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Like all neurodegenerative diseases, multiple system atrophy (MSA) has a prodromal stage, where some clinical symptoms or signs of the disease are evident, but the threshold for clinical diagnosis
has not been reached .
The ability to directly measure this stage is very limited, mainly because few methods can reliably detect precursor MSA
diagnosis
Most of what is known about prodromal MSA comes from retrospective recall or chart review of clinical symptoms in patients with clinical MSA
.
The main symptoms of the prodromal state include autonomic failure and rapid eye movement sleep behavior disorder (RBD)
The occurrence of RBD in MSA has been retrospectively investigated; approximately 30-40% of MSA patients retrospectively recognize symptoms of RBD prior to onset, with most pointing to RBD as the initial symptom (note that more people may have RBD but didn't realize it)
.
Studies have shown that the presence of RBD predicts a poor prognosis in MSA, including earlier death
.
However, studies that prospectively track iRBD in MSA patients are very limited
From this, they describe the evolution of clinical and biomarker profiles in these patients, compare them with those who develop PD and DLB, and explore the impact on the development of potential precursor MSA criteria
.
The assessment of iRBD patients is part of a comprehensive protocol that is repeated annually
.
The protocol includes assessment of motor, sleep, psychiatric and autonomic symptoms, supplemented by motor examinations, quantitative motor testing, neuropsychological examinations, positive barometric measurements, and tests of smell and color vision
Of the 67 phenotypic switchers, 4 developed MSA-P and 63 developed Lewy body disease
.
Two additional MSA-C patients had developed cerebellar symptoms at baseline
Compared with patients with Lewy body disease, MSA-P patients were younger, had less severe tonic REM sleep disorder, had more symptoms of insomnia, and had a better sense of smell
.
.
Clinically apparent autonomic dysfunction is not static during the prodromal phase and tends to develop near or after the transition of motor imagery
.
Among autonomic symptoms, genitourinary dysfunction was the first in all cases
The significance of this study is the finding that prodromal MSA progresses rapidly, often without substantial autonomic dysfunction, and maintains normal olfactory and cognitive abilities throughout the prodromal course
.
Original source:
Postuma RB, Pelletier A, Gagnon JF, Montplaisir J.
Evolution of Prodromal Multiple System Atrophy from REM Sleep Behavior Disorder: A Descriptive Study.
Evolution of Prodromal Multiple System Atrophy from REM Sleep Behavior Disorder: A Descriptive Study.
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