The unbearable lightness of the extrapyramidal system.

The concept of the extrapyramidal system comprises an amalgam of disparate and often conflicting ideas with a tortuous history. To the theoretical neuroscientist or practicing clinician, it promptly evokes semantic associations that are hardly reminiscent of its original meaning. The purpose of this article is to revisit the sources of the extrapyramidal concept and to examine the transformations that it went through from its inception, in the late 1890s, up to the neuroimaging revolution of the 1980s. Our review shows that the use of "extrapyramidal" as a surrogate for the basal ganglia, disorders of movement, or certain manifestations of spastic hemiplegia does not apply to humans; rather, it represents the historical product of the unwarranted translation of results of animal experimentation into the interpretation of clinical findings on human patients, misguided clinico-anatomic deductions, and fanciful phylogenetic notions. We conclude that the extrapyramidal concept is a valid and robust anatomic concept as long as it strictly refers to the collection of descending fibers originating in a few discrete brainstem tegmental motor nuclei that project to the spinal cord.

Causalidad psíquica en un caso de locura. A propósito de Unica Zürn / Psychic causality in a case of folly. Apropos of Unica Zürn

Mediante el análisis de la obra literaria de la autora alemana Unica Zürn se pretende dar cuenta de la causalidad psíquica de su locura. Los tres textos autobiográficos que se trabajan en este análisis son: El trapecio del destino y otros cuentos, El hombre jazmín. Impresiones de una enfermedad mental y Primavera sombría (AU)

By analyzing the literary work of the German writer Unica Zürn, the author seeks to account for the mental causation of her insanity. The three autobiographical texts worked out this analysis are: The trapezium on destination and other stories, The man of jasmine. Impressions from a mental illness and Dark spring (AU)

[Post-encephalitic syndromes in the Spanish medical literature]. / Síndromes postencefalíticos en la literatura médica española.

AIM: A large number of patients with encephalitis lethargica developed different post-encephalitic syndromes (PES), which have an important medical and social impact. We studied the clinical and historical aspects of PES in Spain by reviewing the medical literature published in this country between 1918 and 1936. DEVELOPMENT: There are no statistical data concerning PES in Spain, although Spanish physicians drew attention to their high rate of prevalence and their repercussions on community health. Most of the 140 patients that were reviewed (74%) presented predominant Parkinsonism, but some features of Parkinsonism were observed in nearly all cases. Other movement disorders (focal dystonias, chorea, myoclonus, oculogyric crises, abnormalities affecting breathing rate) were described, as well as sleep, endocrine and vegetative disorders. Psychiatric disorders were often reported, the most frequent being bradyphrenia associated to Parkinsonism, but a hypomanic picture with impulsive behaviour was very characteristic among young people. PES was diagnosed on average two years after the episode of acute encephalitis lethargica, although it often appeared immediately afterwards. Many studies discuss the contribution made by PES to further our knowledge of the pathophysiology of extrapyramidal diseases and about the involvement of the basal ganglia in psychiatric and behavioural disorders. CONCLUSIONS: Despite the absence of statistical data, Spanish authors highlighted the important repercussions the PES had on community health, as well as the role they played in extending our knowledge of the pathophysiology of the basal ganglia. Cases of Parkinsonism were predominant, although all kinds of post-encephalitic manifestations were reported.

Evolution of basal ganglia surgery for movement disorders.

In 1942, it was thought that basal ganglia surgery would cause permanent unconsciousness and significant impairment of motor control. By 1947, when human stereotactic surgery was introduced, the first target was the globus pallidus in a patient with chorea. What happened during those 5 years to set the stage for stereotactic surgery? During the last half of the 19th century, it was first noted that motor disorders were often accompanied by atrophy of various parts of the basal ganglia, and when histopathology became part of necropsy, that relationship between movement disorders and the basal ganglia was strengthened. The impairment of fine motor control was noted in experiments that involved lesioning the basal ganglia, which led to the conclusion that disease of the basal ganglia might cause motor impairment. Finally, in 1939, Russel Meyers took the bold move of surgically resecting the head of the caudate nucleus at craniotomy in a patient with Parkinson's disease, demonstrating that Dandy was wrong in the view that the basal ganglia were the center of consciousness, and that symptoms and motor control might be improved by caudate lesions without motor impairment. He reported his first patient in a meeting in 1940, which was published in 1942, and was encouraged to investigate basal ganglia surgery further. Although results were encouraging, the mortality rate was prohibitive. Since the introduction of pallidoansotomy in 1947, basal ganglia surgery has become both safe and effective and has been expanded and refined.

Sleep disorders and extrapyramidal diseases: an historical review.

