BACKGROUND: A 48 year-old woman presented with an 18 month history of bizarre and complex delusions on a background of social, behavioral and cognitive decline over several years. Her psychosis progressed despite receiving high doses of antipsychotics. The patient's father also had a psychotic episode in his 40s. He subsequently developed motor neuron disease, which caused his death at 68 years of age.
INVESTIGATIONS: Physical examination, neuropsychological testing, nerve conduction studies, brain MRI and transcranial magnetic stimulation.
DIAGNOSIS:
On the basis of the patient's age at onset of the delusions, imaging findings and family history, a diagnosis of frontotemporal dementia (FTD) was favored over a
primary psychotic disorder. The ubiquitin-positive and TAR DNA binding protein 43-positive inclusions that were found at autopsy confirmed the diagnosis of FTD.
MANAGEMENT: The patient was treated with various antipsychotics at high doses; however, her delusions continued to progress. No disease-specific treatments for FTD currently exist.
