Clinical Geriatrics

CASE PRESENTATION
Mrs. P, a 54-year-old Caucasian female, was seen by her primary care provider five years ago with a complaint of “memory problems.” She noted episodes of forgetfulness from as early as age 46. Mrs. P’s husband had noted changes in both her personality and memory. He reported out-of-character use of profanity and a gradual onset of apathy. She had progressively become less attentive to daily routines at home. Mrs. P complained of episodes where she had forgotten the route home, and reported difficulty remembering steps in routine tasks she had performed repeatedly for years.

Further evaluation revealed a maternal family history of early-onset dementia. The patient’s mother was diagnosed with Alzheimer’s disease and was placed in long-term care at an early age. Mrs. P initially attributed her changes in mood and cognition to menopause in her early 40s. Her medical history was significant for degenerative changes of the spine and mitral valve prolapse. Mrs. P denied any history of psychiatric hospitalizations, psychiatric treatments, head injury, or substance abuse. She has been married for 38 years. She completed nine years of school and never worked outside the home. She has one adult son and two grandchildren. Mrs. P’s husband is disabled and relies upon her as his primary caregiver.

In her early 50s she was referred for neuropsychological testing, which revealed that her earliest symptoms were forgetting names, familiar neighbors, and close relatives. Mrs. P was surprised to have forgotten a familiar recipe for making biscuits that she had used for years by memory. She also reported episodes of forgetting to give her husband his medications. Full scale IQ was measured at 89. A Folstein Mini-Mental State Examination was scored at 29 out of 30 items. An evaluation by Neuropsychology initially diagnosed depression with no cognitive deficits. Two interval exams by Neuropsychology over a two-year span revealed cognitive deterioration with evidence of frontal lobe involvement. At the age of 53, Mrs. P was referred to a University-affiliated outpatient psychiatric clinic for further evaluation.

DISCUSSION
Frontotemporal lobar dementia (FTLD) is a progressive dementia characterized by early onset and progressive deterioration of the patient’s personality and social functioning. Early predominant symptoms vary among patients and are described within three distinct syndromes: frontotemporal dementia, progressive nonfluent aphasia, and semantic dementia. Frontotemporal lobar dementia is thought to be the third most common nonvascular cause of cortical dementia.1 Alzheimer’s and Lewy body dementias are the most common causes of nonvascular cortical dementia. Frontotemporal lobar dementia is thought to be equivalent in prevalence to Alzheimer’s disease in patients under 65 years of age.2 Along with early age of onset, typical findings in patients with FTLD include early behavioral changes, early emotional blunting, and early loss of insight. Supportive features include a decline in executive functioning and a relatively well-preserved short-term memory. In contrast to Alzheimer’s disease, FTLD is diagnosed by a consensus criterion.3 The prevalence of FTLD is estimated to be 15 per 100,000.4

Some research indicates that many patients with FTLD meet the criteria for diagnosis of Alzheimer’s dementia.5 It has been suggested that the best way to distinguish these separate diagnostic entities is to be familiar with the FTLD consensus criteria for diagnosis.6 Early behavioral and language changes in a younger patient coupled with a relatively intact episodic memory are examples of findings that can distinguish FTLD from Alzheimer’s disease (Table).

Patients are often diagnosed during mid-course of their illness due to the early and slow onset of FTLD.