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강수진,이병화,김은주,박기정,나덕렬 대한치매학회 2004 Dementia and Neurocognitive Disorders Vol.3 No.2
Background: Frontotemporal dementia (FTD) usually presents with behavioral and psycho-logical symptoms of dementia (BPSD) rather than cognitive deficits. Thus, understanding of BPSD associated with FTD is important not only from the management perspective but also from the diagnostic viewpoint. However, few studies have investigated BPSD in patients with FTD in Korea. The aim of the current study is to describe in detail BPSD associated with FTD. Methods: Firstly, a BPSD list was designed based on the symptoms reported in previous studies of FTD, items from the Neuropsychiatric Inventory (NPI), and the review of medical records of FTD patients from our clinic including FTD patients not selected in this study. Then an interviewer asked caregivers whether the BPSDs listed had been present from onset of disease to the time of interview. Subjects who participated in the interview consisted of 23 patients (9 men and 14 women) with a mean age of 65.6 (range, 41-75) years who met the FTD criteria proposed by the Lund and Manchester group. The BPSD list was completed by face-to-face interviews or by telephone interviews with the caregivers. Results: BPSDs as classified by subscales of the NPI in the order of frequency were aberrant motor behavior (95.6%), apathy (95.6%), disinhibition (91.3%), appetite/eating change (91.3%), agitation/aggression (87%), euphoria (65.2%), anxiety (60.9%), irritability/lability (52.1%), delusions (43.5%), night-time behavior (39.1%), depression/dysphoria (34.8%), hallucinations (0%). Other symptoms not listed in the NPI were stereo-typical behaviors as stereotypy of ordering, hoarding, reading signboards, wandering a fixed route, washing, and counting, utilization behaviors, and sundowning. Conclusion: Our detailed description of BPSD associated with Korean FTD patients would help understand symptoms of FTD and develop a Korean version of BPSD questionnaire for FTD in the future studies.
운동신경원성 질환을 동반한 전두측두엽치매의 임상양상과 신경심리소견
박기정,정용,김은주,진주희,강수진,나덕렬 대한치매학회 2003 Dementia and Neurocognitive Disorders Vol.2 No.1
Backgrounds:Frontotemporal dementia (FTD) is rarely associated with motor neuron disease (MND). This comorbidity (FTD-MND), a subtype of FTD, results in progressive dementia and muscle weakness. Among the few available reports of series of patients, however, there have been controversies about the clinical course of FTD-MND. This study, the first report of a series of FTD-MND patients in Korea, investigated demographic and clinical features, clinical course, and neuropsychological findings of nine patients with FTD-MND. Methods:Nine FTD-MND patients (2 men and 7 women with mean age 55.6±7.2 years) were selected among 45 FTD patients who met the FTD criteria proposed by the Lund and Manchester Groups. Their clinical and neuropsychological findings were analyzed retrospectively. Results:Mean age of onset was 54.3±8.0 years. The time interval from onset to death was 27±11.3 months. Presenting symptoms were personality change, hyperphagia, nonfluent speech or motor weakness. In most cases (8/9), dementia preceded the muscle weakness;in only one patient the muscle weakness preceded the cognitive decline. Regarding the muscle weakness, bulbar symptoms occurred earlier than limb weakness in four patients;bulbar and limb weakness occurred simultaneously in another four patients;in the remaining one patient limb weakness preceded bulbar weakness. Neuropsychologic tests showed no specific patterns but diffuse dysfunction in all cognitive domains. Conclusions:Our study suggests that FTD-MND predominantly affects the bulbar muscles rather than the limb muscles in early stage and symptoms related to MND occurs later than those related to FTD. The time from diagnosis to death was 10.6±6.5 months, suggesting that FTD-MND is a rapidly progressive disease. This rapid clinical course may account for our neuropsychological findings that showed general cognitive deficits father than predominant frontal dysfunctions.