English Translations Of The First Clinical Reports On Narcolepsy And Cataplexy By Westphal And Gélineau In The Late 19th Century, With Commentary

Abstract

Study Objectives:

To publish the first English translations, with commentary, of the original reports describing narcolepsy and cataplexy by Westphal in German (1877) and by Gélineau in French (1880).

Methods:

A professional translation service translated the 2 reports from either German or French to English, with each translation then being slightly edited by one of the authors. All authors then provided commentary.

Results:

Both Westphal and Gélineau correctly identified and described the new clinical entities of cataplexy and narcolepsy, with recurrent, self-limited sleep attacks and/or cataplectic attacks affecting 2 otherwise healthy people. Narcolepsy was named by Gélineau (and cataplexy was named by Henneberg in 1916). The evidence in both cases is sufficiently convincing to conclude that they were likely each HLA-DQB1*0602 positive and hypocretin deficient.

Conclusions:

The original descriptions of narcolepsy and cataplexy are now available in English, allowing for extensive clinical and historical commentary.

Citations:

Schenck CH; Bassetti CL; Arnulf I et al. English translations of the first clinical reports on narcolepsy and cataplexy by Westphal and Gélineau in the late 19th century, with commentary. J Clin Sleep Med 2007;3(3):301–311

To our knowledge, there are no published English translations of the first clinical reports describing narcolepsy (in French, 1880)¹ and cataplexy (in German, 1877).² The first author herein (CHS) had a professional translation agency (Berlitz) translate these 2 reports into English, which he then edited, as described below. (A minimum of 2 language experts reviewed each translated manuscript.) These historic documents richly describe recurrent, self-limited sleep attacks and/or cataplectic attacks in 2 otherwise healthy people.

Preliminary comments on the translations are as follows: First, all punctuations and italics come from the original articles. Second, the article by Gélineau was twice as long as the article by Westphal. Third, the translations were edited slightly. Two paragraphs from the Gélineau report that had no direct bearing on the description of narcolepsy or cataplexy were eliminated from the text, as indicated, and placed in an Appendix. Fourth, the Berlitz agency translator of the Gélineau report made this comment: “The original French of this two-part article is written in an unusually loose style for late 19^th century scientific reports. It is somewhat like a slightly-edited copying of hasty notes on a physician's note pad. Accordingly, it is difficult to render in smooth English; we have in many cases sacrificed esthetics of style for accuracy.” Nevertheless, Passouant, who wrote about Gélineau for the narcolepsy centennial, mentioned that “Throughout his life, Gélineau wrote in a clear, alert, and easy-to-read style.”³ It is evident that Gélineau astutely identified and accurately named narcolepsy; he wrote an impressive set of descriptions on narcoleptic sleep attacks and their contexts, and he provided a detailed and carefully reasoned differential diagnosis and list of treatments. Westphal also correctly described the new clinical entities of cataplexy and narcolepsy. Therefore, both authors, in our opinion, deserve equal recognition for formally identifying narcolepsy and cataplexy.

(At this point the reader may wish to consider going directly to the translations of the original texts found in the final section, before returning to the commentary that immediately follows.)

GÉLINEAU'S DESCRIPTION OF NARCOLEPSY

Dr. Jean Baptiste Edouard Gélineau (1828–1906) at the outset of his report¹ attributed the initial description of narcolepsy to a Dr. Caffe who had published a case 18 years earlier, in 1862. However, a reading of Gélineau's quotes from Caffe's report would instead suggest the diagnosis of obstructive sleep apnea (OSA) as being more likely than narcolepsy. The case involved a 47-year-old man with “an irresistible and incessant propensity to sleep” that had forced him to resign from his job. He was not reported to have cataplexy, sleep paralysis, or hypnagogic/hypnopompic hallucinations. However, he was reported to have “attitude detached; stupor; mental sluggishness; persistent stoutness; effect on overall health,” and his face was “puffy.” These descriptions are more indicative of OSA than of narcolepsy. Dr. Caffe was apparently describing an overweight or obese patient when he used the word “stoutness”, particularly in the context of “persistent stoutness.” (One of the coauthors—IA—reinforced this conclusion in regards to the French word “fort” that describes a person being “overweight” distinctly more so than “strong,” both in the 19^th century and in the contemporary French language). Although various treatments did not help Dr. Caffe's patient, a stay at a spa did improve his condition. Is it possible that he lost weight at the spa, which would have had a beneficial effect on his presumed OSA?

