• 생물정신의학
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• 제23권3호
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• pp.69-79
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• 2016

Frontotemporal dementia (FTD) is a degenerative disease characterized by the selective frontal and temporal lobe atrophy, and progressive deficits in behavior, executive function, or language. The prevalence and incidence of FTD are 15-22/100000 and 2.7-4.1/100000, respectively, in midlife. Hereditary is an important risk factor for FTD. Although there is some controversy regarding the further syndromatic subdivision of the different types of FTD, FTD is clinically classified into behavioral variant of frontotemporal dementia, semantic dementia and progressive nonfluent aphasia. FTD can be misdiagnosed as many psychiatric disorders because of similarity of the prominent behavioral features. Advances in clinical, imaging, and molecular characterization have increased the accuracy of FTD diagnosis, thus developing for the accurate differentiation of these syndromes from psychiatric disorders. We also discuss about therapeutic strategies for symptom management of FTD. Medications such as serotonin reuptake inhibitors, antipsychotics, and other novel treatments have been used in FTD with various rates of success. Further advanced research should be directed at understanding and developing new diagnostic and therapeutic modalities to improve the FTD patients' prognosis and quality of life.

• 대한치매학회지
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• 제17권4호
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• pp.163-173
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• 2018

Background and Purpose: Behavioral variant frontotemporal dementia (bvFTD) is a subtype of frontotemporal dementia, which has clinical symptoms of progressive personality and behavioral changes with deterioration of social cognition and executive functions. The pathology of bvFTD is known to be tauopathy or TDP-43 equally. We analyzed the $^{18}F-THK5351$ positron emission tomography (PET) scans, which were recently developed tau PET, in patients with clinically-diagnosed bvFTD. Methods: Forty-eight participants, including participants with behavioral variant frontotemporal dementia (bvFTD, n=3), Alzheimer's disease (AD, n=21) and normal cognition (NC, n=24) who completed 3T magnetic resonance images, $^{18}F-THK5351$ PET scans, and detailed neuropsychological tests were included in the study. Voxel-wise statistical analysis and region of interest (ROI)-based analyses were performed to evaluate the retention of THK in bvFTD patients. Results: In the voxel-based and ROI-based analyses, patients with bvFTD showed greater THK retention in the prefrontal, medial frontal, orbitofrontal, anterior cingulate, insula, anterior inferior temporal and striatum regions compared to NC participants. Left-right asymmetry was noted in the bvFTD patients. A patient with extrapyramidal symptoms showed much greater THK retention in the brainstem. Conclusions: The distribution of THK retention in the bvFTD patients was mainly in the frontal, insula, anterior temporal, and striatum regions which are known to be the brain regions corresponding to the clinical symptoms of bvFTD. Our study suggests that $^{18}F-THK5351$ PET imaging could be a supportive tool for diagnosis of bvFTD.

• 한국산학기술학회논문지
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• 제18권12호
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• pp.352-358
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• 2017

이마관자엽 치매는 조기 발현형 치매 중 두 번째로 흔한 형태로 행동, 언어, 인지 장애를 보이는 신경퇴행성 질환이다. 이마관자엽 치매에서 운동 기능 이상이 동반되는 경우는 운동 신경원 질환과 파킨슨증, 진행성 핵상 마비 등으로 대표되나, 다른 동반 질환 없이 이마관자엽 치매가 중추신경계의 운동신경영역을 직접 침범하여 나타난 운동 기능 이상은 보고된 바가 없다. 또한, 임상적 치매 집단과 복합부위 통증 증후군 사이의 연관성은 보고된 바가 없다. 저자들은 이마관자엽 치매환자에서 나타난 중추신경계 원인의 하지 근력 저하를 뇌 자기공명영상과 전기진단학적 검사를 통해 진단하였고, 동반된 복합부위 통증증후군을 삼상 골주사 검사를 통해 진단하였으며, 이에 따른 임상적 치료를 시행하였다. 스테로이드를 이용한 복합부위 통증증후군 치료 후에 환자의 통증은 호전되었고, 입원 상태에서 하지 근육에 대한 기능적 전기 자극 치료, 근력 강화 운동 및 보행 훈련을 포함한 포괄적 재활치료를 시행한 후에 저명한 기능적 호전을 보였다. 이마관자엽 치매에서 관찰된 중추신경계 원인의 근력 저하에 대한 재활 치료는 전반적 기능의 향상에 효과적일 것으로 추정된다.

