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• Vol.54 No.12
• CLINICA NEUROL, 54: 963−965, 2014
• Vol.54 No.12 contents

Progress of the Year 2014

Clinical picture and diagnostic criteria

Hirohisa Watanabe, M.D.¹⁾, Mari Yoshida, M.D.²⁾ and Gen Sobue, M.D.³⁾

¹⁾Brain and Mind Research Center, Nagoya Univeristy
²⁾Department of Neuropathology, Institute for Medical Science of Aging, Aichi Medical University
³⁾Department of Neurology, Nagoya University Graduate School of Medicine

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".
Full Text of this Article in Japanese PDF (255K)

(CLINICA NEUROL, 54: 963−965, 2014)
key words: multiple system atrophy, early diagnostic criteria, sudden death, biomarker, natural history

(Received: 22-May-14)