Duane E. Haines

Kanashibari (金縛り): A Ghost’s Business

On July 1, 1976, the German student Anneliese Michel was found dead in her parents’ house, in Klingenberg-am-Main, Bavaria. She was only 24 years old and the real nature of the disease from which she had been suffering during the last eight years of her life was—and continues to be—much written about: Some people say she had been struck down by a neurologic (epilepsy: generalized or uncinate) and/or psychiatric (psychosis) disorder (Goodman, 2005; Wolff, 2006; Wegner, 2009; Duffey, 2011), while others believe she had actually been possessed by the devil (Bullinger, 1983; Siegmund, 1985; Buttner, 1986; Fortea & LeBlanc, 2010). Such a differential diagnosis is of course not a matter for us (!), but the very first scary events Anneliese experienced in mid-September 1968 perfectly illustrate the topic of this column:

Ondine’s curse: With Jean Giraudoux’s finishing touches

Monday February 12, 1883, Vendramin-Calergi Palace, Venice: In an apartment rented by Duke della Grazia, the fourth of that line, a 69-year-old man reads a book while his friend, the German painter of Russian origin Paul von Joukowsky (1813–1883), sketches a pencil drawing of him. Late that evening, the old man tells his wife Cosima (née von Bülow): “I love them, these creatures of the depths.” And he asks: “And you, aren’t you one of these creatures?” (Mistler, 1962, p. 230). The creatures (ondines) and the book (Undine) in question led two Californian physicians to coin a new term in neurological nomenclature over 50 years ago. As for the old man, he was famed composer Richard Wagner (see Fig. 1), and he would die the next day.

“Magic Mirror in my Hand, Who is the Fairest in the Land … and, Incidentally, Are You Transparent or Shining?”

May brothers Jacob Ludwig Carl Grimm (1785–1863) and Wilhelm Carl Grimm (1786–1859) forgive us for this implied reference to one of their most celebrated fairy tales! But let’s leave the Evil Queen and her Magic Mirror aside and concentrate on the septum pellucidum that was sometimes compared with—hence our title—a transparent or a shining mirror. To start with the term septum, broadly speaking it consists of two parts. The ventral part, known as the septum verum (true or real septum), is situated within the paraterminal gyrus and contains two cell masses: the lateral septal nucleus and the medial septal complex (Nieuwenhuys, Voogd, & van Juijzen, 2008, p. 363). The dorsal part, or septum pellucidum, is a “bilateral laminae of fibres, sparse grey matter and neuroglia” (Standring, 2008, p. 352), interposed between the anterior part of the lateral ventricles (medially), the basal surface of the corpus callosum (rostrally), and the rostral convexity of the fornix (caudally). The Latin word septum, or more exactly saeptum (Quicherat, 1962, p. 254), does not present any etymological ambiguity: It means “partition” or “dividing wall” (between both lateral ventricles in this case) and also applies to a great number of other anatomical structures (33 different entries in the index of the current edition of the Gray’s Anatomy: Standring, 2008, p. 1534; no less than 62 entries in Terra, 1913, pp. 491–494). According to the Danish anatomist Thomas Bartholin (1616–1680), Galen had used an equivalent term: the cerebral diaphragm (Galen as cited in Bartholin, 1677, p. 492). On the other hand, the etymology of the adjective pellucidum or perlucidum seems a little more questionable: Per may actually mean either “very” or “through,” and lucidus either “to shine” or “to be transparent” (Joubert, 1738, pp. 698, 1237, 1240; Quicherat, 1962, pp. 833, 1460, 1463; Gaffiot, 1989, pp. 412, 337). Now something transparent is a medium through which light can travel, whereas something shining emits or reflects light. It seems therefore that a structure, whatever its physical properties may be, cannot be both transparent and shining. French anatomist and medical examiner François Chaussier (1746–1828), pointing out that this septum is “presque entièrement opaque” [almost completely opaque] (Chaussier, 1807, p. 53), proposed the topographical adjective median rather than pellucid. Other

