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- W174619081 abstract "Barbeau (1972) first described “pure” akinesia without rigidity and tremor responsive to L-dopa therapy (akinesia due to striatal dopamine deficiency). Since 1974, Narabayashi and the present author described cases with pure akinesia unresponsive to L-dopa treatment as a new condition. This condition exhibits only the freezing symptom, which is a breakdown of repetitive voluntary movements emerging through festination or suddenly, e.g., freezing of gait, micrographia and inaudible speech. Kinesie paradoxale is always accompanied by this type of akinesia. The author suggested that the main pathological structure of the condition is different from the nigrostriatal dopaminergic system and that the condition is different from Parkinson’s disease. L-threo-DOPS, a synthetic norepinephrine (NE) precursor, had a mild-to-moderate effect on some cases with freezing, and the NE hypothesis for freezing was proposed. Up to the present, more than 20 cases with this condition have been known in our clinic. All cases were sporadic and slowly progressive, and some had been followed for as long as ten years, still without rigidity and tremor. Slight muscular hypotonia was observed in the extremities. Slowly progressive ophthalmoplegia appeared later in several cases in which progressive supranuclear palsy (PSP) was strongly suggested despite no axial dystonia. Autopsy cases associated with this condition have been reported in Japan and pathologically revealed PSP or pallido-nigro-luysial atrophy. The nosological position and responsible lesion sites of this condition are discussed." @default.
- W174619081 created "2016-06-24" @default.
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- W174619081 date "1991-01-01" @default.
- W174619081 modified "2023-09-25" @default.
- W174619081 title "Dopa-unresponsive pure akinesia or freezing" @default.
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- W174619081 doi "https://doi.org/10.1007/978-3-7091-9146-0_16" @default.
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