Gélineau presents a 38-year-old man with a 2-year history of very frequent narcoleptic sleep attacks, totaling up to 200 attacks daily. This man could not speak with Dr. Gélineau for even 30 minutes without falling asleep and constantly needed his 13-year-old son at his side to keep awakening him, so that he could attend to his successful business. A wide array of intense emotional states played a prominent role in triggering his sleep attacks. The description of his initial visit with Dr. Gélineau is a dramatic example. In reading the entire report, a question could be raised as to whether this man—besides his “volatile temperament”--had histrionic personality traits that interacted with his narcolepsy.

Gélineau briefly described cataplexy (which he termed falls or “astasia”) and sleep paralysis in his patient but did not comment on the presence of sleep-onset dreaming, dream disturbance, or hypnagogic/hypnopompic hallucinations. He mentioned that his patient had “excellent night-time sleep, waking only once,” which argues against the presence of either disruptive periodic limb movements of sleep or REM sleep behavior disorder, conditions now known to be commonly associated with narcolepsy. Cataplexy was the initial manifestation of his narcolepsy. Gélineau's patient was a member of the “mutual aid society,” and his card bore the diagnosis, “morbis sacer,” Latin for “sacred disease” in reference to epilepsy, which during antiquity had been considered a divine disorder.

Gélineau's male patient reported that his infant child “was conceived in a moment when the illness came over him.” Among the various explanations to account for this intriguing comment, the most likely would be either a hypnagogic hallucination or a vivid sleep-onset REM dream, which are common events with narcolepsy that may have accounted for an imagined sexual event. Another possibility is that this man indeed had coitus with his wife while awake that was immediately followed by a sleep attack, and in retrospect he incorrectly recalled the coitus to have occurred during the sleep attack. Also, he may have experienced peri-coital cataplexy.

This patient received many unsuccessful treatments, including bromides, strychnine arsenate, curare, picrotoxin, apomorphine, phosphates, amyl nitrate vapors, hydrotherapy, electricity, and cauterization of the nape of his neck. Gélineau was thus led to comment, “as we both acknowledged that these successes were not in keeping with our mutual efforts, we lost contact, leaving to time and to nature the care of healing or improving this painful neurosis.”

WESTPHAL'S DESCRIPTION OF NARCOLEPSY-CATAPLEXY

Westphal had 2 cases that he presented at a Berlin Medical and Psychological Society meeting during 1877 that were then published in the Archives of Psychiatry and Nervous Disorders (for which he was an editor).² It is of note that he first chose to speak and write about “larvate epileptic attacks” before he described a patient with cataplectic attacks, and the publication of the former topic was twice the length of the latter topic. Westphal emphasized in italics 2 aspects of his patient's clinical history: “He did not lose consciousness during these attacks,” and “persistent night-time sleeplessness must be noted.” Westphal clearly grasped that the cataplectic attacks involved loss of muscle tone without associated loss of consciousness, and his comment about sleeplessness may have indicated the presence of disrupted nocturnal sleep that is common in narcolepsy.

In being the first investigator to describe narcolepsy with cataplexy, Westphal was also the first to describe the possible existence of familial cataplexy, as the mother of his 36-year-old male patient had also suffered from longstanding, recurrent episodes of cataplexy and/or sleep attacks that were of milder severity than her son's cataplexy, although “she had been troubled by such attacks frequently earlier on”. The observation that some of her attacks occurred when sitting quietly, while sewing or eating, is more suggestive of sleep episodes rather than cataplexy. There was no mention, however, of daytime sleepiness or any other features of the “narcolepsy tetrad.”

