Two cases of frontotemporal dementia with predominant temporal lobe atrophy

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PSYCHOGERIATRICS 2009; 9: 204–207

doi:10.1111/j.1479-8301.2009.00298.x

CASE REPORT

Two cases of frontotemporal dementia with predominant temporal lobe atrophy psyg_298

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Hideaki SHIMIZU,1 Kazuhiko HOKOISHI,2 Ryuji FUKUHARA,1 Kenjiro KOMORI1 and Manabu IKEDA3

1 Department of Neuropsychiatry, Neuroscience, Ehime University Graduate School of Medicine, Ehime, 2Department of Psychiatry, Japan Self Defense Forces Hanshin Hospital, Hyogo, and 3 Department of Psychiatry and Neuropathobiology, Faculty of Medical and Pharmaceutical Sciences, Kumamoto University, Kumamoto, Japan

Correspondence: Dr Hideaki Shimizu MD, Department of Neuropsychiatry, Neuroscience, Ehime University Graduate School of Medicine, Shitsukawa, Toon-city, Ehime 791-0295, Japan. Email: [email protected] Received 19 May 2009; accepted 27 August 2009.

Key words: clinical symptoms, frontotemporal dementia, Gogi aphasia, semantic dementia, temporal lobe.

Abstract Frontotemporal dementia (FTD) is a subtype of frontotemporal lobar degeneration, which also includes semantic dementia (SD) and progressive nonfluent aphasia. Frontotemporal dementia is characterized by changes in personality and behavioral abnormalities, generally associated with predominant frontal lobe atrophy. Conversely, SD is typically characterized by Gogi (word meaning) aphasia based on semantic memory impairment and is associated with predominant temporal lobe atrophy. However, in the present cases, we diagnosed FTD on the basis of clinical symptoms, such as disinhibition, indifference, and stereotypy, without semantic memory impairment, even though neuroimaging showed predominant temporal lobe atrophy. We suggest that clinical symptoms are the most important cues for an accurate clinical diagnosis and there is no exclusive relationship between the syndrome and atrophy of the temporal lobes.

INTRODUCTION Frontotemporal lobar degeneration (FTLD) is the preferred term to describe primary cerebral degeneration involving the frontal and/or temporal lobes associated with non-Alzheimer’s pathology.1 There are three different clinical syndromes of FTLD depending on the distribution of atrophy within the frontal and temporal lobes: (i) frontotemporal dementia (FTD); (ii) semantic dementia (SD); and (iii) progressive non-fluent aphasia. Predominant frontal lobe atrophy and behavioral abnormalities, such as disinhibition, indifference, and stereotypy, are observed early after the onset of FTD; in contrast, predominant temporal lobe atrophy and Gogi (word meaning) aphasia and/or selective semantic memory impairment, such as the inability to recognize familiar faces or famous buildings, are observed early after the onset of SD.1,2 The core features of Gogi aphasia in patients with SD are the selective loss of the semantic memory for words or selective dysfunction in use of semantic aspects of words, together with well-preserved phonologic and syntactic aspects of language.3 Gogi aphasia has 204

been found in Japan4 and it is clearly reflected in disturbances in the written language as a form of surface dyslexia,5 as well as in reading kanji character words, which are more regulated by meaning than kana characters. Patients with SD always have temporal atrophy, but the presence of temporal atrophy does not inevitably denote the clinical syndrome of SD.6 Patients with FTD invariably have both frontal and anterior temporal lobe atrophy and, in a few cases, the temporal lobe atrophy is profound or greater, even in the absence of obvious semantic memory impairment.7 Here, we report on two patients with clinically diagnosed FTD1 without SD-specific Gogi aphasia, even though neuroimaging showed marked temporal lobe atrophy and very mild frontal lobe atrophy.

CASE REPORTS Case 1 This patient was a 73-year-old right-handed woman who had been treated for hypertension over the past © 2010 The Authors Journal compilation © 2010 Japanese Psychogeriatric Society

FTD with predominant temporal lobe atrophy

10 years and had a family history of cerebral hemorrhage (father) and Parkinson’s disease (mother). Prior to the onset of illness, she had been bright and sociable. At 70 year of age, she gave up her hobbies (hula dancing and yoga) because of aspontaneity. Thereafter, her eating habits changed and she became disinhibited (e.g. laughing loudly at inappropriate times). She showed stereotypy (rhythmic repetitive striking) and began to complain of exhaustion. Three years after the onset of these symptoms, the patient did not eat anything other than bananas, pudding, alcohol, or coffee, resulting in a body weight loss of 15 kg over a period of 5 months. She was referred to a local physician, who diagnosed depression and prescribed antidepressants. In the absence of any improvement, the patient was admitted to a neighborhood psychiatric hospital due to aspontaneity and compromised nutritional status. On admission, results of a hematological examination (hemoglobin level 17.4 g/dL) indicated dehydration and drip infusion was started. Head computed tomography (CT) revealed predominant temporal lobe atrophy, suggesting degenerative dementia. The patient was referred to the Higher Brain Function Clinic of the Department of Psychiatry, Ehime University Hospital because her symptoms (aspontaneity, disinhibition, stereotypy etc.) persisted after discharge. Further examinations did not reveal any abnormal neurological findings. The patient presented with disinhibition (inappropriate loud laughing, banging on tables etc.) and had no insight regarding her illness. She could recall three objects, with no apparent episodic memory impairment in daily life. She could calculate, draw a cube, and imitate finger movements with no disorientation. Although the patient had had trouble finding the right words (e.g. she often referred to things as ‘this’ or ‘that’), phonemic cue effects were preserved and there was no apparent impairment in

Figure 1 Head computed tomography of Case 1 revealed marked bilateral temporal lobe atrophy with mild frontal lobe atrophy.

