ORIGINAL ARTICLE
Psychiatric Symptoms and Their Psychosocial Consequences in Frontotemporal Dementia Ulla Passant, PhD, MD,* Christina Elfgren, PhD,* Elisabet Englund, PhD, MD,† and Lars Gustafson, PhD, MD*
Abstract: Based on a retrospective study of 19 neuropathologically verified cases with frontotemporal dementia (FTD), neuropsychiatric symptoms related to behavioral disturbances and their psychosocial consequences were studied. The results indicate that frontotemporal dementia is often misdiagnosed early in the clinical course. Behavioural features with impaired social interactions, impaired personal regulation, and loss of insight were seen in all patients. The psychosocial consequences reported in this paper challenge future research in frontotemporal dementia. Key Words: behavioral disorder, frontotemporal dementia, psychosocial consequences (Alzheimer Dis Assoc Disord 2005;19:S15–S18)
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nterest in the frontal lobes and subsequent studies of frontal lobe dysfunction go back a long way. In 1868 Harlow made a good description of altered personality following frontal damage in the classic case report of Phineas Gage.1 Since then many scientists have focused on the functions of the frontal lobes using different physiological, anatomic, neuropsychological, and clinical approaches.2 The focus on frontotemporal dementia (FTD) started when Arnold Pick3 pointed out the relationship between circumscribed lobar atrophy and aphasia and behavioral changes in dementia, thereby contributing to the evolution of the modern neuropsychiatry. Since then much research has been focused on the variety of clinical syndromes associated with frontal and frontotemporal degeneration. Over the years several subtypes and clinical syndromes based on specific neuropsychiatric symptoms relating to regional brain involvement have been described and rediscovered.4–8 Longitudinal studies of patients in Lund and Manchester led to the consensus statement in 19949 on clinical and neuropathological characteristics and to adoption of the term FTD (Table 1), including frontal lobe degeneration,10,11
From the *Department of Psychogeriatrics and †Department of Neuropathology, University Hospital, Lund, Sweden. This study has been supported by the Swedish Alzheimer Foundation, Stiftelsen Ellen och Henrik Sjo¨brings Minnesfond and by the Swedish Research Council (project no 3950). Reprints: Ulla Passant, MD, PhD, Department of Psychogeriatrics, University Hospital, SE 221 85 Lund, Sweden (e-mail:
[email protected]). Copyright Ó 2005 by Lippincott Williams & Wilkins
Pick’s disease, and motor neuron disease with dementia (MMND). To improve the clinical recognition of FTD, a consensus on pure clinical diagnostic criteria was published 4 years later. This consensus also describes the clinical profile of progressive aphasia and semantic dementia.12 In FTD, the behavioral and personality changes are the most prominent symptoms, which usually precede or overshadow the cognitive disabilities. The clinical picture is far from homogenous and may often be misdiagnosed as a psychiatric condition such as depression or psychosis.13,14 Although the early symptoms may vary, most patients develop the core features of behavioral disorder, emotional and speech disturbances in variable proportions and with progress of the disease (Table 1). The changes in personality and behavior observed by the family usually go unnoticed by the majority of the patients. The wide range of behavioral abnormalities such as loss of insight and judgment, impulsivity, disinhibition, poor self care, and loss of social appropriateness have been reported in many studies.11,14–17 However, there is currently little published information about the social consequences of these behavioral symptoms. This paper will describe the psychosocial consequences of psychiatric symptoms in patients with FTD, with emphasis on personality changes and breakdown in social conduct. Furthermore, it will also deal with reports compiled by the National Competence Center for FTD in Lund, which has invaluable contact with many families and caregivers from all over the country.
MATERIALS AND METHODS Nineteen consecutive patients with neuropathologically verified FTD were analyzed. The morphologic diagnoses were Pick’s disease in two cases, frontotemporal lobe degeneration (FLD) in 16 cases (two with a component of corticobasal degeneration) and corticobasal degeneration in one case. The group consisted of 11 men and 8 women (Table 2). The mean age of onset was 57, 5 years, with a range of 30 to 84 years. All medical records were studied retrospectively regarding personality changes and behavioral abnormalities and the possible psychosocial consequences for the patients and their families. All patients were followed repeatedly at our clinic and seen by a specialist with long experience of FTD patients (UP).
