Peter J. Koehler

How psychogenic is dystonia? views from past to present

In the last few centuries, there has been a constant sway between organic and psychogenic explanations for dystonia. In the current study, we investigate this history, assuming the perspective of a spectrum from organic to psychogenic, between which ideas were moving. We have focussed on (i) primary generalized dystonia, (ii) cervical dystonia, (iii) writers cramp and (iv) fixed dystonia related to complex regional pain syndrome. We have studied medical texts published since the 19th century and their references. Jean-Martin Charcot advocated the concept of hysteria, disorders in which, besides predisposition, environmental factors were involved in their pathogenesis. Sigmund Freud introduced psychoanalysis as an explanatory therapy for psychic disorders. Previous theories, together with the lack of an organic substrate for dystonia, made a strong case for psychogenic explanations. Consequently, many dystonia patients were told that they suffered from psychological conflicts and were treated for them. However, after the description of new hereditary cases in the 1950s, the limited efficacy of psychotherapy in torsion dystonia, the effects of surgical treatments and the lesion studies in the 1960s, more physicians became convinced of the organic nature. The culminating point was the discovery of the DYT1 gene in 1997. In the meantime, experts had already convinced the neurological community that cervical dystonia and writers cramp were focal dystonias, i.e. minor forms of generalized dystonia, and therefore organic disorders. In contrast, the pathophysiology of fixed dystonia related to complex regional pain syndrome remained controversial. Knowledge of this history, which played on the border between neurology and psychiatry, is instructive and reflects the difficulty in discriminating between them. Today, new insights from functional imaging and neurophysiological studies again challenge the interpretation of these disorders, while the border between psychogenic and organic has become more blurred. Abnormalities of sensorimotor integration and cortical excitability that are currently supposed to be the underlying cause of dystonia bring us back to Sherringtonian physiology. We suggest that this may lead to a common explanation of the four afflictions of which we have traced the history.

A history of non-drug treatment in headache, particularly migraine

Although new invasive procedures for the treatment of migraine have evolved during the past decades, the application of invasive procedures for this indication is not new. In this review, the history of non-drug treatments for migraine is discussed. Historical texts by physicians known to have written on headache and migraine (hemicrania), well-known books by physicians from the main historical periods up to 1900 and mainstream 20th century neurology handbooks were analysed. A large number of treatments have been tried, based on contemporaneous pathophysiological models that were not only applied to headache, but to medicine in general. Invasive procedures have been used for the more severe types of headache. Many treatments were based on ancient humoral theories up to the early 19th century. A new kind of invasive procedure appeared on the physicians palette in the 19th and 20th century, following the development of new ideas that were based on solid pathophysiology, after the introduction of scientific method into medicine. After its introduction in the mid-18th century, medical electricity became even more popular for the treatment of migraine following the discovery of vasomotor nerves in the mid-19th century, but at the end of that century a more critical attitude appeared. The discovery of the lumbar puncture (1892) and roentgenogram (1895) and increased knowledge of intracranial pressure led to a new series of invasive procedures for therapy-resistant migraine in the early 20th century. Vasospastic theories of migraine led to surgical procedures on the sympathetic nerves. Following the experiments by Graham and Wolff in the 1930s that emphasized the vasodilatation concept of migraine, sympathicolytic procedures again became popular, including vessel ligation of the carotid and middle meningeal arteries. The influence of suggestion and psychological phenomena recognized at the end of the 19th century probably played an important role in many of the procedures applied. These placebo effects, generally more powerful in invasive treatments, are discussed against the background of present-day invasive treatments for headache, where they are still a matter of concern.

Historical study of coma: looking back through medical and neurological texts

In the 1960s, two major works on coma by Fisher, Plum and Poser were published and ushered in the beginning of a comprehensive clinical examination in coma. How these ideas matured has been rarely investigated. In this article, we describe observations and experiments that led to a better understanding of consciousness and coma in medical texts prior to that episode. We consulted medical texts published between 1640 and 1960. Subject indexes and tables of contents of textbooks were reviewed for the words coma, (loss of) consciousness, stupor and somnolence. Chapters on apoplexy were reviewed for descriptions of impaired consciousness. We found information on terminology, classification, causes, observation and examination, pathophysiology, treatment and experimental coma. Up to the middle of the 19th century, disorders of sense, motion and breathing, and also changes in the patients pulse, were recognized as clinical cues. The distinction between a structural and toxic (endogenous and exogenous) cause was recognized early. Observed phenomena were explained from the perspective of humoral medicine and treated likewise. After the middle of the 19th century, specialization in medicine and experimental research of intracranial pressure resulted in important insights and more accurate clinical examination. Cranial surgery and the discovery of the brainstem reticular activating system in the first half of the 20th century contributed to further increases in knowledge. The understanding, clinical examination and treatment of coma has gone through a gradual evolution over many decades. The recapitulation of clinical signs in impaired consciousness into a teachable and reproducible module marks an abrupt change in clinical approach. This transition is very recent, based on close clinical observation and interpretation of experimental and pathology studies and less on modern neuroimaging.

