Brain and Nerve最新文献

Santiago Ramón y Cajal (1852-1934) is known as a giant who laid the foundation of today's neuroscience and neuroanatomy. Born in Spain, a region that was recognized as a frontier in academic research, he continued to produce numerous new findings throughout his life. It is impossible to cover all his achievements in this brief article. However, it is truly remarkable to recognize that many of the cells and neural pathways we now regard as common knowledge, without deep consideration, were elucidated in Cajal's work. This brief article is a tribute to Cajal and Dr. Hajime Mannen, a master of neuroanatomy in Japan, who was captivated by his work.

This paper explores research on Marchiafava-Bignami Disease (MBD) by the Roman neurologist Ettore Marchiafava. It provides an overview of recent trends in MBD research, primarily focusing on the author's own work, and briefly highlights Marchiafava's role as a microbiologist and clinician, emphasizing his contributions as a notable Roman researcher. MBD is marked by symmetrical demyelinating lesions of the corpus callosum that occur in heavy alcohol consumers. Since 1898, diagnosis has been limited to postmortem findings. By 1985, 149 cases had been reported. Since the 1980s, however, the advent of magnetic resonance imaging (MRI) has enabled ante-mortem diagnosis. Clinical symptoms include astasia-abasia, Gegenhalten, cluttering dysarthria, and impaired consciousness during the acute phase, followed by interhemispheric disconnection symptoms in the chronic phase. Longitudinal X-ray CT and MRI studies have revealed findings suggestive of transient hemorrhage during the subacute phase, which have also been confirmed pathologically. However, the etiology of MBD remains incompletely understood. Ongoing research is needed to elucidate its pathogenesis, and the development of effective treatments is eagerly anticipated. Marchiafava was also a notable researcher of malaria, as well as a microbiologist and clinician with a broad perspective who loved Rome.

This article highlights the little-known theoretical foundations and background of Luria's theory of aphasia and the various aphasia types. Certain aphasia types, such as dynamic aphasia and semantic aphasia, are unique. Dynamic aphasia is based on the linguistic theory of "linear schema," while semantic aphasia is based on the theory of higher neural activity in the semantic field, known as the "equal phase." I would like to emphasize that Luria's theory of aphasia draws on the Russian psychology of Vygotsky and others, as well as Pavlov's theory of higher neural activity.

Jean-Martin Charcot (1825-1893) gradually shifted his medical interest from internal medicine to neurology. Furthermore, he established neurodiagnostic methods that emphasized observation, such as in tabes dorsalis, and summarized core neurological diseases such as amyotrophic lateral sclerosis, Parkinson's disease, and multiple sclerosis in a clinical-anatomical/pathological manner. For his contributions, he is known as the father of neurology. He then worked to elucidate hysteria. Sir Arthur Conan Doyle (1859-1930), born more than 30 years later, opened an ophthalmology clinic after earning his doctorate in research on tabes dorsalis. However, he then changed direction to writing books, such as Sherlock Holmes stories, and created full-fledged detective studies. Later, he shifted his focus to spiritualism, with an interest in Charcot's hypnotism. Although their careers are similar, there is no direct connection between Charcot's neurological studies and Conan Doyle's detective stories. However, neurodiagnostic and detective studies emerged in the second half of the 19th century at the same time, although they shared a commonality in content, in that they both emphasized observation and deduction without preconceptions. This contemporaneity was inevitable, as the background to this was the emergence of a middle class and urbanization in the era of war and revolution under capitalism after the Industrial Revolution.