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TABLE 1. Clinical Diagnostic Features of Frontotemporal Dementia: Consensus Statement by the Research Groups in Lund and Manchester (1994) (Modified) Core Diagnostic Features Behavioral disorder Insidious onset and slow progression Early loss of personal and social awareness Early loss of insight Early signs of disinhibition Mental rigidity and inflexibility Stereotyped and perseverative behavior Hyperorality Distractibility, impulsivity, and impersistence Utilization behavior Affective symptoms Depression, anxiety, excessive sentimentality, suicidal ideation, delusion Hypochondriasis, bizarre somatic preoccupation Emotional unconcern, lack of empathy Amimia Speech disorder Progressive reduction of expressive speech Stereotypy of speech Echolalia, perservation Late mutism
RESULTS All patients were referred to the department of Psychogeriatrics in Lund for medical assessment but had initially been diagnosed by other physicians. The initial clinical diagnoses were: depression in five patients, manicdepressive disorder in one, psychosis/schizophrenia in four, cerebrovascular disorder in two, aphasia in two, and suspected brain disorder in one. Only 4 patients were diagnosed as dementia and of these only one was diagnosed as FTD. On admittance to our clinic all patients except one were diagnosed as FTD, according to the Lund Manchester criteria.9 One woman presented her first symptoms at the age of 84 and was diagnosed as vascular dementia with frontal clinical features. Three patients presented their first symptoms after the age of 70 years. The duration varied from 2 to 26 years (mean duration 9, 5 years).
Neuropsychiatric Abnormalities and Psychosocial Consequences Alterations in eating and oral behavior were seen in about 75% of the patients. In some cases this resulted in
TABLE 2. Neuropathologically Verified Frontotemporal Dementia Cases Sex M/F Age at onset Age at death Duration
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11/8 57.5 (30–84) 62.3 (34–86) 9.5 (2–26)
hospital admission because of aspiration and in a few cases the patients had eaten inedible things such as flowers, cotton wool, chocolate papers, nails, and screws. Two patients regularly walked into the neighbors’ houses, looking for food in the refrigerators. Two patients, previously teetotalers, started to abuse alcohol, whereas several patients with hitherto modest social drinking habits increased their alcohol consumption. Increased and excessive smoking was seen in several patients and this often resulted in minor incidents, caused by cigarettes left glowing. Loss of concern for personal appearance and neglect of personal hygiene was reported in 8 patients. Offensive language with curses and words with sexual connotations and indecent behavior, like uninhibited singing in restaurants, joining private parties uninvited, and explosive outbursts in public were noted in 10 patients. A variety of other behavioral patterns due to disinhibition, loss of social appropriateness, and lack of insight, such as undressing in public places, public urination, and masturbation were reported in several patients. Physical aggression and signs of hostility were seen in 8 of the 19 patients. This was mostly seen within the family but in a few cases rather severe incidents did happen outside the family. Several patients were involved in traffic incidents and physical outbursts in public, requiring police intervention, whereas two were caught for shoplifting, with one case resulting in arrest. Three patients went missing, entailing police search. Other symptoms and incidents due to disinhibition, impulsivity, and lack of judgment were also seen; two patients set fire to objects (in one case a bed and in the other electric wires). Two became involved in highly inappropriate relationships. One patient insisted on moving in with her ex-husband although he had remarried several years previously. Economical extravagances were noticed in several patients. Two patients were observed giving away large sums of money to strangers and there were huge disproportionate financial transactions with tragic consequences for the family in two cases. Pathologic gambling with subsequent debts and marital discord was seen in one patient. Incidents with regard to driving and passenger behavior were seen; one patient tried to get out of the car on the freeway several times, whereas another one tried to switch off the engine while someone else was driving. Three patients did continue to drive even against the advice of their doctors and also after their driving licenses had been withdrawn. Minor traffic accidents were seen in a few cases. In one family small children were left unattended and locked up in a car. Hoarding and compulsive repetitive behavior were seen in 12 patients and in two patients somatic hypochondriac ideas led to repeated medical referrals. One woman had been referred to 11 different medical specialties over a 2-year period with repeated extensive medical assessments, before she was referred to the Psychogeriatric department. Although other psychiatric symptoms such as delusions and hallucinations have been described in FTD, none of these 19 patients had any such symptoms. q 2005 Lippincott Williams & Wilkins