BACKGROUND AND PURPOSE: Sleep disorders have been mentioned since the first descriptions of extrapyramidal diseases in James Parkinson's Essay on the Shaking Palsy, but only recently they have become the subject of attention, thanks to new acquisitions in clinical knowledge and electroencephalographic technology. In the late 1960s, the introduction of L-dopa permitted comparison of sleep patterns in drug-naive patients before and after therapy in conditions very similar to experimental ones. Historically, we can recognise two major lines of study, one dealing with descriptions of sleep behaviours modified by drugs and the other with polysomnographic sleep research carried out before and after treatment. PATIENTS AND METHODS: The data obtained from the first polysomnographic studies led to the definition of sleep macro- and microstructure in patients suffering from Parkinson's disease, but the interpretation of drug-induced changes was not unequivocal. RESULTS: According to some authors, the improvement in sleep architecture was due mainly to improvement of nocturnal motor impairment. Other researchers suggested a primary sleep dysfunction caused by specific neurodegenerative processes in the brain structures regulating the sleep-wake cycle. CONCLUSIONS: The latter hypothesis has recently been supported by the observation that distinct sleep disorders, such as REM behaviour disorder or restless legs syndrome, often herald extrapyramidal diseases or are a frequent adjunctive complaint for these patients.

[The beginning of stereotactic treatment of extrapyramidal disorders in Poland]. / Poczatki stereotaktycznego leczenia chorób ukladu pozapiramidowego w Polsce.

Oskar Liszka in cooperation with I. Goscinski i Z. Wicentowicz in 1961 introduced in Poland Guiot-Gillingham stereotactic method of operation and then modified it in 1967. In the next years stereotactic procedures in extrapyramidal diseases were performed also in Warszawa--J. Subczynski, E. Mempel and J. Bidzinski, in Bialystok J. Lebkowski, in Szczecin J. Slósarek and I. Kojder, in Bydgoszcz M. Harat, in Gdansk P. Sloniewski. Contemporary W. Koszewski and M. Zabek in Warszawa. In Lublin T. Trojanowski et al. use stereotactic radiotherapy in the treatment of angiomas of the brain.

A historical case of probable corticobasal degeneration?

In 1925, Jean Lhermitte and colleagues published a work on spatial representation in apraxic patients, based on two illustrative cases. One of these displayed a clinical picture that would nowadays be clinically classified as probable corticobasal degeneration.

Encephalitis lethargica: lessons for contemporary neuropsychiatry.

Encephalitis lethargica (von Economo's encephalitis), pandemic from 1917 to 1926, opened a window on the study of behavioral consequences of infection-induced subcortical disorder. Widely varying acute manifestations included extrapyramidal disorders, myoclonus, eye movement disorders, paralyses, delirium, mood changes, inverted diurnal rhythms, and catatonia. Major pathological changes involved the substantia nigra, globus pallidus, and hypothalamus. A symptom-free recovery period was often followed by postencephalitic disturbances, typically parkinsonism in adults and conduct disorder in children. Occurrence of depression, mania, obsessive-compulsive disorder, and hyperactivity in post-encephalitic patients anticipated current concepts of the role of the basal ganglia in mood, personality, and obsessional syndromes. Observations of deferred onset and "tardy" hyperkinesias presaged current theories of the pathophysiology of tardive dyskinesia.

[History of the basal ganglia system. Slow development of a major cerebral system]. / Histoire du système des ganglions de la base. La lente émergence d'un système cérébral majeur.

Initially, basal ganglia was a descriptive term for onto- and phylogenetic or topographic classifications. A variable list of structures were included as basal ganglia. A major step was made when the thalamus was separated from the "striated bodies" (Vic d'Azyr, 1786) which was sometimes taken into account in the French description of the noyaux gris centraux. Even if the term is not perfect, it is preferable to "the system of basal ganglia". The subdivisions of the putamen, the distinction between the striatum and the pallidum were not really made until the beginning of the twentieth century. Modern tracing methods were needed to demonstrate the main connections. It was not until the end of the 1960s that the importance of the striato-pallido-nigral network within the basal ganglia and the cortico-striatal connections, the main afferent system, were recognized. With the description of the cortico-striatal connections, the sub-cortical system with multiple complex "loops" was questioned. The term "extra-pyramidal system" had an exaggerated success. Initially, it designated descending non-pyramidal afferents (some which do not exist) and their source. In 1992, Spatz based his separation of this heterogeneous group on the iron content. The terms of extra-pyramidal "system" and "syndrome" should be abandoned by clinicians. Physiological interpretations have varied. The role of automatic "habitual" motricity, derived from a concept of hierarchic, Jacksonian cerebral organization, was questioned when the pyramidal network was described. Clinico-pathological analysis (hemiballism, Parkinson's disease ...) has placed new emphasis on the motor role, for a time the only role accepted as real. More recently, debate has centred on other roles, particularly in cognition and motivation. An illustration of functions other than purely motor functions of the basal ganglia is given by the syndromes of loss of psychic auto-activation secondary to bilateral lesions.

Extrapyramidal side effects: a historical perspective.

A historical review of the adverse reactions to neuroleptic agents--extrapyramidal side effects--is presented. Soon after its introduction in 1952, chlorpromazine was noted to induce symptoms resembling Parkinson's disease. At first, these symptoms were thought to be related to the drug's antipsychotic effect. Later, more careful research showed that they are not directly associated with the antipsychotic activity of neuroleptic agents. More recently, extrapyramidal side effects have gained importance because they are significant factors in both the patient's acceptance of the particular drug and his or her social adjustment.