Brain and Nerve最新文献

This review summarizes the monumental achievements of Joseph Godwin Greenfield, the father of neuropathology. His books and papers remain relevant, even in modern medicine. They offer valuable insights that warrant revisiting. Aspiring neuropathologists are strongly encouraged to study his works.

Deep brain stimulation (DBS) has been a common treatment for Parkinson's disease, tremors, and dystonia since its approval in Japan for movement disorders in 2000. In the EU and USA, DBS has been studied as a therapeutic option for depression, obsessive-compulsive disorder, Tourette syndrome, and cluster headache. DBS functions via a neurophysiological mechanism in movement disorders rather than pharmacological mechanisms. In this review, I have attempted to provide an overview of DBS from its history to the most recent information.

Oliver Sacks transcended the boundaries between neurology and literature, conveying the mysteries of the brain and mind, and the profound depths of human existence to people worldwide. He was not only an expert in neurology but also well-versed in biology, history, the humanities, and the arts; thus, he was one of the defining "polymaths" of the 20th century. His writings depicted various neurological disorders with medical rigor and literary empathy, viewing patients, not merely as "case reports", but as "individuals with stories". He pioneered a new path by merging clinical neurology with literature, as such, he was a forerunner of "narrative medicine".

Robert Wartenberg (1887-1956) was a Jewish neurologist who made significant contributions to modern neurology through the refinement of the neurological examination and his role in founding the American Academy of Neurology. His life and work bridged European and American medical traditions. However, in 1953, he defended Nazi collaborator Julius Hallervorden's lecture, sparking a major ethical controversy. This article examines his diagnostic legacy and the ethical dilemmas arising from Cold War-era politics.

Guillaume-Benjamin-Amand Duchenne de Boulogne once remarked that he found neurology "a sprawling infant of unknown parentage, which he succored to lusty youth." He was born in the Boulogne-sur-Mer region of France in1806. He studied medicine in Paris from 1826 to 1831 and later established a practice in Boulogne. In 1835, he observed isolated muscular contractions produced by electropuncture and began investigating the electrical excitation of muscle. In 1842, he left Boulogne for Paris to continue medical research, often visiting hospitals with his electrical equipment to examine unusual cases. He was an inventive investigator who developed several technical innovations, including electrodiagnosis, electrotherapy, needle muscle biopsy, and medical photography. He enumerated his major discoveries in "L'Electrisation Localisee," which included descriptions of progressive muscular atrophy (now called spinal muscular atrophy), atrophic paralysis of childhood (poliomyelitis), progressive locomotor ataxia (tabes dorsalis), glosso-labio-laryngeal paralysis (progressive bulbar palsy), and pseudohypertrophic paralysis (Duchenne muscular dystrophy). Based on Duchenne's intensive investigations, Jean-Martin Charcot refined his medical concepts and established nosological disease entities, calling Duchenne "mon maître en neurologie" (my master in neurology). Duchenne never held a hospital or university position. He died of cerebrovascular disease on September 17, 1875.

Pierre Paul Broca studied the brains of patients with articulatory language deficits and reported that articulatory function is localized in the frontal cortex of the left hemisphere. This discovery of functional localization laid the foundation of neuroscience in general and marked the beginning of brain science of language. According to recent reexaminations of the patients' brains, the lesions responsible for apraxia of speech may instead involve the insula or medial fiber connections. Based on the results of functional neuroimaging and lesion studies, we have identified a "grammar center" in the left inferior frontal gyrus, long regarded as "Broca's area." Therefore, the traditional view that Broca's area is the sole center of speech production requires revision.

Hiroshi Kawahara (1858-1918) graduated from Tokyo Medical School, the predecessor of the present Faculty of Medicine at the University of Tokyo, where he was strongly influenced by Erwin von Baelz. Soon after, Kawahara was appointed to a professorship of internal medicine at Aichi Medical School (now Nagoya University, School of Medicine). One of Kawahara's greatest contributions to Japanese neurology was the first publication of a textbook of clinical neurology written in Japanese. Published in 1897, it contains 469 pages of text with many original figures observed by Kawahara himself, covering a variety of organic and functional neurological diseases. Kawahara was well versed in many European neurological journals and writings and incorporated the essence of modern European neurology into this textbook. His masterpiece in neurology is the first description of brother cases worldwide of X-linked recessive spinal and bulbar muscular atrophy (SBMA) in 1897. Later, both basic research and clinical study were developed by researchers mainly at Nagoya University, and the pathophysiology of SBMA was clarified; eventually, disease-modifying therapy was first approved in Japan. Akira Takahashi, who recognized Kawahara's contributions and valued him as a founder of Japanese neurology, should also be appreciated.

Clinical neurology emerged from three European schools of neurology in the 19th century. Romberg established the first neurology school at the University of Berlin, Germany, around 1840, followed by Charcot at the Salpêtrière in Paris in 1862, and, around the same time, Jackson at Queen Square in London. Each went on to establish their own unique neurology schools, supported by the dedicated work of their disciples in the fields of neuroanatomy, neurophysiology, and neuropathology. Among these disciples, Babinski made one of the most important contributions to clinical neurology through the discovery of the "Babinski sign" and his clarification of its clinical significance.

Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is an immune-mediated neuropathy and patients suffer from muscle weakness and impaired quality of life. Current standard treatments for CIDP include immunoglobulin, corticosteroid, and plasma exchange. Efficacy of these treatments has been demonstrated by high level evidence from several randomized controlled trials. Otherwise, there are some concerns about these treatments such as side effects, CIDP recurrence, unstable drug supply, difficulty of use, and the presence of refractory patients. Novel therapies for CIDP are under development, among which an approach to reduce pathogenic IgG, which is thought to be involved in the pathogenesis of CIDP. Efgartigimod is a human IgG1 antibody fragment designed to reduce IgG including pathogenic IgG by binding to the neonatal Fc receptor and blocking the IgG recycling process. The efficacy and safety of subcutaneous efgartigimod were evaluated in the ADHERE trial, a multicenter, randomized-withdrawal, double-blind, placebo-controlled phase II trial in patients with CIDP, in which subcutaneous efgartigimod significantly suppressed the recurrence of CIDP compared to the placebo and was well-tolerated. Based on these results, subcutaneous efgartigimod was approved for the treatment of CIDP in December 2024 in Japan. Other new therapies targeting complement pathways or B cells are under clinical development. (Received May 9, 2025; Accepted July 23, 2025; Published December 1, 2025).

Henry Head (1861-1940) is not well-known in Japan today, yet his achievements were so great that he was nominated four times for the Nobel Prize in Physiology or Medicine, starting with the first award (1901). He is regarded as one of the great founders of clinical neurology. I have encountered his work three times in the literature. The first time was when research on the "thumb localizing test" began. In a joint publication with Gordon Holmes, Head proposed a test to demonstrate defective recognition of posture. They described it as follows: "The patient's eyes are closed, and the affected limb is placed in a position different from its initial one. The patient is then instructed to touch a specific part of the affected limb, such as the index finger, with his normal hand." Subsequently, some investigators described similar tests; however, they did not gain popularity, perhaps due to the difficulty in interpretation. We have examined a large number of cases and established its value as a screening tool for detecting disorders of the posterior column-medial lemniscus system. My second encounter with his work was in their dermatome studies, and the third was in their research on body schema, particularly the report of a case involving phantom limb sensation.