Frontiers of Neurology and Neuroscience最新文献

In neurology and neuropsychology, behavior refers to the way human beings act and make decisions in contact with their environment. Behavioral impairment is therefore defined as a pathology, following brain lesion, that impacts the interactions between the brain-lesioned individual and his/her surrounding social world. First descriptions of behavioral disorders, including neuroanatomical correlates, date back to the mid-19th century. However, attempts towards their systematic identification and analysis only began at the turn of the 19th to 20th century. In this chapter, we shall span 3 main themes by introducing the first case reports based on thorough clinical descriptions, dating back to the 19th century. We then examine the emergence of checklist questionnaires and their application to large cohorts of individuals starting after World War II. Finally, we outline how, over the last 3 decades, the pace has significantly accelerated in the pursuit of defining the fine-grained processes underlying behavioral functioning, as well as the development of new and more complex measures, along with the emergence of the social cognition and social brain concepts. As the assessment tools have expanded and become more specific, an increasing complexity of mechanisms underlying behavior has begun to emerge.

In this chapter, I will present an overview of early case descriptions of specific isolated cognitive deficits in children for which no clear brain impairment could be demonstrated and which were therefore considered to be congenital or developmental in nature. Three kinds of syndromes will be discussed. First, more general deficits like the attention and hyperactivity disorder and congenital aphasia will be presented. The second category relates to the more specific cognitive deficits, like developmental prosopagnosia, that have been reported, especially from the early 1980s onwards. In particular, early reports of specific congenital deficits in the areas of attention, language, perception, and memory will be presented. And finally, I will briefly discuss early case descriptions of individuals with autism and savant syndrome. Instead of suffering from a specific cognitive deficit, the latter show a special talent.

In a series of papers which appeared in 1906, Pierre Marie advanced a new concept of aphasiology against the classical view based on functional localization of cerebral cortex. He denied the role of Broca's area in language function and proposed as the center for articulation "zone lenticulaire," the lesion of which causes anarthria. But his illustration of "zone lenticulaire" which appears repeatedly in his papers dealing with aphasia, is anatomically incorrect since the most important portions of Broca's area, opercular part and triangular part of the inferior frontal gyrus are missing in his illustration. The detailed anatomical investigation of Marie's illustrations reveals that he repeated the same errors in identifying rolandic and frontal opercula. But the similar mistake of identifying these opercula is also seen in Dejerine's "Anatomie des Centres Nerveux."

Édouard Manet (1832-1883) is considered the "father" of impressionism and even of twentieth century modern art. Manet's genius involved getting away from the classical narrative or historical topics and replacing them by the banality of daily life. Technically, he erased volumes into flat two-dimensional coloured planes, and distorted conventional perspective with often gross brushstrokes intentionally giving an "unfinished" aspect to the work. It is little known that Manet had a very painful second part of his life, due to excruciating limb and chest pains, which developed in parallel with proprioceptive ataxia and gait imbalance. Manet always remained discreet about his private life, and we mainly know that his future wife was his family piano teacher, with whom he had a liaison at the age of 17 years. Later, the great but platonic passion of his life was the painter Berthe Morisot (1841-1895), who married Manet's brother Eugène. In fact, we do not know whether he had a mistress at all, although he had several elegant "flirts" in the mundane and artistic milieu. Thus, while Manet's progressive painful ataxia from the age 40 years yields little doubt on its tabetic origin, how he contracted syphilis at least 15-20 years before will probably remain a mystery. It is fascinating that Manet's daily struggle against pain and poor coordination may have led his art to become one of the most significant of modern times, opening the way to twentieth century avant-gardes, along with another victim of syphilis, Paul Gauguin (1848-1903). Manet never showed any sign of general paresis, and like his contemporary, the writer Alphonse Daudet, his clinical picture remained dominated by paroxysmal pain and walking impairment. Difficult hand co-ordination made him quit watercolour painting, and during the last 2 years of his life he had to focus on small format oil works, the subject of which was nearly limited to modest bunches of fresh flowers, now often considered to be his maturity masterpieces. Having become bedridden, he had to be amputated of one leg, which was developing gangrene, probably associated with ergot overuse. While he died shortly thereafter, we have some witness anecdotes suggesting that he experienced a phantom limb: when Claude Monet (1840-1926) visited him and sat down on his bed, Manet violently shouted that he was sitting on his (absent) leg, which provoked terrible pains. With its facts and mysteries, the subtle interaction between Manet's illness and his work output remains one of the most intriguing stories in the neurology of art.