Westphal also described repeated sleep attacks in his patient: “At times…these attacks [viz. cataplexy] do cause the patient to fall asleep. The falling asleep appears, as it were, to be an extension or increase of the attack.” The patient would also have sleep attacks in public while “strolling around quietly and aimlessly.” These descriptions of sleep attacks and cataplectic attacks indicate that Westphal recognized and described narcolepsy with cataplexy before Gélineau, although he did not name these conditions, as did Gélineau for narcolepsy in 1880 and Henneberg⁴ for cataplexy in 1916. Whereas Gélineau described narcoleptic sleep attacks in great detail, Westphal only briefly described sleep attacks in a circumscribed manner as an extension of a cataplectic attack or as a consequence of aimless wandering. It is noteworthy that only in 1902 a third author (Löwenfeld) confirmed Westphal's and Gélineau's suggestion that narcolepsy with cataplexy represents a “disease sui generis.”⁵

THE HISTORICAL CONTEXT OF THE WESTPHAL AND GÉLINEAU REPORTS

The forceful unification of Germany by Prussia's Otto van Bismark was completed after first defeating Austria and then the French armies during the short 1870 war against Napoleon the Third. Germany was a strong but barely united country. France had lost the Alsace and the Lorraine regions and was separated from Germany both culturally and linguistically. Psychoanalysis was not formally established, as Sigmund Freud had not yet completed medical school, but there was growing interest in the unconscious and in psychological explanations for physical disorders. The pioneering work of Jean Martin Charcot's “Leçons sur les Maladies du Système Nerveux” had just been published, introducing the notion of hysteria.⁶ Neurology and psychiatry were still virtually one discipline.

ON THE AUTHORS

Karl Friedrich Otto Westphal, born in 1833 in Berlin, was the son of a well-known and wealthy physician. After a European medical education that included studies in Germany, Switzerland, and France, he joined the smallpox clinic at the Berlin Charité hospital to rise to become full professor of psychiatry in 1874. He trained a number of well-known physicians including Arnold Pick and Carl Wernicke. His achievements were numerous and included the first descriptions of agoraphobia;⁷ the first description of periodic paralysis; the report of a relationship between tabes dorsalis and general paralysis of the insane, prefiguring the syphilis connection; work on pseudosclerosis; and the first description of the deep tendon reflex. In 1887, two years after Ludwig Edinger, he described the accessory nucleus of the 3^rd nerve which bears his name. His picture is that of a well-groomed, bearded aristocratic man with a bow tie (Figure 1). Dr. Westphal died in 1890 and is not frequently credited for his report on narcolepsy-cataplexy,² which was linked to the possible forensic implications of sleep attacks.⁸

Figure 1.

Portrait of Karl Friedrich Otto Westphal (1833–1890) reproduced with permission from www.mrcophth.com/ophthalmologyhalloffame/westphal.jpg

Jean Baptiste Edouard Gélineau had quite a different career that took place outside of the medical establishment. Born in 1828 close to Bordeaux in the south of France (Blaye, Gascony), he was educated as a navy physician in Rochefort and practiced medicine on ships, studying tropical disorders on his frequent and long travels to the Indian Ocean. He spent the war as a surgeon-major and was decorated for his services.^3,9 With his large muttonchop beard (Figure 2), it is easy to imagine him with the flamboyant and proud character of people born in the country of Cyrano de Bergerac and of The Three Musketeers. Not only was Dr. Gélineau a prolific writer of medical articles and monographs, he also had a great deal of business acumen. Dr. Gélineau was known for his arsenic-bromide tablets to calm neurosis and epilepsy, was involved in coordinating a medical insurance system for older physicians, and founded a successful society of health spas and mineral waters. In 1878, he moved to Paris, to rapidly establish a successful private practice, a position he left only in 1900 to retire as a wine grower, owner of the castle of Saint-Luce-La-Tour and seller of Bordeaux wines (probably thanks to the success of his tablets). Dr. Gélineau's publications are eclectic and cover literature, the history of his native town, commercial ventures, and medical studies. His medical work includes observations on tropical diseases, postpartum psychosis, neurosis, angina pectoris, phobias, deafness, and epilepsy. He is credited for coining the term “narcolepsy” in the attached translated 1880 report,¹ and for forcefully defending it as a disease entity distinct from epilepsy.

Figure 2.

Portrait and signature of Jean Baptiste Edouard Gélineau,⁹ reproduced with permission.

Interestingly, Dr. Gélineau also published a monograph in 1880 on agoraphobia,¹⁰ citing Westphal's work on the topic (“agoraphobie des Allemands”). This indicates knowledge of the work of the German physician prior to his own 1880 article or discovered just after his Gazette des Hôpitaux publication. In 1881, Dr. Gélineau wrote a more detailed account on 14 narcolepsy cases in a monograph “De la narcolepsie,”¹¹ still not citing Westphal's 1877 narcolepsy report. A careful review of the cases reported in the monograph, however, suggests that most, if not all (except the original 1880 case) are not genuine narcolepsy-cataplexy. Whether or not Dr. Gélineau spoke German and if the 2 physicians met or corresponded is unknown, but certainly possible.