• 사상체질의학회지
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• 제30권3호
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• pp.95-103
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• 2018

Objective The purpose of this study is to investigate the effect of Yanggyuksanhwa-tang on Neurogenic bladder by Frontotemporal dementia in a 65-year-old male patient. Method Patient was treated with Yanggyuksanhwa-tang. Patient's symptom was assessed Total Frequency of urination, Frequency of nocturia, single voided volume. The treatment was executed three times everyday from March, 21th to March 29th of 2018. Patient was observed from March, 20th to March, 30th. Results During 8 days of Yanggyuksanhwa-tang, patient's symptoms were evaluated by Total Frequency of urination, Frequency of nocturia, single voided volume. After the treatment, Total Frequency of urination, Frequency of nocturia decreased. Single voided volume increased. Conclusion The results suggest that Yanggyuksanhwa-tang can be a valuable option in treating Neurogenic bladder by Frontotemporal dementia.

• BMB Reports
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• 제41권12호
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• pp.827-832
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• 2008

Many age-dependent neurodegenerative diseases are associated with the accumulation of abnormally folded proteins within neurons. One of the major proteolytic pathways in the cell is the autophagy pathway, which targets cytoplasmic contents and organelles to the lysosomes for bulk degradation under various physiological and stressful conditions. Although the importance of autophagy in cellular physiology is well appreciated, its precise roles in neurodegeneration remain largely unclear. Recent studies indicate that components of the endosomal sorting complex required for transport (ESCRT) are important in the autophagy pathway. Reduced activity of some ESCRT subunits leads to the accumulation of autophagosomes and failure to clear intracellular protein aggregates. Interestingly, rare mutations in CHMP2B, an ESCRT-III subunit, are associated with frontotemporal dementia linked to chromosome 3 (FTD3). Mutant CHMP2B proteins seem to disrupt the fusion of autophagosomes and lysosomes in cell culture models. These findings suggest a potential mechanism for the pathogenesis of FTD3 and possibly other neurodegenerative diseases as well.

• Nuclear Medicine and Molecular Imaging
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• 제41권2호
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• pp.102-111
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• 2007

Dementia is a major burden for many countries including South Korea, where life expectancy is continuously growing and the proportion of aged people is rapidly growing. Neurodegenerative disorders, such as, Alzheimer disease, dementia with Lewy bodies, frontotemporal dementia, Parkinson disease, progressive supranuclear palsy, corticobasal degeneration, Huntington disease, can cause dementia, and cerebrovascular disease also can cause dementia. Depression or hypothyroidism also can cause cognitive deficits, but they are reversible by management of underlying cause unlike the forementioned dementias. Therefore these are called pseudodementia. We are entering an era of dementia care that will be based upon the identification of potentially modifiable risk factors and early disease markers, and the application of new drugs postpone progression of dementias or target specific proteins that cause dementia. Efficient pharmacologic treatment of dementia needs not only to distinguish underlying causes of dementia but also to be installed as soon as possible. Therefore, differential diagnosis and early diagnosis of dementia are utmost importance. F-18 FDG PET is useful for clarifying dementing diseases and is also useful for early detection of the diseases. Purpose of this article is to review the current value of FDG PET for dementing diseases including differential diagnosis of dementia and prediction of evolving dementia.

• 대한핵의학회지
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• 제37권1호
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• pp.13-23
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• 2003

Neurodegenerative disorders cause a variety of dementia including Alzheimer disease, frontotemporal dementia, dementia with Lewy bodies, corticobasal degeneration, progressive supranuclear palsy, and Huntington's disease. PET scan is useful for early detection and differential diagnosis of these dementing disorders. Also, it provides valuable information about clinico-anatomical correlation, allowing better understanding of function of brain. Here we discuss recent achievements PET studies regarding these dementing disorders. Future progress in PET technology, new tracers, and image analysis will play an important role in further clarifying the disease pathophysiology and brain functions.

