The Shy-Drager syndrome. What did Shy and Drager really describe?
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Biomedical subjects
Publications and source records attributed to G Wenning.
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Molecular genetic studies of the cytochrome P450 system enzyme CYP2D6, which hydroxylates debrisoquine, have indicated an excess of mutant alleles in large series of patients with Parkinson's disease (PD) when compared with controls. We have investigated CYP2D6 polymorphism in 91 patients with multiple system atrophy (MSA) in order to determine if this finding is specific to PD or if there is similar evidence of genetic susceptibility to neurotoxicity in MSA. The distribution of CYP2D6 alleles was not significantly different between MSA patients and controls, and there were fewer poor metabolisers in the MSA group than in the control group.
The past year has seen advances in delineating the clinical features, natural history and imaging characteristics of multiple system atrophy. The initiating pathogenetic mechanisms remain unknown. However, any aetiological or pathophysiological hypothesis must consider not only neuronal loss and gliosis but also the recently described characteristic oligodendrolial cytoplasmic inclusions.
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After non-invasive stimulation of the motor cortex by brief electromagnetic pulses evoked potentials were biolaterally recorded in 29 healthy individuals. Normal values for the following target muscles were calculated: abductor pollicis brevis (APB), biceps brachii (BIC), tibialis anterior (TIB) and extensor digitorum brevis (EDB). In a subgroup (N = 12) results for APB and EDB were compared with those obtained by electrical stimulation techniques.
Most doctors have vague and imprecise notions of what is meant by the terms olivopontocerebellar atrophy, Shy-Drager syndrome, striatonigral degeneration, multiple system atrophy and multisystem degenerations. This article attempts to tame this nosological jungle.