Preferred Label : Shy-Drager syndrome;
ICD-11 definition : Shy-Drager is a form of multiple system atrophy. Multiple system atrophy (MSA) is
a sporadic, adult onset (mean age of onset 55-58 years) progressive (mean disease
duration 7-9 years) neurodegenerative disease characterized by a variable combination
of autonomic failure, parkinsonism and cerebellar signs. Neuropathological examination
shows striatonigral, oliovopontocerebellar and autonomic nuclei neuronal loss, gliosis,
myelin pallor and axonal degeneration. There are ?-synuclein positive glial cytoplasmic
inclusions. [1] Gilman, S. et al. (2008) Second consensus statement on the diagnosis
of multiple system atrophy. Neurology 71, 670–676;
ICD-11 inclusion : neurogenic orthostatic hypotension;
Origin ID : 859030797;
Automatic exact mappings (from CISMeF team)
- Currated CISMeF NLP mapping
- Validated automatic mappings to NTBT
Shy-Drager is a form of multiple system atrophy. Multiple system atrophy (MSA) is
a sporadic, adult onset (mean age of onset 55-58 years) progressive (mean disease
duration 7-9 years) neurodegenerative disease characterized by a variable combination
of autonomic failure, parkinsonism and cerebellar signs. Neuropathological examination
shows striatonigral, oliovopontocerebellar and autonomic nuclei neuronal loss, gliosis,
myelin pallor and axonal degeneration. There are ?-synuclein positive glial cytoplasmic
inclusions. [1] Gilman, S. et al. (2008) Second consensus statement on the diagnosis
of multiple system atrophy. Neurology 71, 670–676