Moyamoya (もやもや): When cerebral arteries go up in smoke

In 2014, we devoted a column to the Kanashibari phenomenon (Olry & Haines, 2014). The present article takes us again to the “Land of the Rising Sun,” this time to analyze the roots of a strange Japanese term, moyamoya, coined some decades ago to refer to a hitherto undescribed cerebral vascular pathology. Initiated in 1612 by Tokugawa Ieyasu (1543–1616), the first shogun of the Edo era, the proscription of the Christians (Dickson, 1898, pp. 176–192), and especially the Jesuits (Lacouture, 1991, p. 269), was later extended to the Portuguese by his successors, Tokugawa Hidetada (1579–1632) and Tokugawa Iemitsu (1604–1651; anonymous, 1680, p. 23). From the mid-seventeenth century, the Dutch became the only Europeans to have a relationship with the Japanese. Hence, the influence of Western medical knowledge over Japanese anatomy (Aramata, 1991), medicine, and surgery (Huard et al., 1974) at that time was essentially nonexistent. Between 1853 and 1867, Japan put an end to its isolationist foreign policy (Hérail, 2009, pp. 929–984), a decision that allowed what we would call a cross-fertilization between cultures, as can be seen, for example, in medicine. Many terms of Southeast Asian origin have actually been taken up in the current Western medical terminology. We found many of these terms in two reference books: the 2017 edition of the “bestseller” of French medical dictionaries (Garnier et al., 2017), and the 2015 edition of a classic English medical treatise (Kasper et al., 2015). There were 59 terms of this kind. Most (48) originate from Japan; others came from China (6), Korea (1), Cambodia (1), Vietnam (1), Laos (1), and Malaya (1). More medical terms of Southeast Asian origin can be found in other places; our list (see Table 1) is merely a sampling to introduce the present column. Moyamoya (Fig. 1) is a unior bilateral progressive occlusive disease involving the supraclinoid segment of the internal carotid artery, and often the proximal anterior and middle cerebral arteries. Different types of moyamoya have been defined (Gosalakkal, 2002), including ethmoidal moyamoya (blood supply by branches of the ophthalmic artery), posterior basal moyamoya (blood supply by perforating branches of the posterior cerebral artery), and vault moyamoya (blood supply by transmeningeal collaterals between pial vessels and branches of the external carotid artery). Although originally believed to be restricted to Japan (Kudo, 1968), this condition has been observed in South Korea (Ikezaki et al., 1997), Europe (Yonekawa et al., 1997), and the United States (Chiu et al., 1998). However, its incidence remains higher in Japan than in other countries (Uchino et al., 2005).

The devil always experienced malicious pleasure in imposing himself in neuropsychiatric nosology

Similarly, his colleague of Hungarian origin, Thomas Stephen Szasz (1920–2012), writes that it is “a wonderfully vague concept [. . .] a disease medicine [could not] list” (Szasz, 1983, p. 12). Both deny the existence of schizophrenia itself but, in their arguments, are compelled to use the term schizophrenia. Hence, to use a term might not imply a belief in the reality to what it applies. We previously approached this paradox with an anatomical example, Reissner’s fiber (Olry & Haines, 2003), but this article centers on a much trickier problem in neuropsychiatry nomenclature. Having an inquiring mind by nature, the Devil always managed to interfere in all spheres of human activity, including the sciences. In 1831, the German mycologist Harald Othmar Lenz (1798–1870) coined the term “Satan’s bolete” (Rubroboletus satanas) to refer to a poisonous mushroom (Lenz, 1831, p. 67). Biologists use an enzyme called “luciferase” — Lucifer has been described as the “light-bearing” fallen angel, hence the bioluminescence — to spot certain proteins by chromogenous reactions (Lodish et al., 2005, p. 92). Mathematicians do not follow far behind: The “devil’s stair” is obtained by fractal partition of a rectangle (Sapoval, 2001, pp. 263–264), and “diabolical numbers” are real numbers in which the sum of the first n decimals is equal to 666 (Lignon, 2012, p. 451). Some very large numbers have, in fact, been named after other well-known “figures” of medieval demonology: Leviathan (the Leviathan number = [10]!; Pickover, 2001, p. 196), Behemoth (Behemoth numbers are any extremely large numbers; Pickover, 1995, p. 102), and Belphegor (Belphegor prime is the large palindromic prime 10[666]01; http:// googology.wikia.com/wiki/Belphegor’s_prime, consulted May 14, 2016).

Tabes dorsalis: Not, at all, “Elementary my dear Watson!”

