Review Question - QID 107289

A patient under the age of 65 with progressive emotional changes (apathy) and personality disinhibition, hyperorality, and frontotemporal atrophy on MRI is likely to be suffering from Pick's disease, also called frontotemporal dementia (FTD).

Frontotemporal dementia is a common form of dementia in patients younger than 65 and typically presents with behavioral and language deficits with a relatively preserved memory. Common behavioral and personality deficits include loss of insight, social inappropriateness, and emotional blunting. Common language features are loss of comprehension and object knowledge, and nonfluent and hesitant speech (progressive nonfluent aphasia).

Cardarelli et al. report on the topic of FTD. They report that 55 percent of patients have FTD with ubiquitin-positive inclusions and 45 percent have FTD with tau-positive inclusions. They also state that FTD should be treated symptomatically with selective serotonin reuptake inhibitors, antipsychotics, and galantamine. Specifically they recommend patients be treated with with selective serotonin reuptake inhibitors for behavioral symptoms such as disinhibition, depressive symptoms, and repetitive behaviors while patients with aggressive behavior or outbursts may be treated with small doses of antipsychotics.

Boxer et al. discuss the role of memantine, a NMDA antagonist, in treating FTD. They report that memantine treatment had no effect on neuropsychiatric symptoms (P=0.47) after 26 weeks of treatment. They also note that patients in the memantine group had more frequent cognitive adverse events (six patients) than those in the placebo group (one) and therefore do not recommend memantine for treatment of FTD.

Illustration A shows axial MRI images of the brain showing atrophy and degeneration of the frontal and temporal lobes, suggestive of frontotemporal degeneration, or Pick's Disease.

Incorrect Answers:
Answer 1: This patient clearly has significant cognitive deficits with affects her daily functioning. This is not normal aging.
Answer 2: This patient has personality and emotional deficits without significant memory deficits. This makes Alzheimer's less likely.
Answer 3: Lewy body dementia characteristically presents with memory loss and visual hallucinations, none of which are found in this patient.
Answer 4: This patient has no risk factors for strokes (hypertension, hypercholesterolemia, smoking) that can result in vascular dementia.