Frontiers of Neurology and Neuroscience最新文献

The custom of Couvade and Couvade syndrome is a phenomenon observed since ancient times. Whether it constitutes a disease entity or it should be considered a ritual or custom remains a matter of debate. Historical transcripts shed light into the distinct origins and inclinations of couvade behaviors, some of them having religious inclinations. Currently, there are several views on this phenomenon including medical, psychoanalytic, and psychological. Some explain this syndrome as part of men's preparation and participation in pregnancy and post-partum period. Others see it as men rivalling with women for procreative ability or ability to carry an unborn child in the womb. There are a set of criteria that can be used in diagnosing Couvade syndrome, which may be helpful in standardizing clinical detection and management of patients. It is important to embed this syndrome in contemporary society to understand the complexity of male involvement in pregnancy and fatherhood, which at the end may not be a disease. In this review, the authors will guide the reader through history, possible etiologies, and clinical aspects of Couvade syndrome.

A right "minor hemisphere" does not exist as the right hemisphere is dominant for awareness (nosognosia), spatial attention, emotional regulation, facial and voice expressions, visual recognition, and topographical orientation. Without the right hemisphere, the world would be flat, deprived of general and spatial attentions, pointing preferentially to the right side of the space, lacking visual experiences and emotions, exhibiting diminished awareness of the self and environment. Clinical-related syndromes of the right hemisphere are unilateral spatial neglect, object and face visual agnosia, the anosognosia for hemiparesis and/or hemianopia, misidentification syndromes, mania, and other obsessions for the food and the body. Another key function of the right hemisphere is the modulation of the emotional processes of the linguistic communication (as prosody and facial expressions), and the tuning of some holistic aspects of language as the understanding of the abstract and figurative characters. The great mysteries of the right brain hemisphere concern the origin of the emotional nature of the human being, the way by which cognition interacts with perception and finally the human consciousness. Multidisciplinary researches in the domains of neurology, cognitive psychology, neuropsychiatry, functional neuroimaging, and neurophysiology will reveal in the future some of these mysteries.

In 1937, Heinrich Klüver and Paul Bucy described a dramatic behavioral syndrome in monkeys after bilateral temporal lobectomy. The full Klüver-Bucy syndrome (KBS) - hyperorality, placidity, hypermetamorphosis, dietary changes, altered sexual behavior, and visual agnosia - is evident within 3 weeks following operation. Some KBS features (i.e., hyperorality, placidity, hypermetamorphosis) persist indefinitely, whereas others gradually resolve over several years. Klüver and Bucy were initially unaware of an earlier report of KBS by Sanger Brown and Edward Schäfer in 1888. Human cases were recognized in the 1950s, as surgeons employed bilateral temporal lobectomies to treat seizures. Various attempts were made to localize the component features to specific areas of the temporal lobe, with mixed success. Bilateral ventral temporal ablations and bilateral temporal lobectomies produced marked impairment in visual discrimination, whereas lateral resections or unilateral lesions did not. Discrete bilateral lesions of the lateral amygdaloid nucleus produced a permanent "hypersexed state." By the 1970s, it was clear that the major symptoms of KBS are produced by destroying either the temporal neocortex or the amygdala bilaterally. KBS is now thought to be caused by disturbances of temporal portions of limbic networks that interface with multiple cortical and subcortical circuits to modulate emotional behavior and affect. The clinical features of KBS in man are similar to those in monkeys, but the full syndrome is rarely seen, probably because the anterior temporal lobe dysfunction is usually less severe than that following total temporal lobe ablation in monkeys. Human KBS does not occur in isolation, but is typically part of a complex behavioral syndrome that almost always includes amnesia and aphasia, and that may also include dementia and seizures. The treatment of KBS is difficult and often unsatisfactory.

Different eponyms such as "Wood syndrome," Meige syndrome, "Brueghel syndrome," "Blepharospasm plus syndrome" have been used to describe segmental craniocervical dystonias. These facial and/or oromandibular movement disorders are characterized by muscle contractions and spasms involving eyes, facial region, and sometimes pharynx, jaw, floor of the mouth, and tongue. The pathophysiology of craniocervical dystonia is poorly understood, but abnormal plasticity and impaired inhibition are suspected. Injection of botulinum toxin appears to be the best therapeutic option for treating segmental craniocervical dystonia. The objective of this chapter is to depict the history of segmental craniocervical dystonia in order to delineate the phenotypic spectrum of the disorders and to distinguish this entity from other facial and/or oromandibular movement disorders.

Descriptions of hallucinatory phenomena have figured prominently since the beginning of recorded history. Jean Etienne Esquirol (1772-1840) is usually credited for having introduced the term in 1817, differentiating between hallucinations and illusions. Both are wrong perceptions, but in illusions, an external stimulus is always present whereas hallucinations are perceptions that occur in the absence of corresponding sensory stimuli. They occur in a variety of conditions but more often in the mentally ill, especially in schizophrenia where hallucinations, particularly auditory hallucinations represent for many, such as Henri Ey one of the cardinal features. This chapter, however, deals with visual hallucinations as found in individuals who are not necessarily mentally ill: the Charles Bonnet syndrome and autoscopy.

