Frontotemporal Dementia Complicated by Comorbid Borderline Personality Disorder: A Case Report

| April 23, 2009 | 0 Comments

by LCDR Stephen Salzbrenner, MD; Jaime Brown; Gavin Hart; ENS Jonathan Dettmer; LT Raquel Williams, MD; LT Monica Ormeno, DO, USN; LCDR Ethel O’Neal, MD; and LT Jennifer Shippy, MD

Dr. Salzbrenner is an Attending Staff Psychiatrist at Naval Medical Center in Portsmouth, Virginia; Ms. Brown is a third-year medical student at Edward Via Virginia College of Osteopathic Medicine; Mr. Hart is a third-year medical student at Eastern Virginia Medical School; ENS Dettmer is a fourth-year medical student at Uniformed Services University of the Health Sciences; Dr. Williams is a second-year Psychiatry Resident at Naval Medical Center in Portsmouth, Virginia; Dr. Ormeno is a second-year Psychiatry Resident at Naval Medical Center in Portsmouth Virginia; Dr. O’Neal is a third-year radiology resident at Naval Medical Center in Portsmouth, Virginia; and Dr. Shippy is a first-year psychiatry resident at the Naval Medical Center, Portsmouth, Virginia.

Psychiatry (Edgemont) 2009;6(4):28–31

Funding

There was no funding for the interviews, development, and writing of this article.

Financial disclosure

The authors report no relevant conflicts of interest or commercial ties with respect to this material.

Disclaimer

Drs. Salzbrenner, Williams, Ormeno, O’Neal, and ENS Dettmer are military service members. This work was prepared as part of their official duties. Title 17 U.S.C.105 provides that ‘Copyright protection under this title is not available for any work of the United States Government.’ Title 17 U.S.C. 101 defines a United States Government work as a work prepared by a military service member or employee of the United States Government as part of that person’s official duties.

Abstract

Frontotemporal dementia is the fourth most common cause of dementia in the United States and characteristically presents with an early decline in social conduct, impaired regulation of interpersonal conduct, emotional blunting, and general loss of insight, with relative preservation of memory. This a case of frontotemporal dementia in a 46-year-old woman who presented with existing diagnoses of borderline personality disorder and major depressive disorder. She had been repeatedly evaluated for suicidality and mood lability, which led to her most recent hospitalization, the first at our facility. When the individual’s outpatient psychiatrist was contacted following admission, the patient’s major depressive disorder was considered largely refractory to treatment. It is the opinion of the authors that the patient’s history of borderline personality disorder and the fact that frontotemporal dementia is a relatively uncommon condition delayed her accurate diagnosis. This case illustrates the difficulty faced by the clinician in recognizing a relatively uncommon condition, frontotemporal dementia, when a patient presents with psychiatric symptoms.

Key Words

frontotemporal, dementia, borderline, personality, disorder, disinhibition

Introduction

This case emphasizes the importance of recognizing an atypical presentation of a well-documented clinical syndrome, frontotemporal dementia, specifically in a patient previously diagnosed with borderline personality disorder.

Case Report

The patient was a 46-year-old woman who presented after a suicide attempt with less than 12oz of a strawberry daquiri and 175mg of diphenhydramine. The patient denied any specific precipitants, claiming only, “I did not want to worry about things anymore.” Collateral information provided by family members indicated a progressive decline in socially acceptable impulse control of at least five years duration. A representative event occurred two years ago, when the patient was fired from her teaching position for threatening to kill a student.

Her past psychiatric history was significant for three prior psychiatric admissions, each following a suicide attempt, as well as bulimia and a remote history of self mutilation and a pattern of binge drinking. She had an existing diagnosis of major depressive disorder (MDD) and had been treated with a selective serotonin reuptake inhibitor (SSRI) for 11 months. Social history was notable for an ongoing 25-year marriage with two grown children.

On exam, the patient was alert and oriented but was abrupt, issuing monosyllabic “yes” or “no” answers before the examiner finished the questions. Her speech was impoverished with slightly delayed responses lacking inflection and had a quiet, child-like, muffled tone. Her mood was “worried” and affect was anxious, timid, and fearful, alternating between labile and constricted. The patient appeared tearful at times; however, an abrupt diversion of her attention seemed to relieve intermittent surges of affect. Rigidity of thought was also noted. Thought process was generally linear, but her sentences tended to end abruptly.