Publications on Peripheral Nerve Injuries during World War I: A Dramatic Increase in Knowledge.

Publications from French (Jules Tinel and Chiriachitza Athanassio-Bénisty), English (James Purves-Stewart, Arthur Henry Evans and Hartley Sidney Carter), German (Otfrid Foerster and Hermann Oppenheim) and American (Charles Harrison Frazier and Byron Stookey) physicians from both sides of the front during World War I (WWI) contributed to a dramatic increase in knowledge about peripheral nerve injuries. Silas Weir Mitchells original experience with respect to these injuries, and particularly causalgia, during the American Civil War was further expanded in Europe during WWI. Following the translation of one of his books, he was referred to mainly by French physicians. During WWI, several French books were in turn translated into English, which influenced American physicians, as was observed in the case of Byron Stookey. The establishment of neurological centres played an important role in the concentration of experience and knowledge. Several eponyms originated during this period (including the Hoffmann-Tinel sign and the Froment sign). Electrodiagnostic tools were increasingly used.

The centennial lesson of encephalitis lethargica

We commemorate the centenary of Constantin von Economos description of encephalitis lethargica, a mysterious disease that had a significant effect on 20th-century neuroscience. In the acute phase, encephalitis lethargica was marked by intractable somnolence, which von Economo attributed to lesions in the diencephalon, thereby paving the way for future efforts to localize the regulation of sleep in the subcortical brain. At the same time, neuropathologic findings in postencephalitic parkinsonism affirmed the role of the substantia nigra in the pathophysiology of parkinsonism. The occurrence of psychiatric symptoms in patients with encephalitis lethargica—such as mood disorders, obsessive-compulsive behavior, and bradyphrenia—drew attention to the organic basis of mental illness.

Fixed and dilated: the history of a classic pupil abnormality

The aim of this study was to investigate the development of ideas about the nature and mechanism of the fixed dilated pupil, paying particular attention to experimental conditions and clinical observations in the 19th century. Starting from Kochers standard review in 1901, the authors studied German, English, and French texts for historical information. Medical and neurological textbooks from the 19th and 20th centuries were reviewed to investigate when and how this information percolated through neurological and neurosurgical practices. Cooper experimented with intracranial pressure (ICP) in a dog in the 1830s, but did not mention the pupils. He described dilated pupils in clinical cases without referring to the effect of light. Bright demonstrated to have some knowledge of the pupil sign (clinical observations). Realizing the unreliability of the pupil sign, Hutchinson in 1867-1868 tried to reason in which cases trepanation would be advisable. Von Leydens 1866 animal experiments, in which he increased CSF volume by injecting protein solutions intracranially, was the first observation in which the association between fixed dilated pupils and increased ICP was established. Along with bradycardia and motor and respiratory effects, he noticed wide pupils were usually present in a comatose state. Asymmetrical dilation could not always be attributed to increased ICP, but to an oculomotor nerve lesion. Pagenstecher in 1871 extended knowledge by meticulously studying consecutive pupil phenomena with increasing pressure. In 1880, von Bergmann emphasized the significance of the ipsilateral dilation in experiments as well as in clinical cases. He distinguished the extent of pressure increase and its duration. Probably confusing irritation (epileptic head turning to the other side with pupil dilation) and lesion effects, he suggested a cortical area responsible for oculomotor phenomena, indicating what is now known as the frontal eye field. Naunyn and Schreiber (1881) understood the relationship between increased ICP with pupil dilation and decreased pulse frequency and blood pressure, warning not to decrease the latter. Concentrating on experimental traumatic effects, Duret (1878) investigated compression and commotion, in which he distinguished two phases, notably pupil constriction by bulbar lesions, due to CSF shock, followed by dilation from congestion and inflammation, due to blood around the oculomotor nerve. The key observation of a fixed dilated pupil as a sign of acute mass effect came gradually and after some localization stumbles. Following the period of extensive experimental research in ICP, the results of which were translated to clinical observations, the prognostic significance was gradually acknowledged by authors of neurological textbooks. It is well known that Cushing did similar experiments in Berne (1900-1901), and later suggested he would not have done so if he had studied the literature.

Trigeminal Neuralgia and Multiple Sclerosis: A Historical Perspective

Trigeminal neuralgia (TN) associated with multiple sclerosis (MS) was first described in Lehrbuch der Nervenkrankheiten für Ärzte und Studirende in 1894 by Hermann Oppenheim, including a pathologic description of trigeminal root entry zone demyelination. Early English-language translations in 1900 and 1904 did not so explicitly state this association compared with the German editions. The 1911 English-language translation described a more direct association. Other later descriptions were clinical with few pathologic reports, often referencing Oppenheim but citing the 1905 German or 1911 English editions of Lehrbuch. This discrepancy in part may be due to the translation differences of the original text.