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word meanings. She was able to complement proverbs and accurately read idiomatic Japanese, reading and ), in which kanji character words (e.g. the pronunciation of the word is arbitrary and independent of any standard pronunciation of the component characters. However, she sometimes said ‘I don’t know’ as an immediate answer to questions put to her, indicating that she was lazy about thinking. The patient’s Clinical Dementia Rating (CDR) was 1. A head CT revealed marked bilateral temporal lobe atrophy with less conspicuous frontal lobe atrophy (Fig. 1). The patient was diagnosed as FTD because she showed marked disinhibition, stereotypy, and aspontaneity without word meaning impairment. Case 2 This patient was a 59-year-old right-handed housewife who had been living with her husband and had an unremarkable medical history and family history. Prior to the onset of her illness, she could be described as serious and punctilious. At 57 years of age, she became increasingly reluctant to do the housework (cleaning, cooking, laundry). She exhibited stereotypy (daily repetitive walking, eating the same foods) 1 year after the onset of her symtoms. At 59 years of age, her husband took her to a local psychiatrist and she was referred to the Higher Brain Function Clinic for a more detailed examination of dementia. At the initial visit, she complained that her husband forcibly brought her to the hospital without cause, reflecting a lack of insight into her condition. Clinical evaluation showed no neurologically abnormal findings. The patient could recall three objects and had no apparent episodic memory impairment. She could calculate, draw a cube, and imitate finger movements. Her visuospatial and cognitive functions were unimpaired. Although the patient did have trouble finding the right words, she could answer if given a cue (e.g. the initial sound ‘kai’ for the term

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R ‘kaichudentou’ [flashlight]). She also understood word meanings. She could identify objects. The patient’s daily life was strictly scheduled and highly regimented. She roamed for several hours over the same route. Her CDR grade was 0.5. Head magnetic resonance imaging (MRI) revealed marked left anterior temporal lobe atrophy with very mild frontal lobe atrophy (Fig. 2). The patient was diagnosed as FTD because she showed marked disinhibition, stereotypy, and aspontaneity without word meaning impairment.

DISCUSSION Of the different types of dementia, FTD is a troublesome disease because there are several variations of symptoms. Compared with Alzheimer’s disease (AD), which evokes amnestic and cognitive symptoms at an early stage, FTD causes disinhibition, aspontaneity, stereotypy, and behavioral changes. Early onset is relatively frequent in FTD and it causes more devastating effects in social life.8 Early and accurate diagnosis is required and it appears that, in the future, amyloid-imaging technology will prove to be a most promising and reliable method with which to differentiate FTD from AD;9 however, in the treatment of FTD, only symptomatic medicines are used, such as selective serotonine reuptake inhibitors10 or Yokukansan.11 The aim of the present paper is to provide a better understanding of the clinical diagnosis of FTD using current techniques and based on clinical observations rather than pathological examinations. In general, neuropsychiatric symptoms, such as disinhibition and aspontaneity, can be attributed to lesions in the frontal lobe, whereas semantic symptoms, such as linguistic or cognitive deficits, can be attributed to lesions in the temporal lobe.7,12 When both the frontal and temporal lobes are involved, stereotypy and abnormal eating behaviors are prominent.13,14 Our recent report suggests that isolated language impairment is the initial symptom in SD and that, 206

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Figure 2 Head magnetic resonance imaging of Case 2 revealed marked left anterior temporal lobe atrophy with very mild frontal lobe atrophy.

subsequently, there is a gradual development of behavioral changes in relation to frontal lobe involvement.15 In contrast, changes in social behavior, affection, and daily activities are the initial symptoms in FTD, with no Gogi aphasia observed even when the left temporal lobe is affected.15 The present report describes two cases of temporal-dominant atrophy who showed profound behavioral abnormalities without word meaning impairment, indicating that there is no exclusive relationship between the syndrome and atrophy.6 Even though neuroimaging also showed atrophy of the hippocampus and amygdala, because the two patients described herein presented with stereotypic and abnormal eating behaviors,13,14, 16–18 but did not exhibit episodic memory or visuospatial impairment,1,15 we were able to clinically differentiate FTD from AD.

ACKNOWLEDGMENT The authors thank the late Professor H. Tanabe (Department of Neuropsychiatry, Neuroscience, Ehime University Graduate School of Medicine, Ehime, Japan) for his encouragement and helpful suggestions on our research.

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