Agitation is a behavioral and psychological symptom of dementia (BPSD); however, until recently, there has been no consensus-based definition. When initiating therapeutic interventions, the type of dementia that is the cause of the BPSD must be considered and treatment must be tailored accordingly. As with other types of BPSD, non-pharmacological interventions are considered the first-line treatment for agitation, unless the symptoms present an exceptional level of urgency. Person-centered care is effective as a non-pharmacological intervention for agitation. Other examples include music therapy, animal-assisted therapy, and aromatherapy; however, these are not very effective for agitation. Useful information on non-pharmacological interventions for agitation can be obtained from the website "Ninchisho Chienowa-net," which is a web-based system that collects information on the coping strategies of caregivers for BPSD. If non-pharmacological interventions are ineffective or the symptoms are urgent, pharmacological treatment should be considered. Brexpiprazole has been approved as effective for agitation in Alzheimer's disease. For the treatment of agitation, medications other than brexpiprazole are frequently used in clinical settings and may offer some degree of efficacy.

Charcot's observations of hysteria were reproduced in a textbook written by Paul Richer (1881). These consisted of hysterical seizures (grande hystérie), interictal pseudo-neurological signs, and grand hypnotism. Hysterical seizures were reported to start during the epileptoid period, followed by the contortion-grand movement period, attitude passionnelle period, and finally ended in the delusional period. We compared Charcot's observations with those derived from psychogenic non-epileptic seizures. In conclusion, Charcot may have not only diagnosed patients with hysteria but also danced with them, producing once-in-a-lifetime symptoms as a result of the chemistry between him and his patients.

In 1868, Jean-Martin Charcot systematically described the clinical and pathological findings of multiple sclerosis (MS), establishing it as a distinct disease entity. Additionally, he noted the importance of the triad of tremors (intentional tremors), nystagmus, and dysarthria (scanning speech). While he did not propose them as diagnostic criteria, he recognized their value in differentiating MS from other disorders affecting the central nervous system.

This paper outlines the contributions of Jean-Martin Charcot (1825-1893) to the study of spinal cord diseases. Charcot pioneered the anatomoclinical method, which correlated clinical symptoms with pathological findings through detailed observations and autopsies. He elucidated the dual structure of the motor system-gray and white matter-and established the clinical and pathological framework of amyotrophic lateral sclerosis, distinguishing it from other disorders. He also contributed to the understanding of tabes dorsalis by linking sensory ataxia to lesions of the dorsal columns and roots, and identified neurogenic joint disease (Charcot joint). Furthermore, Charcot described compressive myelopathy based solely on clinical signs and postmortem findings, highlighting the importance of symptom distribution, such as hand muscle atrophy, in the differential diagnosis. His legacy continues to influence modern neurology, reinforcing the value of precise clinical observation in the diagnostic process.

Charcot neuroarthropathy is characterized by progressive joint destruction resulting from the loss of pain sensation. The most common manifestation is Charcot foot associated with diabetic peripheral neuropathy, which is a serious complication that often leads to amputation due to ulcers or infection. Early diagnosis is challenging but crucial as it strongly influences treatment outcomes. Off-loading is the cornerstone of management, and surgical intervention should be considered in advanced cases. The primary goals of treatment are to minimize deformity, prevent ulceration, and preserve the functional ambulatory foot. Lifelong management is essential to achieve favorable outcomes.

Charcot-Marie-Tooth disease is a hereditary motor and sensory neuropathy characterized by progressive distal muscle weakness, atrophy, and sensory impairment. In this article, we review the original reports published in 1886 by Charcot, Marie, and Tooth, analyzing the clinical features they used to distinguish Charcot-Marie-Tooth disease from other forms of muscle atrophy. We then trace the historical development of the disease concept and examine evolving classification systems based on clinical, electrophysiological, and genetic findings. The identification of more than 140 causative genes has complicated traditional classifications based on nerve conduction studies and inheritance patterns. Thus, we also provide an overview of the current diagnostic strategies that combine clinical evaluation with genetic testing. Although no curative treatment is currently available, advances in molecular biology have introduced new avenues for pharmacological and gene-based interventions. Accordingly, we present emerging therapeutic approaches, including siRNA and CRISPR-Cas9 targeting PMP22 for CMT1A, as well as other nucleic acid-based drugs under development.