Clinical tools, such as the Mini Mental Statues Exam (MMSE) and Frontal Behavioral Inventory (FBI), were utilized with the following outcomes: 28/30 (MMSE) and 21/72 (FBI). A score of 17 or higher on the FBI is 100-percent sensitive and 63-percent specific for frontotemporal dementia (FTD), while a score of 27 or higher is 90-percent sensitive and 100-percent specific.[1]

The patient’s hospital stay was remarkable for multiple episodes of disinhibited behavior. On several occasions, the patient inappropriately hugged psychiatrists, psychiatric technicians, and nurses. Additionally, the patient exposed herself several times to ward staff. Following her initial interview with the treatment team, the patient was given a working diagnosis of MDD. Borderline traits were also acknowledged in the patient, including binge drinking, self mutilation, and emotional lability dating back to age 16.

Laboratory evaluation of the patient demonstrated a complete blood count, comprehensive metabolic panel, ceruloplasmin, serum copper, B12, and thiamine levels that were within normal limits. Heavy metal screen and urine drug screen were negative. Thyroid-stimulating hormone (TSH) was slightly elevated on admission, with T4 within normal limits. Ultrasensitive TSH was then tested and was normal. Assays for thyroid autoantibodies were negative and a rapid plasma regain was nonreactive. The patient underwent initial magnetic resonance imaging (MRI) of the brain, which demonstrated diffuse atrophy of the frontal and temporal lobes (Figure 1). Comparision MRI study of a normal 51-year-old woman is shown in Figure 2. This age-inappropriate atrophy, the patient’s unremarkable laboratory workup, and her continued bizarre behavior prompted the acquisition of a positron emission tomography (PET) scan, which revealed significant frontal and temporal lobe hypometabolism (Figure 3). The abnormal imaging in the context of the patient’s unusual clinical picture supported a diagnosis of FTD.

Background

The symptoms of frontotemporal lobar dementia (FTLD) were first characterized by Arnold Pick in 1892 as a type of frontal lobe degeneration.[2] Subsequently, there have been a number of refinements in the clinical syndrome, pathological findings, and diagnostic criteria. In 1998, Neary et al[3] reported on a consensus statement of a conference of internal investigators concerning the current clinical and pathologic diagnostic criteria for the disease, proposing a definition for FTLD and its subtypes. Currently there are three neurobehavioral syndromes associated with FTLD: FTD, progressive nonfluent aphasia, and semantic dementia. The most defining features of all three subtypes of FTLD are insidious onset and alternations in behavior and personality, with a relative preservation of memory and visuospatial skills.[3]

The most common type of FTLD is FTD, which characteristically presents with profound alternations in personality and conduct. These changes can manifest as inertia, social disinhibition, distractibility, and loss of volition, with relative preservation of memory.[3] Additional prominent features can include an early decline in social conduct, impaired regulation of interpersonal conduct, emotional blunting, and general loss of insight.[4] Mega and Cumming noted that patients with frontal-subcortical dysfunction present with disorders of action rather than of perception or stimulus integration. Consequently, the key features of FTD are altered executive function, depressed mood, impulsivity, and disinhibition.[5] Frontal-subcortical circuits are important in the development of the affective and behavioral symptomatology observed in demented and nondemented individuals. Damage to these regions has been shown to be a common substrate for affective disorders in neurological diseases.[6]

Neuroimaging plays an essential role in establishing a diagnosis of FTD. Classically, MRI demonstrates atrophy of the frontal and temporal lobes, while PET scan illustrates hypometabolism of these same areas. Compared to the characteristic progressive waveform slowing seen in Alzheimer’s disease, electroencephalogram (EEG) often does not show any abnormalities in FTLD.[7]

Since the development of criteria for FTLD, epidemiological studies have shown the disease to be overall the fourth most common form of dementia, and the second most common form of early-onset dementia (age <65 years). Johnson et al[5] studied a sample of 353 patients with FTLD from three university clinics in the United States and Germany, reporting an average age of onset for FTD of 57 (±9.7) years.[8] In addition to an earlier onset, FTLD has also been shown to progress faster than AD. Roberson et al[9] studied 177 patients with FTLD and 385 patients with AD. Their analysis demonstrated that patients with FTD had a median survival of 8.7 (±1.2) years from first symptom and 3.0 (±0.5) years from presentation. Comparatively, AD patients had a median survival of 11.7 (±0.6) years from first symptom and 5.7 (±0.1) years from presentation.[9]

Discussion

The frontal lobe has been described as the “senior executive” of the brain and personality, facilitating and mediating information processing and exerting inhibitory control over emotional expression, perception, and thought formation.[10] Therefore, it is reasonable to expect that dysfunction of the frontal lobe will present with deterioration in many aspects of everyday living. This dysfunction was evident in our patient (i.e. threatening her student and overly affectionate behavior toward hospital staff). However, our patient had seen multiple mental health professionals for her self mutilation, mood lability, and suicidality since she was 16. We feel that our patient’s preexisting borderline traits possibly prevented the earlier recognition of her FTD clinical syndrome. See Table 1 for a comparison of FTD to borderline personality disorder.

