Frontiers of Neurology and Neuroscience最新文献

In the late 19th century, jumping (French Canadians in Maine, USA), miryachit (Siberia), and latah (Southeast Asia) were among a group of similar disorders described around the world, each of which manifests as an exaggerated startle response with additional late-response features that were felt by some to overlap with hysteria or tics. The later features following the exaggerated startle reaction variably include mimesis (e.g., echopraxia, echolalia) and automatic obedience. These reaction patterns tended to persist indefinitely in affected individuals. Because of their dramatic stimulus-driven behaviors, affected individuals were prone to be teased and tormented by being repeatedly and intentionally startled. Despite clinical overlap between jumping and Tourette syndrome, these entities are now recognized as distinct: in jumping, the key feature is an abnormal startle response, the abnormal reaction is always provoked, and tics are absent, whereas in Tourette syndrome, the key feature is spontaneous motor and vocal tics, although patients with Tourette syndrome may occasionally also have an exaggerated startle response. These disorders have been conceptualized from anthropological, psychodynamic, and neurobiologic perspectives, with no complete resolution to date. Attempts at treatment have been generally unsuccessful, including attempts with bromization and hypnosis, although anecdotal reports of successful deconditioning have been published. In population groups affected, these disorders are usually considered as behavioral peculiarities and not as diseases per se, and there is no apparent tendency to develop disabling mental illness or neurodegenerative disorders. The genesis of these disorders, their cultural and social components, and their interactions with the presumed underlying physiological substrate need further study. Careful descriptive and analytic epidemiological studies are also lacking for all of these disorders.

Camptocormia is defined as an involuntary flexion of the thoracolumbar spine, without fixed kyphosis, which increases during walking and standing, and abates in the supine position. First described during World War 1 in soldiers suffering from war psychoneuroses, camptocormia has progressively come to refer to any cause of trunk forward-flexed posture during standing and ambulation. It is now admitted that camptocormia should be considered as a syndrome related to many etiologies. In this chapter, we present the historical aspects of the syndrome and its main etiologies. We highlight camptocormia in Parkinson disease and its relationships with Pisa syndrome.

The Italian poet, novelist, translator, editor and diarist Cesare Pavese (1908-1950) famously committed suicide after winning the coveted Strega prize for his 1949 trilogy of novels La bella estate (The Beautiful Summer). This article discusses one of the novels in that trilogy, Among Women Only (Tra donne sole), presenting it as a psychological "laboratory" in which the intensely private Pavese explores the rationale for suicide on the very public stage of the novel. The author argues that the writing of Tra donne sole was one of the self-willed steps that made it not just possible for Pavese to commit suicide, but impossible not to.

Diogenes syndrome (DS) is not a specific disease but a real neurobehavioral syndrome, characterized by severe domestic squalor, pathological hoarding, lack of insight into the condition, and no need for help. DS can be secondary when associated to psychosis or bipolar disorder, or primary when it occurs as a single entity, usually in the elderly. DS is a clinically complex transnosographic syndrome for which multidimensional approaches need to be considered: medical, psychiatric, neurological, social, scientific, and ethical. Known for more than 40 years mainly by geriatricians, psychiatrists, nurses or social workers and more recently by forensic specialists, the fine grained mechanisms of the syndrome are still incompletely understood. From a neurocognitive standpoint, frontal vulnerability certainly disrupts normal decision-making processes, explaining squalor, pathological hoarding, and lack of insight but we need to better understand the connection between the main symptoms and the neural underpinning of the full syndrome. We should definitely intervene earlier, before patients refuse any help, and when the syndrome is supposedly milder, to improve our clinical knowledge, follow patients prospectively, experiment hypothesis in laboratory settings, and launch randomized controlled trials for treatments. There is hope for future pharmacological and non-pharmacological strategies to alleviate this socially disastrous condition.

Ganser's syndrome is a rare and controversial condition, whose main and most striking feature is the production of approximate answers (or near misses) to very simple questions. For instance, asked how many legs a horse has, Ganser patients will reply "5", and answers to plain arithmetic questions will likewise be wrong, but only slightly off the mark (e.g., 2 + 2 = 3). This symptom was originally described by Sigbert Ganser in 1897 in prisoners on remand and labeled Vorbeigehen ("to pass by"), although the term Vorbeireden ("to talk beside the point") is also frequently used. A number of associated symptoms were also reported: "clouding of consciousness," somatoform conversion disorder, hallucinations, sudden and spontaneous recovery, subsequent amnesia for the episode, premorbid traumatic psychosocial experience and/or (usually mild) head trauma. Etiological, epidemiological and diagnostic issues have never been resolved for Ganser's syndrome. Ganser saw it as a form of "twilight hysteria," whereas others suggested that malingering, psychosis or dissociation were more appropriate labels, oftentimes combined with organic impairment and a subjectively intolerable psychosocial context. A central conundrum of Ganser's syndrome is whether it could simultaneously be a cultural and pathological representation of insanity, whereas cognitive, organic, affective, motivational and social factors would converge towards a naïve idea of what mental illness should look like, especially through the provision of approximate answers.

