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루이소체 치매의 증상과 치료

Clinical Features and Pharmacological Treatment of Dementia with Lewy Bodies

김태희
대한생물정신의학회 (The Korean Society Of Biological Psychiatry)|2016.05|생물정신의학 (Journal of Korean Society of Biological Psychiatry)|제23권 제2호|pp. 41-47

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키워드

루이소체 치매Dementia with Lewy bodies파킨슨병 치매Parkinson’s disease with dementia
주제분류정신과학

초록

루이소체 치매(Dementia with Lewy bodies, 이하 DLB)1) 는 알츠하이머병(Alzheimer’s Disease, 이하 AD)에 이어 두번째로 흔한 퇴행성 뇌질환이다.2) 1921년 파킨슨병 환자에서루이소체(Lewy body, LB)가 처음 발견되고, 1960년에 대뇌 피질의 루이소체(Lewy bodies) 사례가 보고되었다.3) 1989년 알파 시누클레인 면역세포화학 염색 기법(alpha synuclein immunocytochemical staning)으로 피질의 루이소체(Lewy bodies)와 루이소체 신경염(Lewy neurites)을 쉽게 확인할 수있게 되면서 비교적 흔한 것으로 인식되기 시작했다.3) DLB는일반인구의 약 5%, 모든 치매 환자의 약 31%까지 이르는 것으로 보고되고 있다.4) 국내에서는 1% 미만의 매우 낮은 유병률이 보고된 바 있다.5) 하지만 DLB를 놓치고 오진하는 경우가 많기 때문에 실제 DLB의 유병률은 더 높을 것으로 예상된다.6) DLB 진단에 있어 가장 큰 장벽은 DLB를 의심하지 않는 것으로, 환자나 보호자들이 주 호소 증상 외의 증상들을자발적으로 보고하지 않기 때문에 간과하기 쉽고 DLB의 다양한 증상들이 단일 시점에서 한꺼번에 출현하는 것이 아닐뿐더러 각 증상들의 유무를 명확히 구분하는 것이 어렵기 때문이다.7) DLB 환자의 보호자가 흔히 보고하는 증상은 인지기능저하이고 가장 부담스러워하는 증상은 환시나 망상 등의 정신행동증상이다.8) 자발적 파킨슨증, 렘수면행동장애(REM sleep behavior disorder, 이하 RBD), 심한 신경이완 감수성, 낙상혹은 실신, 일시적 의식소실, 자율신경계 이상, 우울증 등 다양한 증상이 나타날 수 있다. 이러한 증상들은 신경과 또는내과를 거치더라도 대부분 정신건강의학과를 찾게 되는 증상이다. 그러나 DLB에 대한 임상의의 인식 부족으로 인해 정신과 의사에게 DLB를 진단받는 사례는 10% 미만인 것으로 조사된 바 있다.8) DLB 환자는 섬망, 일과성 뇌허혈증, 경련, 공황 발작, 전환장애, 신체화 장애, 낙상에 의한 골절, 불안정협심증, 항정신병약물뿐 아니라 부교감신경 억제제나 진경제 복용 후의 파킨슨증 등을 진단받은 병력이 종종 관찰된다. 초기 증상으로 시공간 능력이나 실행 능력의 이상을 보였더라도 이후로 증상없이 수개월간 안정기를 가질 수 있는데, 안정기에 환자를 보는 임상의가 관찰 시점의 상태에만 주목하고 DLB를 의심하지 못한다면 진단은 더욱 늦어질 것이다. 환자나 보호자가 자발적으로 이야기하지 않더라도 다양한 증상의 유무를 확인하고 향후의 변화를 관찰하는 것이 매우 중요하겠다.9) 따라서 임상의는 DLB의 임상적 증상을 숙지할 필요가 있고, 정신행동적 증상과 파킨슨 증상, 자율신경계 증상을 치료할 때 예상되는 각 증상에 대한 상충적 작용을 이해해야할 것이다. 이에 본지에서는 증상과 진단을 정리하고, 약물치료 결과를 소개하고자 한다.

Abstract

Dementia with Lewy bodies (DLB) is the second most common causes of dementia. It can exhibit a variety of clinical symptoms including cognitive decline, cognitive fluctuation, visual hallucinations, parkinsonism, REM sleep behavior disorder, hypersensitivity to neuroleptics and autonomic dysfunctions. Despite more well-known criteria for DLB, there are often misdiagnosis and inappropriate treatment. It gives a lot of clinical burden to the clinician as well as to patients and families. When reducing the misdiagnosis, the burden of all will be reduced. The special concern and solicitation are needed in order not to miss the diagnosis when the cardinal features of DLB may not be volunteered by patients and the caregivers. To control the symptoms, clinicians must find and reduce drugs that can have the negative effects on DLB symptoms. There is limited evidence about specific interventions but available data suggest cholinesterase inhibitors improve the cognitive and behavioral symptoms and menmantine slightly improves the global impression.

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