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The first clinical observations reported by the families were often related to emotional alterations. Very often the spouse expressed that the husband or wife was not the same person any longer, but could not specify exactly what was wrong. This was often interpreted or dismissed as a marital problem or midlife crisis. Early in the course, the professional performance and working ability of several of the younger patients was brought into question, mainly because of their irresponsible behavior. The first contact with a physician often resulted in sickleave due to the diagnosis presented earlier in this paper. The time lapse between the first symptoms and referral to the Psychogeriatric clinic varied from 1 year to 6 years. The spouse and/or other family members often reported frustration and hopelessness during this period. Emergency hospital admissions were seen in all 19 patients, sometimes during the course of the disease, while 9 were committed to psychiatric care at different times. During the course of the disease many of the patients also had prolonged hospital admissions related to the behavioral symptoms and in all cases there were pharmacological difficulties. In most cases family members became the caregivers, not always by choice but often because of lack of flexible health and social services. In all cases social conflicts were reported and in a few families there were severe financial problems as a result of lack of judgment and insight on the part of the patients. Loneliness and social isolation were reported in all families. Being a spouse of a person with FTD is both physically and emotionally exhausting; in almost half of the cases, the spouse had been on sick-leave for certain periods. In several of the families it was obvious that the children, especially those under age, were subjected to stress and did suffer. This was often reported with regard to embarrassing situations in public, which children usually find difficult to handle. Among these 19 families there were two divorces and in one case, the spouse committed suicide. The symptoms and their psychosocial consequences reported here are based on 19 patients at the department of Psychogeriatrics in Lund, the southern part of Sweden. Through our National Competence Center for FTD in Lund we also have a rather unique situation, where we have contact with many families and caregivers from all over the country. This national project was started in 1999 with support from the Swedish National Board of Health and Welfare. One important component in this project is the telephone counseling. This is run by a specialist and a nurse, both with extensive experience with FTD patients. For the first 3 years most inquiries were related to diagnostics and pharmacological treatment. Today about 75% of all phone calls are related to neuropsychiatric symptoms, especially to behavioral disturbances. These are very much the same as those we found among the 19 patients in our study, who exhibited different kinds of inappropriate behavior resulting in social conflicts, traffic incidents, agitation, and physical aggression. Very often we also receive phone calls because the family members, including young children are under stress and do not know where to seek help. q 2005 Lippincott Williams & Wilkins
Psychiatric Symptoms and Psychosocial Consequences in FTD
DISCUSSION In recent years some dementia research has focused on psychosocial consequences, mostly caregiver distress,18,19 but so far there is rather little published information about the consequences of the behavioral disorder in FTD. This study describes neuropsychiatric features mostly associated with behavioral disorder and their social consequences in 19 neuropathologically verified FTD patients. It indicates: Frontotemporal dementia is frequently misdiagnosed and mistaken not only for primary psychiatric disorders but also with neurologic diseases such as cerebrovascular disorders. Only 1 of the 19 patients was initially diagnosed with FTD. Psychiatric features commonly associated with behavioral and social conduct are common in all patients during the course of the disease. The impact of FTD on the patients’ families and the psychosocial consequences are probably underestimated and overlooked. In this presentation, three patients presented their first symptoms after 70 years. Although the appearance of a late onset, over 75, is rare, it has been reported by others.14,16,20,21 Our study supports the alterations in personality and variety of behavioral disturbances associated with FTD described by several groups.11,13,14,16,20,22 Our study shows that in addition to the emotional trauma of a dementia disorder, the behavioral disorder often associated with lack of judgment and impulsivity means that the immediate family also must face extremely stressful and sometimes embarrassing situations related to inappropriate behavior. This may lead to loneliness and isolation, which was also reported in our study. Furthermore, most spouses reported that, due to circumstances, the role of caregiver was imposed upon them. Dementia is always hard on the spouse but FTD is particularly trying because of the symptoms, which may be extremely difficult to cope with. The fact that FTD also affects younger people who may have dependent children makes the disease so much more devastating. Caring for an individual with FTD is most often a fulltime job and our study shows the impact of this dementia disorder, especially on the patients’ families. Caring for patients with FTD necessitates a whole new category of research. REFERENCES 1. Harlow JM. Recovery from the passage of an iron bar through the head. Publications of the Massachusetts Medical Society. 1868;2:327–347. 2. Stuss DT, Bensson FD. The Frontal Lobes. New York: Raven Press; 1986. ¨ ber die Beziehungen der senilen Hirnatrophie zur Aphasie. 3. Pick A. U Prager Med Wochenschr. 1892;17:165–167. 4. Snowden JS, Goulding BJ, Neary D. Semantic dementia: a form of circumscribed cerebral atrophy. Behav Neurol. 1989;2:167–182. 5. Hodges JR, Patterson K, Oxbury S, et al. Semantic dementia: progressive fluent aphasia with temporal lobe atrophy. Brain. 1992;1115:1783– 1806. 6. Edwards-Lee T, Miller BL, Benson DF, et al. The temporal variant of frontotemporal dementia. Brain. 1997;120:1027–1040. 7. Gregory CA. Frontal variant of frontotemporal dementia: a cross-sectional and longitudinal study of neuropsychiatric features. Psychol Med. 1999; 29:1205–1217.