ON THE CLINICAL DESCRIPTIONS

There is no doubt that both Westphal's and Gélineau's cases have genuine narcolepsy-cataplexy. Both physicians report on the presence of sleepiness and of strange episodes of either sleep or atonia triggered by emotions, which we now call cataplexy. In both cases, onset was somewhat late in life, 34–36 years old, and abrupt, following what could be considered a psychological insult. Earlier reports of narcolepsy have been attributed to Willis (1672, in “De anima brutorum”), Schindler (1829), Bright (1836), Graves (1851), Caffé (1862), and Fischer (1878),¹² but they in fact described cases of either isolated severe, overwhelming (narcolepsy-like) sleepiness or atypical/ imprecisely described (cataplexy-like) “fits.”^12–14 In contrast, the cases described by Westphal and Gélineau are likely to be HLA-DQB1*0602 positive, hypocretin deficient cases.

A missing aspect in these reports is the lack of description of automatic behavior, abnormal dreaming, and sleep paralysis. Hypnagnogic hallucinations in particular had been described in 1848 by Alfred Maury¹⁵ and sleep paralysis by Binns in 1842¹⁶ and by Mitchell in 1876,¹⁷ but were not reported in either Gélineau's or Westphal's case. Nevertheless, the reports of Gélineau and Westphal are remarkable for their diversity and, in both cases, by the certainty of the 2 authors reporting on a new disease entity (later authors erroneously equated “narcolepsy” with every condition associated with severe daytime sleepiness¹⁸). The descriptions are tainted by their schooling and influenced by their time. Nonetheless, nothing better would be written for many years thereafter, and it could be argued that the next major discovery in narcolepsy was on the association of narcolepsy with REM sleep onset by Vogel in 1960,¹⁹ almost a century later.

In Wesphal's case, the description of the case is mostly focused on episodes of muscle weakness with persistence of consciousness, and in the discussion the author agonized at length on whether these episodes did or did not represent genuine epilepsy, and wisely summarized that it was impossible to conclude for or against this hypothesis. Westphal pointed out correctly the presence of subtle “positive” motor phenomena during cataplexy consisting of “small sporadic nostril contractions” and “slight twitching movements in the face…as were movements of the jaw.” The precise observation has been confirmed by electrophysiological recordings.²⁰ Emotional triggers are also noted but are not very well described (“mental stimulation of seeing two boys fighting in the street”; “any type of excitation”). Laughter and joking, for example, are not reported as triggers. It is in this context to note that Oppenheim, in his 1902 article on “Lachschlag” (syncope with laughing), while discussing the differential diagnosis of spells associated with laughing, did not mention narcolepsy.²¹

Sleep attacks are noted to occur “especially if not engaged in some physical activity, but is sitting quietly, talking or reading” but also “while standing” and “while walking in the street.” Sleep attacks while engaged in physical activity are indeed typical although not specific for narcolepsy. A relationship and an association of the muscle weakness episodes with sleepiness is emphasized by Westphal, and considered as an extension of the muscle weakness episodes (“at times, however, these attacks do cause the patient to fall asleep”). The German author did not completely differentiate the sleep attacks from cataplectic episodes, an ambiguity which may have reflected the simultaneous co-occurence of both symptoms in his patient (as can be observed occasionally in narcoleptics). This ambiguity may have also reflected Westphal's uncertainty about the true nature of the sleep attacks. It is of interest to note in fact that in Oppenheim's “Lehrbuch der Nervenkrankheiten,” the most important German textbook of neurology at the beginning of the 19th century, such episodes were considered to represent episodes of “psychic immobility” with muscle weakness, rather than “true” sleep attacks.²² Insomnia and the absence of any response to potassium bromate were also noted by Westphal in his report.

Interestingly, Westphal noted that the patient's mother also suffered from similar episodes, following a head trauma. In this case, however, it is difficult to make a conclusion as maintenance of postural tone is reported, and the episodes may be more reminiscent of absence seizures. Similarly, in the case of Fisher (1878),¹² i.e. a younger case where cataplexy is not described with certainty, a sister is suggested to have had the same condition, to later outgrow it during adulthood.

