TY - JOUR
T1 - Clinical picture and diagnostic criteria
AU - Watanabe, Hirohisa
AU - Yoshida, Mari
AU - Sobue, Gen
N1 - Copyright:
Copyright 2017 Elsevier B.V., All rights reserved.
PY - 2014
Y1 - 2014
N2 - In 2013, European MSA Study Group demonstrated the prospective natural history of MSA. This study was greately useful for better patients' management and future development of disease-modifying therapy. Although the diagnosis of early symptomatic stage is also an important factor for successful outcome of disease-modifying theraphy, current diagnostic criteria for MSA, which has focused on the combination of motor and autonomic manifestations cannot diagnose MSA patients showing isolated autonomic failure in the early course of illness. We presented four premotor MSA patients who had sudden death prior to fulfilling the diagnostic criteria. 4 cases had very mild OPC and SN pathology, but significant autonomic system involvement including the intermediolateral nucleus, Onuf's nucleus, and medullary autonomic nucleus. Contrary, Petrovic et al reported four pathologically proven MSA patients with disease duration of 15 years or more. All patients presented isolated parkinsonism for a long time and mean duration from onset to dysautonomia was 9 years. Novel diagnostic biomarkers, which have a potential for evaluation of the component of glial cytoplasmic inclusion such as alpha-synuclein radiotracer and serum and CSF alpha-synuclein levels may be a key way to support the diagnosis of patients at the stage of "mono system atrophy".
AB - In 2013, European MSA Study Group demonstrated the prospective natural history of MSA. This study was greately useful for better patients' management and future development of disease-modifying therapy. Although the diagnosis of early symptomatic stage is also an important factor for successful outcome of disease-modifying theraphy, current diagnostic criteria for MSA, which has focused on the combination of motor and autonomic manifestations cannot diagnose MSA patients showing isolated autonomic failure in the early course of illness. We presented four premotor MSA patients who had sudden death prior to fulfilling the diagnostic criteria. 4 cases had very mild OPC and SN pathology, but significant autonomic system involvement including the intermediolateral nucleus, Onuf's nucleus, and medullary autonomic nucleus. Contrary, Petrovic et al reported four pathologically proven MSA patients with disease duration of 15 years or more. All patients presented isolated parkinsonism for a long time and mean duration from onset to dysautonomia was 9 years. Novel diagnostic biomarkers, which have a potential for evaluation of the component of glial cytoplasmic inclusion such as alpha-synuclein radiotracer and serum and CSF alpha-synuclein levels may be a key way to support the diagnosis of patients at the stage of "mono system atrophy".
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U2 - 10.5692/clinicalneurol.54.963
DO - 10.5692/clinicalneurol.54.963
M3 - Article
C2 - 25672682
AN - SCOPUS:84944448975
VL - 54
SP - 963
EP - 965
JO - Clinical Neurology
JF - Clinical Neurology
SN - 0009-918X
IS - 12
ER -