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8. Passant U, Elfgren C, Risberg J, et al. Brain imaging in frontotemporal dementia. Int J Geriatr Psychopharmacol. 2000;2:90–96. 9. The Lund and Manchester groups. Clinical and neuropathological criteria for frontotemporal dementia. J Neurol Neurosurg Psychiatry. 1994;57: 416–418. 10. Brun A. Frontal lobe degeneration of non-Alzheimer type. I. Neuropathology. Arch Gerontol Geriatr. 1987;6:193–208. 11. Neary D, Snowden JS, Northen B, et al. Dementia of frontal lobe type. J Neurol Neurosurg Psychiatry. 1988;51:353–361. 12. Neary D, Snowden JS, Gustafson L, et al. Frontotemporal lobar degeneration. A consensus on clinical diagnostic criteria. Neurology. 1998;51:1546–1554. 13. Gustafson L. Frontal lobe degeneration of non-Alzheimer type. Clinical picture and differential diagnosis. Arch Gerontol Geriatr. 1987;6:209–223. 14. Pasquier F, Lebert F, Lavenu I, et al. The clinical picture of frontotemporal dementia: diagnosis and follow-up. Dement Geriatr Cogn Disord. 1999; 10:10–14. 15. Gustafson L, Brun A, Passant U. Frontal lobe dementia of non-Alzheimer type. Baillieres Clin Neurol. 1992;1:559–582.
16. Gregory CA, Hodges JR. Clinical features of frontal lobe dementia in comparison with Alzheimer’s disease. J Neural Transm. 1996;47:103–123. 17. Miller BL, Darby A, Benson FF, et al. Aggressive, socially disruptive and antisocial behaviour associated with fronto-temporal dementia. Br J Psychiatry. 1997;170:150–155. 18. Donaldson C, Tarrier N, Burns A. Determinants of carer stress in Alzheimer’s disease. Int J Geriatr Psychiatry. 1998;13:248–256. 19. Mourik JC, Rosso SM, Niermeijer MF, et al. Frontotemporal dementia: behavioural symptoms and caregiver distress. Dement Geriatr Cogn Disord. 2004;18:299–306. 20. Miller BL, Cummings JL, Villanueva-Meyer J, et al. Frontal lobe degeneration: clinical, neuropsychological and SPECT characteristics. Neurology. 1991;41:1374–1382. 21. Diehl J, Kurz A. Frontotemporal dementia: patient characteristics, cognition and behaviour. Int J Geriatr Psychiatry. 2002;17:914– 918. 22. Bathgate D, Snowden JS, Varma A, et al. Behaviour in frontotemporal dementia, Alzheimer’s disease and vascular dementia. Acta Neurol Scand. 2001;103:367–378.
AFTD is a nationwide tax-exempt non-profit organization founded in November 2002. The association’s mission is threefold: to promote and fund research into finding the cause and cure for the frontotemporal dementias; to provide information, education, and support to persons diagnosed with FTD and their families and caregivers; and to educate physicians and allied health professionals about FTD. AFTD is governed by a 15-member caregiver Board of Directors and a 16-member Medical Advisory Council comprised of leading FTD clinicians and researchers. For more information about AFTD, contact us at: www.FTD-Picks.org AFTD Board of Directors Helen-Arm Comstock, Pennsylvania – Chair Kent S. Jamison, PhD, Connecticut – Vice Chair Joyce Shenian, Pennsylvania – Recording Secret Bruce L. Richardson, Colorado, Treasurer Joseph Becker, PhD, Washington Fytie Ludington Drayton, Pennsylvania Robert A. Kemp, California Philip H. Lovett, New York Walter S. McKee, Maryland Colleen Quinn, District of Columbia Robert Potamkin, Florida Lisa Radin, New Jersey Kenna Ramirez, Spain Darlene M. Ryan, Texas Joanne Sackheim, California
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AFTD Medical Advisory Council Murray Grossman, MD, EdD, University of Pennsylvania, Chair Thomas D. Bird, MD, University of Washington, Vice Chair Bradley F. Boeve, MD, Mayo Clinic, Rochester Tiffany W. Chow, MD, University of Toronto Bernardino Ghetti, MD, Indiana University Jordan Grafman, PhD, NINDS/National Institutes of Health Michael Hutton, PhD, Mayo Clinic, Jacksonville Andrew Kertesz, MD, FRCP Ó, University of Western Ontario Virginia M.-Y. Lee, PhD, University of Pennsylvania Carol F. Lippa, MD, Drexel University Irene Litvan, MD, University of Louisville M.-Marcel Mesulam, MD, Northwestern University Bruce L. Miller, MD, University of California, San Francisco John C. Morris, MD, Washington University Linda E. Nee, MSW, NINDS/National Institutes of Health John Q. Trojanowski, MD, PhD, University of Pennsylvania
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