Further discussion of Wesphal's cases also attest to the rise of “pre-psychoanalytic” ideas, already evident in Westphal's prior studies on “sexual inversion” and homosexuality.²³ Detailed reference to and discussion of the case of Van Zastrow, a famous criminal pedophile evaluated by the author in prison, is made. Contrary to what was generally believed in this time, the author was surprised not to find the criminal epileptic (epilepsy was frequently considered at the time a sign of “mental degeneration”), but rather excessively sleepy, falling frequently asleep in public (the symptom was severe enough that people were laughing about it). A relationship between his sleepiness and his alleged frequent masturbation, repressed homosexuality, and associated shame is suggested, with the prisoner attributing his sleepiness to the “secret vice of masturbation” to which he had become “addicted.” Whether Mr. Van Zastrow had obstructive sleep apnea or Kleine-Levin syndrome is impossible to reconstitute, but narcolepsy is not likely.

Dr. Gélineau's report is complementary to Westphal's. Its style is more descriptive, “story telling.” A potential head trauma 2 years prior to onset is reported as a possible contributing factor. Whereas Westphal was more interested in the loss of muscle tone and the sleep attacks (as reflected by the title of his communication), Gélineau was fascinated by sleep attacks during active tasks such as eating and by the existence of refreshing short naps. Cataplexy is confused with sleep attacks, but its triggers are very well described, i.e., playing cards (and having a good hand), smiling at someone poorly dressed in the street, being surprised by a sudden danger, and anticipating the pleasure of a good play in the theater. Most telling is the story of this patient going to the zoo of the Jardin des Plantes and “falling asleep” in front of the monkey's cage when everyone was laughing around him. The patient had up to 200 episodes per day, suggesting a form of “status cataplectitus.”

Although Gélineau did not formally distinguish cataplexy from sleep attacks, it is of interest to note that in naming narcolepsy he identified “narcolepsy's twofold analogy with drowsiness and catalepsy.” Catalepsy, however, involves prolonged maintenance of body posture, which is a form of motor dyscontrol opposite to cataplexy. Gélineau's use of the word catalepsy may refer to the patient maintaining posture during some sleep attacks, which were not cataplectic attacks. Nevertheless, his use of the term “astasia”, viz. falling episodes, in relation to narcolepsy prefigured the naming of cataplexy in 1916 by Henneberg, who clearly differentiated sleep attacks from episodes of muscle paralysis triggered by emotions.

A second article follows the initial report where Gélineau excludes potential differential diagnoses including vertigo, epilepsy, agoraphobia, anxiety, meningitis, and sleeping sickness, and to conclude that narcolepsy is a unique disease entity. As mentioned above, Gélineau also wrote a monograph reporting on 13 additional cases, none of whom is likely to have genuine narcolepsy. Gélineau described how decreased brain tissue oxygenation and metabolism in the pons, the “site of emotional regulation and dreams” could occur in selected predisposed patients or was caused, in 2 patients, by too much sex (“Venus' pleasures”). Decreased oxygenation would be precipitated by emotions, considered as consuming too much oxygen and energy. Gélineau also reports on numerous therapeutic attempts. Therapies aimed at relieving a potential vasomotor abnormality, including picrotoxin and amyl nitrate to induce vasodilation, were tried without success. Further trials with apomorphine had no efficacy. Interestingly, he tried to give strychnine (which is now known to block postsynaptic glycinergic transmission, in particular at the spinal motor neuron where it could antagonize REM sleep-induced atonia), but obtained only a transitory effect. Dr. Gélineau finally suggested caffeine to treat the narcoleptic sleepiness (as originally suggested in 1672 by Willis²⁴), despite the fact it was of little benefit in his only genuine case. A more potent treatment than caffeine, i.e., ephedrine sulfate, was suggested by Janota and Daniels about 50 years later.²⁴

Gélineau considered in his monograph that the sleep of narcoleptic patients was deep and devoid of dreams, which suggests, as emphasized below, that the 13 other cases were probably not narcoleptic. Importantly however, he introduced the still-valid notion of a duality in narcolepsy, that of sleepiness and falls (also called astasia).