The first step in making an accurate diagnosis of FTD is to rule out other possible explanations of the symptoms, such as Alzheimer's disease, a primary psychiatric condition or Parkinson's disease.

A basic FTD work up should include a patient history and exam, neuropsychological testing, a family history and an MRI. Beyond that there are several other tests that can help narrow down a diagnosis.

Diagnostic criteria to confirm a diagnosis of definite, probable and possible FTD are being developed by an international consortium. The key clinical and pathological feature of FTD is the focal, often asymmetric, cortical involvement.