Symptoms suggestive of psychiatric illnesses have been observed in a variety of dementia patients. Joseph10 reviewed case reports, MRIs, and PET and CT scans that covered approximately 50 years of cases. He found that frontal dementias can mimic “blunted” schizophrenia, major depression, impulsivity, confabulatory verbosity, grandiosity, increased sexuality, mania, and obsessive compulsive personality disorder.[10] Lopez et al[11] studied the relationship between frontal-subcortical dysfunction and depressive symptomatology in patients with FTD and AD. The authors used psychiatric evaluations, diagnostic criteria from the DSM-III, and the Hamilton Depression Rating Scale for the basis of their assessment. Results of this study demonstrated that patients with FTD exhibited more syndromal major depression, irritability, mood lability, anxiety, agitation, disinhibition, and social withdrawal than patients with AD.[11] With our patient, we employed a FBI to further differentiate between AD and FTD and to follow the behavioral aspects of her illness.

We felt this case to be interesting because of our patient’s confounding psychiatric symptoms and existing psychiatric diagnoses. Our patient had displayed a number of borderline traits and depressive symptoms since adolescence, most likely contributing to the delay in the diagnosis and treatment of her FTD. However, several key features of her illness support a diagnosis of FTD, including the atrophy of her frontal and temporal cortices on MRI with corresponding decreased metabolic activity on the 18F-fluoro-2-deoxy-D-glucose PET, her relatively early age of onset, her waning executive function, and her progressive behavioral changes.

The early age of onset and the grave prognosis of FTD necessitate the prompt recognition and diagnosis of this condition. This case illustrates the difficulties faced by the clinician in recognizing a relatively rare condition, such as FTD, when the clinical picture is complicated by comorbid psychiatric symptoms.

Acknowledgment

The authors would like to express their gratitude and appreciation to LCDR Craig Carroll, DO, Staff Neurologist at Naval Medical Center Portsmouth, for his assistance with the management of this case.

References

1. Kertesz A, Davidson W, Fox H. Frontal behavioral inventory: Diagnostic criteria for frontal lobe dementia. Can J Neurologic Sci. 1997; 24(1):29–36.
2. Kertesz A. Frontotemopral dementia/Pick’s disease. Arch Neurol. 2004;61:969–971.
3. Neary D, Snowden J, Gustafson L, et al. Frontotemporal lobar degeneration: a consensus on clinical diagnosis. Neurology. 1998;51(6):1546–1554.
4. Goetz C. Textbook of Clinical Neurology. Philadelphia, PA: Elsevier, 2007.
5. Mega M, Cummings J. Frontal-subcortical circuits and neuropsychiatric disorders. J Neuropsychiatr Clin Neurosci. 1994;6:358–370.
6. Cummings J. Frontal-subcortical circuits and human behavior. Arch Neurol. 1993;50:873–880.
7. Josephs KA. Frontotemporal lobar degeneration. Neurol Clin. 2007;25(3):683–696.
8. Johnson J, Diehl J, Mendez M, et al. Frontotemporal lobar degeneration: demographic Characteristics of 353 patients. Arch Neurol. 2005;62:925–930.
9. Roberson E, Hesse J, Rose K, et al. Frontotemporal dementia progresses to death faster than Alzheimer disease. Neurology. 2005;65:719–725.
10. Joseph R. Frontal lobe psychopathology: mania, depression, confabulation, catatonia, perseveration, obsessive compulsions, and schizophrenia. Psychiatry. 1999;62:138–172.
11. Lopez O, Gonzalez M, Becker J, et al. Symptoms of depression and psychosis in Alzheimer’s disease and frontotemporal dementia. Neuropsychiatry Neuropsychol Behav Neurol. 1996;9:154.

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Category: Alzheimer's Disease, Case Report, Dementia, Neurologic Systems and Symptoms, Neurology, Past Articles, Personality Disorders, Psychiatry, Schizophrenia

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