Psychopathology has been closely related with artists. A link between creativity and a tendency to affective disorders has become widely accepted. Several studies have shown that artists suffer disproportionately high rates of mood disorders, particularly manic depression and major depression. The famous twentieth century Spanish artist Joan Miró suffered from depression during the entirety of his life, as was recognized by some authors in private letters. The artist worked using several styles, as well as in ceramics and sculpture. Some of his work reflects the feelings he experienced during the Spanish Civil War and the Second World War. He contributed considerably to the world of art with works such as The Farm as the culminant work of detailism, The Harlequin's Carnival as one of the main expressions of surrealism, the Birth of the World as the precursor of abstract expressionism, or The Dutch Interiors with "mironians" shapes, among others. Whether depression was the inspiration for his work, or his work was the treatment for his depression, will never be clarified. However, he left a great legacy for humanity and his work is admired all over the world.

Since its initial description in 1851, Munchausen syndrome has been widely used interchangeably with factitious disorder. Nevertheless, this syndrome is only one form of factitious disorder that is both severe and chronic. The syndrome was named after Karl Friedrich Hieronymus, Baron von Münchhausen (1720-1797), a German nobleman who became famous as a narrator of false and exaggerated exploits. His name was progressively corrupted to Munchausen. Factitious disorders and Munchausen syndrome remain a great diagnosis challenge for physicians. All medical specialities are concerned by these disorders. The diagnosis process involves a first step to exclude an unusual presentation of a common medical condition. The second step consists of excluding somatoform disorders and malingering. Unfortunately, the boundaries between factitious disorder, somatization, and malingering are often unclear. In 1977, the term "Munchausen's syndrome by proxy" was coined to define a situation where a person produces false symptoms in another one, especially a child. This term was extended to similar interactions between human and pets. Because varied conditions have been included in the definition of this syndrome, there is ongoing debate about alternative names.

The dancing mania erupted in the 14th century in the wake of the Black Death, and recurred for centuries in central Europe - particularly Germany, the Netherlands, and Belgium - finally abating in the early 17th century. The term "dancing mania" was derived from "choreomania," a concatenation of choros (dance) and mania (madness). A variant, tarantism, was prevalent in southern Italy from the 15th to the 17th centuries, and was attributed at the time to bites from the tarantula spider. Affected individuals participated in continuous, prolonged, erratic, often frenzied and sometimes erotic, dancing. In the 14th century, the dancing mania was linked to a corruption of the festival of St. John's Day by ancient pagan customs, but by the 16th century it was commonly considered an ordeal sent by a saint, or a punishment from God for people's sins. Consequently, during outbreaks in the 14th and 15th centuries, the dancing mania was considered an issue for magistrates and priests, not physicians, even though the disorder proved intractable to decrees and exorcisms. However, in the 16th century Paracelsus discounted the idea that the saints caused or interceded in the cure of the dancing mania; he instead suggested a psychogenic or malingered etiology, and this reformulation brought the dancing mania within the purview of physicians. Paracelsus advocated various mystical, psychological, and pharmacological approaches, depending on the presumptive etiologic factors with individual patients. Only music provided any relief for tarantism. Later authors suggested that the dancing mania was a mass stress-induced psychosis, a mass psychogenic illness, a culturally determined form of ritualized behavior, a manifestation of religious ecstasy, or even the result of food poisoning caused by the toxic and psychoactive chemical products of ergot fungi. In reality, dancing manias did not have a single cause, but component causes likely included psychogenic illness, malingering, and ritualized behaviors.

Visual art is one of the means of non-verbal communication that bypasses cultural, societal, language and, more importantly, time differences. It allows for establishing a multilevel connection between the artist and art receiver. Production of visual art is a form of expression of emotions. Art reception involves the initiation of a cascade of emotions and thoughts based on visual input. One of the ways to express artistic content is through abstraction. Abstract visual art is based on portraying elements that do not represent any real, objective shapes, with the means of lines, colours, tones and texture. Abstract expressionism is a form of abstract art infused with strong emotional and expressive content. The combination of expression of emotions in abstraction requires almost direct translation between neuronal function and artistic output without using formal shapes or references as means of communication. That is why it is very interesting to look at the artistic output in abstract expressionists with neurological disorders affecting the brain. Here, we review several key abstract expressionists, including James Brooks, Agnes Martin and Willem de Kooning, and their artistic production in the context of brain disease.

In 1974, Critchley described misoplegia as the phenomenon in which a hemiplegic patient develops a morbid dislike towards the offending immobile limbs. Patients with misoplegia may employ, but more commonly strike their paretic limbs not recognized as self. The pathophysiological mechanism is not well understood. The handful of cases of misoplegia described in the literature, frequently presented a right hemispheric damage. However, patients with chronic spinal cord injury may also present this symptomatology. Not only the modification of behavior by this organic injury, but also the patient reaction to disability and previous personality, may provoke the emergence of misoplegia, probably from other right hemispheric self-unawareness syndromes. No data exists related to treatment option, but we have to remember that the lack of awareness of the deficits in these patients makes the rehabilitation process difficult. Misoplegia is one of the passionate syndromes of the still "not-enough well-known" self-awareness syndromes of the right hemisphere, which shows how brain damage goes much further beyond neurological deficit.