Brain and nerve = Shinkei kenkyū no shinpo
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Amyotrophic lateral sclerosis (ALS) is the most rapidly progressive motor neuron disease (MND) in adults, characterized by the selective death of motor neurons in the motor cortex, brainstem, and spinal cord. Riluzole and edaravone are the only approved drugs available in Japan to date. Approximately 10% of ALS cases are familial in rature, defined as the existence of disease-causing mutation. ⋯ This review article describes the clinical characteristics of familial ALS based on each disease-causing mutation. The pathomechanism of ALS including proteostasis, RNA metabolism, and axonal pathology are discussed in detail. We also reviewed the status of development of therapeutic strategies for familial ALS based on analysis of animal models and induced pluripotent stem cells.
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Artificial intelligence and brain science have kept a swinging relationship with opposing views: "Artificial realization of intelligence should be free from biological constraints" and "We should reverse-engineer the best existing implementation of intelligence." In this article, we first review today's achievements of artificial intelligence and its impacts on brain and life sciences. We then discuss how progresses in brain science can contribute to future developments in artificial intelligence.
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Half a century ago, cerebellar learning models based on a simple perceptron were proposed independently by Marr and Albus. Soon, these models were combined with Ito's flocculus hypothesis that the cerebellar flocculus controls the vestibulo-ocular reflex through teacher signal-dependent learning, and consequently integrated into the so-called Marr-Albus-Ito cerebellar learning hypothesis. Ten years later, Ito found the synaptic plasticity of long-term depression at cerebellar Purkinje cell synapses, which underlies cerebellar learning. ⋯ Artificial intelligence (AI) based on the neural network models originating from a simple perceptron, has now developed to deep learning. As the LSM model of the cerebellum is the counterpart of deep learning in the brain, the cerebellum is considered to be the origin of current AI. Finally, we discuss the impact of the evolution of AI on future clinical cerebellar neurology.
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A 52-year-old woman taking a Chinese herbal medicine for 10 months was admitted to our hospital for recurrent severe headaches, nausea and vomiting. Brain magnetic resonance imaging revealed convexity subarachnoid hemorrhage in the left occipital and parietal lobes. Brain magnetic resonance angiography (MRA) showed multifocal segmental stenosis of cerebral arteries. ⋯ Follow-up MRA at 31 days after the onset showed complete disappearance of multifocal stenosis of cerebral arteries, confirming the diagnosis of reversible cerebral vasoconstriction syndrome (RCVS). It was suggested that licorice and evodia fruit, which were components of a Chinese herbal medicine named tokishigyakukagoshuyushokyoto were the precipitating factors of vasoconstriction. It is important for physicians to recognize that herbal supplements could be one of the causes of RCVS. (Received January 22, 2019; Accepted April 3, 2019; Published July 1, 2019).
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The complement was named after "a complement", a protein molecule that supports an antibody. It was considered previously that the complement mainly participates in protecting against microbial infections. But later, as research on biological functions in complement activation advanced drastically, it was elucidated that the complement could be involved in the onset of various diseases. ⋯ In Japan, ECZ was approved for PNH and atypical hemolytic uremic syndrome (aHUS) in 2010 and 2013, respectively. The success of ECZ created an opportunity for drug companies to develop new therapeutics targeting the complement system; development of complement therapeutics is now a major venture of pharmaceutical companies worldwide. Here, I will provide an outline of the approved complement therapeutics and those that are in development and clinical trial phase currently.