教育演講6:神經退化疾病的診斷與治療
The Diagnosis and Management of Neurodegenerative Disorders

程 序 表

E6-3
Multiple system atrophy and dementia with lewy body: The diagnosis and management
林靜嫻
臺大醫院神經部

  Parkinsonism syndrome is a cluster of neurodegenerative diseases characterized by Parkinson’s disease (PD)-like symptoms combined with other system degeneration, such as autonomic system or cerebellar system etc. There are a variety of causes of Parkinsonism syndrome, including multiple system atrophy (MSA), progressive supranuclear palsy (PSP), corticobasal syndrome (CBS), and dementia with lewy bodies (DLB). These Parkinsonism syndromes are characterized by a rapidly evolving parkinsonism feature but has a poor or transient response to dopaminergic therapy compared to patients with PD. With variable clinical presentations of patients with parkinsonism syndromes, the pathology hallmarks and pathogenic mechanisms were heterogenous in individual disease group. Compared to the main neuropathological hallmark of PD, MSA and DLB is a-synuclein accumulations in neurons or oligodendrocytes (termed as synucleinopathy), the main pathological protein deposited in the neurons of patients with PSP and CBS (termed tauopathy).
  This talk will focus on MSA and DLB. MSA is characterized by progressive autonomic failure, parkinsonian features, and cerebellar and pyramidal features in various combinations. It is classified as the parkinsonian subtype if parkinsonism is the predominant feature and as the cerebellar subtype if cerebellar features predominate. In contrast to the pathology of PD is neuronal accumulations of a-synuclein, the pathology findings of patients with MSA are oligodendroglial cytoplasmic inclusions (also called Papp–Lantos bodies) containing fibrillar forms of a-synuclein. On the other hand, DLB and PD shared the same main pathology findings that both diseases have a-synuclein accumulations in neurons. However, patients with DLB have the main features of rapid progressive dementia, fluctuated cognition and alertness, rapid eye movement sleep behavior disorder and mild parkinsonism feature. Early differential diagnosis of MSA and DLB from PD is important for the worse prognosis and more crucial care should be given to patients with MSA and DLB. We summarize the current updated consensus diagnostic criteria for patients with MSA and DLB, outline some of the challenges for the future, which will clarify the pathogenesis and lead to future disease-modifying treatments.