Let us begin this column with two slices of life of the celebrated Sir Arthur Conan Doyle (1859–1930). First, he studied medicine at Edinburgh University in Scotland where he defended, in April 1885, his MD thesis devoted to the vasomotor changes in tabes dorsalis (Doyle, 1885)—hence, the first part of our title. Second, he is, of course, chiefly remembered for his creation of the “subtle, hawk-eyed amateur detective Sherlock Holmes” (Drabble, 1996, p. 292). The first work featuring Sherlock Holmes and Dr. (John H.) Watson, A Study in Scarlet, was published in 1887, followed by three novels and 56 short stories until 1927. Commonplaces are hard to get rid of: contrary to what some quite reliable references still convey (Demougin, 1992, p. 722), Sherlock Holmes never said word for word to his foil: “Elementary my dear Watson!”; this famous but apocryphal exclamation only appeared in later screen adaptations of Holmes’ adventures. Hence, the second part of our title: We would actually never describe the historical roots of the term “tabes dorsalis” as “Elementary my dear Watson!” Since our first “Neurowords Column” almost 20 years ago (Olry & Haines, 1997), this term caused us no end of trouble. Tabes dorsalis belongs, with general paresis, to the late (tertiary, the onset of symptoms usually occurring 25–30 years after infection) manifestations of parenchymatous neurosyphilis. It is histologically characterized by a demyelination of the posterior columns, posterior roots, and posterior root ganglia. Its clinical manifestations include ataxic widebased gait, paresthesia, loss of some sensations (position sense, deep-pain, thermal sense), bladder disturbances, trophic joint degeneration—the famous French neurologist Charcot’s (1825–1893) arthropathy (Charcot, 1868)—and the Argyll Robertson (1837–1909) pupil (Robertson, 1869), among others (Cabanne & Bonenfant, 1986, p. 1282; Lukehart, 2015, p. 1136).

The sleeping brain: Extenuating circumstances of the Marquis de La Fayette on October 6, 1789

During the night of October 5 to 6, 1789, a crowd of threatening people besieged the Versailles Palace. The Marquis de La Fayette (1757–1834), a major of the newly created Gardes nationales, and hence in charge of public safety, told King Louis XVI that he vouched for tranquillity of the assailants and went to bed. Some hours later, the royal family was forced to leave Versailles: The Revolution had just scored one of its most important victories, while La Fayette, as if unconcerned about the future of the French monarchy, was sleeping quietly. Antoine de Rivarol (1753–1801), the “king of polemicists” (Faÿ, 1978, p. 115), could not help jumping at the opportunity, and he nicknamed La Fayette “General Morpheus” (Rivarol, 1824, p. 300) with, of course, reference to the god of sleep. Today, many regions of the brain are known to bemore or less directly involved in the neural control of sleep, including the hypothalamic preoptic area (ventrolateral preoptic area and median preoptic nucleus: Saper, Chou, & Scammell, 2001) for the control of slow-wave sleep and the dorsal pons (sublaterodorsal nucleus: El Mansari, Sakai, & Jouvet, 1989) and dorsal midbrain (ventrolateral periaqueductal gray matter: Carlson, 2010, p. 318) for the control of rapid eye movement (REM) sleep. In this article, we will analyze the origins of some neuroanatomical terms referring to sleep, broadly speaking.

Pivotal Insights: The Contributions of Gordon Holmes (1876–1965) and Olof Larsell (1886–1964) to Our Understanding of Cerebellar Function and Structure

Among the notables who have contributed to our knowledge of cerebellar structure and function, two individuals stand out. The neurologist Gordon M. Holmes, consequent to his clinical observations on patients with cerebellar damage, especially those with injuries in WW I, provided a remarkable understanding of deficits, their laterality in relation to lesion location, and whether or not it involved cortex, nuclei, or both. He also defined, and refined, the clinical terminology describing cerebellar deficits to a level of accuracy, and especially relevance, that it is commonly used today. The anatomist Olof Larsell, in 1920, embarked on a line of investigation that would result, over 25+ years later, in a coherent and organized terminology for the lobes and lobules of the cerebellum that is widely used today and was the structural basis for numerous later experimental investigations. In this effort Larsell used a developmental approach, mapped the sequential approach of the cerebellar fissures and folia, and offered a terminology that clarified the existing, and confusing, approach that existed prior to 1920.

Hallervorden-Spatz Disease: Did One Set the Fox to Mind the Geese?