Thomas Mann (1875-1955) is considered one of the most influential writers of the late nineteenth and early twentieth century. In addition to his novels and essays, he was well known for his criticisms of the Nazi party, and particularly against the racial nationalism promoted by Adolf Hitler after the First World War, as well as for his depiction of diseases. Here, we provide a quick sketch of Mann's life and his relationship with nineteenth to twentieth century German society. We then proceed to describe how Mann became interested in diseases, how he used the diseases as metaphors, and his specific contribution to the field of neurology. We describe some of the neurological cases portrayed in Mann's work, and particularly epilepsy described in The Buddenbrooks, Felix Krull and The Magic Mountain, meningitis, neurosyphilis and migraines depicted in Doctor Faustus, and essential tremor described in The Magic Mountain and Doctor Faustus. We conclude with reflections about Mann's interest in diseases and particularly in neurology.

The studies on the relation between artistic production, especially visual art, and brain function gave a basis to the development of neuroesthetics. Most of the information on brain artistic creativity comes from studies on brain disease in well-established visual artists. Brain disease may cause change, dissolution, or emergence of artistic creativity. The visual artistic production may become impaired in individuals with a variety of brain diseases, including focal and generalised disorders of sudden and slowly progressive onset. In addition to that, neurological disorders may add content into visual art production, even becoming the artistic theme. Here, we discuss the particular case of abstract expressionist Willem de Kooning and the evolution of his artistic production in the context of his neurodegenerative illness. The change of de Kooning's artistic style has been the subject of many reviews, and the main focus of this paper is on artistic style across his prolific artistic career in the context of his progressive neurological condition, which he developed in his late years, and his long history of alcohol addiction. There are conflicting data from the literature on the effect of his neurological condition and clear cognitive decline on his artistic output, with preservation of recognition and the quality of his art. Hence, two pertinent questions relate to how he was able to maintain his output despite his cognitive decline, and how his condition could have affected his work.

The delusional misidentification syndromes (DMS) are a group of disorders, characterized by patients mistaking the identity of people they know, although they recognize them physically. The term DMS is an umbrella term which may cover disorders whose definition extends to objects other than people, such as animals, places, or familiar material objects. The most common and best known DMS is Capgras syndrome. In this disorder, the misidentification leads to the delusional conviction that a close friend or relative has been replaced by an identical - or almost identical - "double," whose original has disappeared. This double is an imposter without name or identity. Most often considered as a persecutor, the double may be subjected to aggression, which may be very violent. Neuropsychological hypotheses based on cerebral dysfunctions are now commonly considered to be at the origin of the disorder. They have been elaborated from achievements in the neurosciences, particularly the facial recognition models. In return, knowledge about the normal cognitive processes involved in recognition and familiarity has benefited from the work that cognitive neuropsychiatry has invested in these disorders. The DMS are observed in various contexts of morbidity: primary psychiatric diagnosis, or secondary to various organic disorders, particularly in neurodegenerative disease; they are rarely met in isolated form. Most often, they develop in line with the associated pathology. In the absence of consensual clinical description, the epidemiology of DMS is uncertain; they may be more frequent than previously supposed. There is no specific treatment for these disorders; neuroleptics are generally used in association with treatment of the concomitant disorder. The frequent association of DMS with organic disorders which may be curable and the particularly dangerous profile of these patients are factors that underline the need for better screening.

Non-bizarre delusion, defined as a false belief possible although highly unlikely, is the main manifestation of delusional disorders, previously known as paranoia. Based on the predominant delusional themes, 5 main subtypes may be described - erotomanic, grandiose, jealous, persecutory, and somatic. We present here 2 main delusional disorders, the De Clérambault syndrome and the Othello syndrome, and another closely related to the previous ones - Folie à deux. In the De Clérambault syndrome, the main delusional theme is erotomanic type, related to passional delirium where the patient has strong sexual feelings towards another person and has the belief that this other person is deeply in love with him or her. Patients with the Othello syndrome present a delusional disorder of jealous type, a pathological delusion that the partner is unfaithful. In Folie à deux, 2 individuals shared the same psychiatric symptom. However it may be variable, describing variants such as folie imposée or folie simultenée. The risk of aggressive behavior exists in these patients. Knowledge of these syndromes is essential to allow an accurate diagnosis and prompt treatment.

This chapter is aimed at highlighting the recent findings concerning physiopathology, diagnosis, and management of conversion, factitious disorder, and malingering. Conversion disorder is the unintentional production of neurological symptom, whereas malingering and factitious disorder represent the voluntary production of symptoms with internal or external incentives. They have a close history and this has been frequently confounded. Practitioners are often confronted to medically unexplained symptoms; they represent almost 30% of neurologist's consultation. The first challenge is to detect them, and recent studies have confirmed the importance of "positive" clinical bedside signs based on incoherence and discordance, such as the Hoover's sign for the diagnosis of conversion disorder. Functional neuroimaging has allowed a better understanding of the pathophysiology, and highlighted abnormal cerebral activation patterns in conversion disorder in relation to motor, emotional, and limbic networks, different from feigners. This supports the theory evoked by Charcot of a "psychodynamic lesion," which is also reflected by the new term introduced in the DSM-5: functional neurological disorder. Multidisciplinary therapy is recommended with behavioral cognitive therapy, antidepressant to treat frequent comorbid anxiety or depression, and physiotherapy. Factitious disorder and malingering should be clearly delineated from conversion disorder. Factitious disorder should be considered as a mental illness and more research on its physiopathology and treatment is needed, when malingering is a non-medical condition encountered in medico-legal cases.