, cortical basal syndrome, and memory impairment [, , , , , , ]. Age of onset. The age of onset of GRN-FTD ranges from 35 to 87 years with a mean of 64.9 ± 11.3 years [, , , ]. Comparison studies demonstrate that onset age in individuals with GRN-FTD does not differ significantly from that in individuals without an identified GRN pathogenic variant , while some studies suggested a younger onset age in those with GRN-FTD . Neurocognitive symptoms.

Diagnostic Criteria

Suggestive Findings

GRN frontotemporal dementia (GRN-FTD) should be suspected in individuals with the following clinical presentations and neuroimaging findings.

Clinical Presentations

Clinical presentations of GRN-FTD vary widely both among and within families and may resemble behavioral variant FTD (bvFTD), primary progressive aphasia (PPA), atypical parkinsonism, or corticobasal syndrome. Behavioral variant FTD

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:

Surveillance Guidelines

Patients are often followed in a memory disorder clinic or a similar multidisciplinary clinic involving neurologic and psychiatric services and follow-up medical care.

Source: GeneReviews — "GRN Frontotemporal Dementia"