There is a recent trend in the history of medicine that considers shedding more light on the great human tragedies of WWII, particularly those related to the Nazi party. Research has also been conducted to identify the victims, especially those whose bodies were used for anatomical/pathological purposes (Hildebrandt, 2014, with a very detailed bibliography), including teaching materials: Eduard Pernkopf’s (1888–1955) atlas of anatomy has probably been the most debated example (Israel & Seidelman, 1996; Hubbard, 2001). This trend is also expressed in another way, consisting of in-depth analyses of the biographies of some physicians in the era in exquisite detail, in order to decide whether his or her name deserves — on the basis of his or her behavior and not of his or her contributions to science — to be kept or to be eliminated from medical terminology (Strous & Edelman, 2007). These so-called “tainted” eponyms (Woywodt, Lefrak, & Matteson, 2010) are the topic of this article. Fully aware of the sensitivity surrounding this subject and of the deep scar left on humankind by these historical events, we want to make clear to the reader that this article does not necessarily object to, or endorse, this trend. Our only goal is to provide food for thought from a linguistic point of view, especially as similar matters of conscience might well mushroom throughout other scientific (and even popular) vocabulary. Let’s start with a well-documented example taken from the field of neurosciences: the physician and neuroscientist Julius Hallervorden (1882–1965) and the neuropathologist Hugo Spatz (1888–1969). In 1922, these scientists described a hereditary (autosomal recessive) disorder characterized by accumulation of iron pigment in the globus pallidus and substantia nigra, leading to rigidity (beginning in the lower extremities), choreoathetoid movements, dysarthria, progressive mental deterioration, with death usually occurring before the thirtieth year (Hallervorden & Spatz, 1922). As usual in the history of medicine, this condition was subsequently referred to as Hallervorden-Spatz disease. In the following years, Hallervorden and Spatz held high positions at the Kaiser-Wilhelm Institut für Hirnforschung (Head of Neuropathology Department in 1938, and Director in 1937, respectively). Biographical studies have revealed their sympathies with the Nazi Party and (at

Trigeminal Neuralgia: Pleonasm and Miscalculation

The Bavarian physician Johann Lorenz Bausch was born in Schweinfurt on September 30, 1605. He studied medicine in Germany (Jena, Marburg), Italy (Padua) and then settled back in his native city where he died on November 17, 1665. Johann Lorenz Bausch is especially remembered for having founded on January 1, 1652, with his colleagues Johann Michael Fehr (1610–1688), Georg Balthasar Metzger (1623–1687), and Georg Balthasar Wohlfart (1607–1674), one of the very first European scientific societies, the Academia Naturae Curiosum (Hirsch, 1884–1888, vol. 1, p. 337; Keller, 1955). Johann Lorenz Bausch was probably never involved in neuroscience except by the (indirect) cause of his death: trigeminal neuralgia, the term we intend to study in this article. Trigeminal neuralgia is characterized by “excruciating paroxysms of pain in the lips, gums, cheek, or chin and, very rarely, in the distribution of the ophthalmic division of the fifth nerve” (Beal & Hauser, 2012, p. 3360). It may be primary (demyelination of large myelinated trigeminal fibers potentially related to vascular — usually the superior cerebellar artery — compression, or by frank compression without demyelination by the same vessel) or symptomatic (multiple sclerosis, intracranial tumor, or aneurysm). The very first description of trigeminal neuralgia probably happened ages ago, but ancient authors could not be rightly credited with accurate clinical descriptions of authentic cases. The characterization of headaches was too vague in the Hippocratic corpus (Hippocrates, 460–375 BC) (Lewy, 1938); Aretaeus of Cappadocia (81–ca. 138) described signs and symptoms that would more likely belong to migraine (Cole et al., 2005); and Avicenna’s (980–1037) “tortura faciei” (or “tortura oris”) was actually mistranslated by “facial torture,” while the Arabian term “lakwat” should have been understood as the negation “la” and the substantive “kuwwet” (that is “no” and “strength, power”): What Avicenna described was therefore much more probably facial paralysis (Eboli et al., 2009). Actually, historians of neurology ascribe the very first authentic case report of trigeminal neuralgia to Johannes Michael Fehr and Elias Schmidt who, in the eulogy of their colleague Johann Lorenz Bausch, published a description of his disease (trigeminal neuralgia leading to limitation of dietary intake, emaciation, and finally stroke) in 1671 (Fehr & Schmidt, 1671), hence our introductory paragraph.