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Multiple system atrophy (MSA) is a sporadic, adult-onset disease characterized by progressive degeneration of nervous systems including cerebellar, pyramidal, extrapyramidal, and autonomic system. Although a few recent studies reported that cognitive impairments could occur in patients with MSA, prominent dementia with progressive decline is not a typical clinical manifestation of MSA. In particular, dementia with MSA-cerebellar type is very rare. We have experienced a patient with 2-year history of severe cognitive impairment, who was finally diagnosed as MSA-cerebellar type.
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Parkinson’s disease (PD) is a chronic neurodegenerative disorder with multifactorial etiology. In the past decade, the genetic causes of monogenic forms of familial PD have been defined. However, the etiology and pathogenesis of the majority of sporadic PD cases that occur in outbred populations have yet to be clarified. The recent development of resources such as the International HapMap Project and technological advances in high-throughput genotyping have provided new basis for genetic association studies of common complex diseases, including PD. A new generation of genome-wide association studies will soon offer a potentially powerful approach for mapping causal genes and will likely change treatment and alter our perception of the genetic determinants of PD. However, the execution and analysis of such studies will require great care.
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To investigate the relationship between presenting clinical manifestations and imaging features of multisystem neuronal dysfunction in MSA patients, using 18F-fluorodeoxyglucose positron emission tomography (18F-FDG PET).
We studied 50 consecutive MSA patients with characteristic brain MRI findings of MSA, including 34 patients with early MSA-parkinsonian (MSA-P) and 16 with early MSA-cerebellar (MSA-C). The cerebral glucose metabolism of all MSA patients was evaluated in comparison with 25 age-matched controls. 18F-FDG PET results were assessed by the Statistic Parametric Mapping (SPM) analysis and the regions of interest (ROI) method.
The mean time from disease onset to 18F-FDG PET was 25.9±13.0 months in 34 MSA-P patients and 20.1±11.1 months in 16 MSA-C patients. Glucose metabolism of the putamen showed a greater decrease in possible MSA-P than in probable MSA-P (
Our results may suggest that the early neuropathological pattern of possible MSA with a predilection for the striatonigral or olivopontocerebellar system differs from that of probable MSA, which has prominent involvement of the autonomic nervous system in addition to the striatonigral or olivopontocerebellar system.
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Various nonpharmacologic managements are important fundamental elements in the treatment of Parkinson’s disease (PD). We aimed to investigate the role of telephone counseling in managing PD patients.
From November 2006 to January 2007, we studied 243 PD patients at outpatient clinic of Asan Medical Center. Detailed telephone counseling was provided using a list structured questionnaires.
There were 73 men and 170 women with an age range of 17 to 85 years (mean age, 64.9 years). Mean age at onset was 59.5 years (range, 14–82 years) and mean disease duration was 5.6 years (range, 0.3–25 years). The contents of telephone counseling included adverse effects of anti-Parkinsonian medications (24.4%), aggravation of motor symptoms (18.7%), problems due to comorbidities (17.8%), how to take medicine (13.6%), activities of daily living (diet, bowel, sleeping and safety) (12.6%), complementary or alternative medicines (3.9%) and knowledge about PD (3.0%). Persons who responded to use the telephone counseling included patients (37.9%), offspring (36.2%), spouses (17.7%) and other relatives (7.4%). Persons who received the telephone counseling were determined by level of education, sex, cohabitation and Hoehn-Yahr stage. Contents of telephone counseling varied significantly with Hoehn-Yahr stage and persons who used the telephone counseling.
Our results suggest that support system with telephone counseling may provide beneficial therapeutic intervention in PD patients, especially for those with advanced PD. The most cost-effective method for telephone support needs to be studied.