• 정신신체의학
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• 제25권1호
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• pp.3-11
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• 2017

연구목적 소위 '행동증상 아형 전두측두엽 치매 표현형모사 증후군(behavioral variant frontotemporal dementia phenocopy syndrome)' 환자들은 일차성 정신장애와 감별이 힘들고, 기존의 치료 약물에 대한 반응도 회의적이어서, 오진의 위험과 법적 문제의 유발 가능성이 높으므로, 이러한 양상을 보이는 환자군에 대한 지속적인 관심과 연구가 필요함을 환기 시키기 위해서 본 연구를 시행하였다. 방법 2000년 부터 2016년 까지 발행된 영문 학술지에 게재된 논문을 인터넷에서 'frontotemporal', 'phenocopy', 'behavioral'과 같은 단어의 조합으로 검색하여 찾은 참고문헌을 정리, 고찰하였다. 또한 저자가 직접 경험한 두 증례를 간략히 기술하였다. 결과 환자의 행동 증상은 가족과 지역사회에 큰 영향을 끼치므로 행동증상 아형 전두측두엽 치매에서 정확한 진단이 중요하다. 그러나, 행동증상 아형 전두측두엽 치매에서 현재까지 질병 수정 치료법은 개발되지 않았으며, 현재의 약물학적 치료는 특수한 증상에 도움이 될 뿐이고, 적절한 정신의학적 치료에도 불구하고 점진적인 퇴행이 진행된다. 상당수의 '행동증상 아형 전두측두엽 치매 의심(possible bvFTD)' 환자에서 임상적으로 '행동증상 아형 전두측두엽 치매 가능(probable bvFTD)'으로 진행되지 않으며, 인지기능이나 사회적 기능이 저하되지 않고, 활동 기능의 저하를 보이지 않으며, 생존기간이 조금 더 길고, 수년에 걸쳐서 정상적인 뇌영상화 검사 소견을 보인다. 결론 환전두측두엽 치매 및 일차성 정신장애 환자에서 보이는 전형적인 임상적 양상이나 경과, 뇌영상화 검사를 포함한 진단적 평가와는 다른 비전형적인 소견을 보이는 환자군들은 생각보다 많으며, 정신의학과 의사들이 이에 대한 의학적 지식과 판별 능력이 발전되면, 그 발견의 비율은 훨씬 더 높아질 것이다. 그러나, 현재로서는 이러한 비전형적인 환자군의 정체에 대해서는 이견이 많으며, 향후 적극적인 연구가 행해져야할 분야이다.

• 생물정신의학
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• 제10권2호
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• pp.97-106
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• 2003

Criticisms about amyloid cascade hypothesis of Alzheimer's disease(AD) are based on the findings, first, that the degree of dementia does not correlate with the number of plaques, and second, that the neurofibrillary tangle formation seems to predate plaque formation. In addition, neurofibrillary tangle counts correlate well with the degree of cognitive impairment. These findings suggest the independent importance of tau abnormality in AD research which is involved in the neurofibrillary tangle formation. Recently, tau pathology without amyloid deposits and mutations in tau protein gene were reported to be the major pathogenic mechanism in Pick's disease, progressive supranuclear palsy, corticobasal degeneration and FTDP-17(frontotemporal dementia and parkinsonism linked with chromosome 17). These data suggest that understanding the causes and consequences of tau dysfunction might give new clinical and therapeutic solutions to many known tauopathies.

• Annals of Clinical Neurophysiology
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• 제19권2호
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• pp.101-112
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• 2017

Detection of underling proteinopathies is becoming increasingly important across neurodegenerative conditions due to upcoming disease intervention trials. In this review, we explored how temporal lobe changes in amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD) can potentially predict underlying TDP-43 pathology subtypes in FTD. To date, emphasis has been given to frontal lobe changes in the study of the cognitive and behavioural impairments in both syndromes but an increasing number of pathological, imaging and neuropsychological studies suggest how temporal lobe changes could critically affect the cognition and behaviour of these conditions. In this current article, we reviewed pathological, imaging as well as clinical/neuropsychological findings of temporal involvement in the ALS-FTD continuum, how they relate to temporal lobe changes and the underlying TDP-43 pathology in FTD. Findings across studies show that TDP-43 pathology occurs and coincides in many structures in ALS and FTD, but especially in the temporal lobes. In particular, anterior and medial temporal lobes atrophy is consistently found in ALS and FTD. In addition, memory and language impairment as well as emotional and Theory of Mind processing deficits that are characteristics of the two diseases are highly correlated to temporal lobe dysfunction. We conclude by showing that temporal lobe changes due to TDP-43 type B might be particular predictive of TDP-43 type B pathology in behavioural variant FTD, which clearly needs to be investigated further in the future.