Many artists were involved in the First World War. Some of them were mobilized, like millions of soldiers, others enlisted to fight on the battlefield. The stories of writers who returned neurologically wounded from the war, such as Guillaume Apollinaire (1880-1918) or Blaise Cendrars (1887-1961), are well-known. The cases of painters and sculptors who suffered from First World War neurological wounds are scarce. Nevertheless, their injuries led to intense modifications of artistic practice. We detail four examples of artists whose creative mind was impacted by their First World War neurological wounds or diseases. The painter Jean-Julien Lemordant (1878-1968), who suffered from blindness after his injury, stopped his artistic work and became an icon of Franco-American friendship. The sculptor Maurice Prost (1894-1967), suffering from a neuroma due to the loss of his arm, built a special device to continue his work as a wildlife artist. The painter Georges Braque (1882-1963) was trepanned but carried on with his cubist work without ever mentioning the conflict. Conversely, the painter Fernand Léger (1881-1955), who suffered from a war neurosis, produced a significant war testimony through drawings and letters.

Yawning, laughing, and crying are normal physiological behaviors of humans in good health. As with all physiological behaviors, their deregulation can reveal disorders. Pathological yawning occurs in salvos of 10-20 successive yawns, and the number of yawns per day can exceed one hundred. After listing the functional etiologies, we will give the clinical keys for differentiating the most serious causes: iatrogenic, tumors, strokes, amyotrophic lateral sclerosis, and intracranial hypertension. Sudden, uncontrollable episodes of emotional display involving pathological laughing and crying (PLC) may be encountered in various neurological diseases: amyotrophic lateral sclerosis, multiple system atrophy (cerebellar), cerebrovascular disease, traumatic brain injuries, mass lesions in the cerebellopontine junction, and epilepsy. After describing the pathophysiology of PLC and the use of diagnostic scales for PLC, we will discuss the current treatments.

The word glossolalia, also referred to as "speaking in tongues," originates from the Greek "glossa" which means "language" and "Lalia" which means "speak." It simply means to talk language. On a linguistic perspective, glossolalia is characterized by almost no recognizable words or semantic content, apart from biblical words and phrases, with an overrepresentation of a small phonemes number, accelerated speech output, and modification of accents and melody. Its phonemic properties have been said to resemble those of the language(s) of the speaker. It is generally a voluntary intimate communication act associated with religious spiritual thoughts, although it has also been described in mental disorders. Glossolalia state is a special mental state, completely different from aphasia. Aphasia is characterized by some phonological and grammatical rules; however, these are related to neurolinguistics and not to developmental principles. Unlike aphasia, glossolalia is not associated with central nervous system affection. There is, however, a psychiatric type of glossolalia, which, although similar in terms of phenomenology, is probably a different phenomenon. A common thought is that there is a linguistic trend, whose basic component is a stream of speech. This stream is suggested to use early-acquired rules of phonation, adapted according to socially meaningful values and attitudes, in an unconscious way. Therefore, the act of glossolalia is out of the "glossolalists" control. It would appear, therefore, that there is a broad spectrum of anomalous speech, of which religious glossolalia is only one manifestation. The aim of this chapter is to understand the mental state of glossolalia in comparison to aphasia in the light of neurolinguistic and psychiatric features. Moreover, we tried to highlight the underlying brain network. Pilot neuroscientific data suggest that it is associated with a deactivation of the cognitive system and an activation of some parts of the mirror neuron networks.

Franz Kafka's Letter to His Father is one of the greatest examples in world literature of memory of a traumatic childhood. In it, the author takes a retrospective journey through his life, recollecting and analyzing the reasons for the estrangement and hostility between a father and a son. This essay considers Letter to His Father in the light of current knowledge about autobiographical memory. The essay first sets forth basic aspects of Kafka's life in order to place Letter to His Father in the context of Kafka's biography, and then presents Kafka's relevance to the literature and thought of the twentieth and twenty-first centuries. The essay then considers the different forms of childhood abuse and their consequences in light of evidence from neurodevelopmental psychology. We present evidence about the relationship between trauma and the construction of self-image. Furthermore, we discuss the subjectivity of Kafka's recollections from the perspective of recent advances in neurobiology. Memory is shown to be dynamic, selective, inherently malleable and dependent on perception, which is a subjective construction, in which the brain interprets and gives coherence to experienced stimuli. We consider the inaccuracy of memory, which is related to neuroplastic changes in the brain that take place over time: consolidation, reconsolidation and transformation. Finally, the relationship between literature and autobiography in the Kafkaesque universe is considered.

The writer Louis Ferdinand Céline (1894-1961) developed a personal style which changed twentieth century French literature. As an enlisted soldier in 1912, he was involved in the Great War and his right arm was severely wounded. After the war, he became a medical doctor and a writer who published his first novel, Voyage au bout de la nuit (Journey to the End of the Night), in 1932. In the middle of the 1930s, he began to write anti-Semitic and racist pamphlets and turned to a collaborationist stance with Nazi Germany. After the Second World War, he was declared a national disgrace in France and fled to Denmark. In 1951, he was granted amnesty and went back to France, where he regained fame with his last three novels. Céline was a First World War neurologically wounded soldier who received a severe injury in the right arm leading to a radial nerve paralysis. Furthermore, in his texts and letters, he complained of many symptoms that he considered to be related to the First World War. In reality, to build a heroic image of himself, Céline rewrote his personal First World War history, in particular his war wounds. The aim of this reconstruction was to help him achieve literary fame. At the end of the Second World War, he also used this rewriting to organise his defence when he was accused and tried for collaborationism. Using medical and military archives, Céline's First World War medical mythology is reviewed to distinguish facts from fiction concerning his wound and other war neurological disturbances. We present the history of his radial nerve lesion and surgery, and confirm that Céline was never trepanned. Two other controversial neurological points, his left ear disease and his possible shell shock, are also discussed.