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Progression of autonomic dysfunction in isolated REM sleep behavior disorder – The key to understanding pathophysiology of synucleinopathy

      Isolated REM sleep behavior disorder (iRBD) is considered a prodromal stage of Parkinson's disease (PD), dementia with Lewy bodies, and multisystem atrophy. The pathological hallmark of these diseases is the intraneuronal accumulation of misfolded alpha-synuclein, referred to as Lewy bodies, resulting in degeneration of neurons in the substantia nigra and other parts of the brain. The deposition of alpha-synuclein in the substantia nigra and its subsequent degeneration is the cause of parkinsonism, the main motor symptom of PD [
      • Kalia L.V.
      • Lang A.E.
      Parkinson's disease.
      ]. Non-motor symptoms of PD that have been increasingly studied recently include autonomic dysfunction. Autonomic PD symptoms also occur in persons with iRBD, and their spectrum is similar to that of PD. Autonomic dysfunction in iRBD is clinically relevant and represents an important clue to the pathophysiology of PD/iRBD, particularly the spread of synuclein in the human body before the onset of PD symptoms. Despite its scientific importance, information on the progression of autonomic symptoms is limited.
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      Linked Article

      • Imaging progressive peripheral and central dysfunction in isolated REM sleep behaviour disorder after 3 years of follow-up
        Parkinsonism & Related DisordersVol. 101
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          Most patients with isolated rapid eye movement sleep behaviour disorder (iRBD) convert to Parkinson's disease (PD), dementia with Lewy bodies, or multiple system atrophy within 15 years of diagnosis. Furthermore, iRBD patients develop non-motor symptoms similar to those of manifest PD patients and display dysfunction of the sympathetic and parasympathetic nervous system, comparable to that seen in PD. However, progression rates of autonomic dysfunction in iRBD have not been studied with objective measures in detail, which is the aim of this study.
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