PubMed · 5300663
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C N Shealy. 1968. Stimulation vs. ABLATION.. https://doi.org/10.1111/j.1526-4610.1968.hed0801033.x
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Because multiple molecular signal transduction pathways regulate cerebellar long-term depression (LTD), which is thought to be a possible molecular and cellular basis of cerebellar learning, the systematic relationship between cerebellar LTD and the currently known signal transduction pathways remains obscure. To address this issue, we built a new diagram of signal transduction pathways and developed a computational model of kinetic simulation for the phosphorylation of AMPA receptors, known as a key step for expressing cerebellar LTD. The phosphorylation of AMPA receptors in this model consists of an initial phase and an intermediate phase. We show that the initial phase is mediated by the activation of linear cascades of protein kinase C (PKC), whereas the intermediate phase is mediated by a mitogen-activated protein (MAP) kinase-dependent positive feedback loop pathway that is responsible for the transition from the transient phosphorylation of the AMPA receptors to the stable phosphorylation of the AMPA receptors. These phases are dually regulated by the PKC and protein phosphatase pathways. Both phases also require nitric oxide (NO), although NO per se does not show any ability to induce LTD; this is consistent with a permissive role as reported experimentally (Lev-Ram et al., 1997). Therefore, the kinetic simulation is a powerful tool for understanding and exploring the behaviors of complex signal transduction pathways involved in cerebellar LTD.
We describe the unusual case of a 51-year-old woman with spinocerebellar ataxia type 1 (SCA1) who showed choreiform movements in addition to cerebellar ataxia. To date, extrapyramidal signs including involuntary movements have been rarely reported in SCA1. Surface electromyogram in our patient revealed grouped discharges whose duration was longer than that of chorea observed in HD, indicating that the involuntary movements represented choreoathetosis rather than pure chorea. These choreiform movements have not been seen in non-hereditary spinocerebellar ataxia. Therefore, if "sporadic" cases of cerebellar ataxia show such movements, the possibility of genetic origin of the ataxia is high and a surveillance of various forms of hereditary spinocerebellar ataxia including SCA1 is required.
OBJECTIVE: To determine the normal appearance and development of the fetal cerebellar vermis using mid-sagittal planes obtained by transvaginal sonography. SUBJECTS AND METHODS: A total of 101 fetuses were evaluated by transvaginal sonography between 21 and 39 weeks of gestation. The mid-sagittal antero-posterior and cranio-caudal vermian diameters, circumference and surface area were measured and calculated. Nomograms were produced. RESULTS: The vermis was observed in 96% of the cases and all the measurements were performed in 92%. The vermis grows in a linear fashion throughout pregnancy. The growth pattern correlates well with gestational age, biparietal diameter, head circumference and transverse cerebellar diameter. CONCLUSION: Transvaginal sonography is a valuable tool in the study of the fetal vermis during the second half of pregnancy. Knowledge of its normal appearance may help identify developmental anomalies and enable accurate prenatal counseling.