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Dopa-unresponsive pure akinesia or freezing

Part of the Key Topics in Brain Research book series (KEYTOPICS)

Summary

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. Kinésie 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.

Keywords

Progressive Supranuclear Palsy Progressive Supranuclear Palsy Clin Neurol Nigrostriatal Dopaminergic System Postural Disorder 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.

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Copyright information

© Springer-Verlag/Wien 1991

Authors and Affiliations

  • H. Imai
    • 1
  1. 1.Department of NeurologyJuntendo University School of MedicineBunk-ku, Tokyo 113Japan

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