Early history of the European Journal of Neurology. A mirror of the evolution of Europe after 1989.

This paper retraces the early history of the European Journal of Neurology (EJN), as it is about to enter its 30th year. It describes how our discipline organized itself during the latter part of the 20th century in Europe. In some ways, the creation and the evolution of the journal parallel the process of unification of Europe in its current form in the late 1980s and early 1990s. It started as a new journal with no impact factor and no indexation. It grew progressively thanks to the support of the European Federation of Neurological Societies (EFNS) and from the European scientific community The progressive merging of EFNS with the European Society of Neurology and the creation of the European Academy of Neurology were essential for reaching the current prominence of EJN within neurological publishing and for making it the widely heard official voice of European neurology.

Reconstructing the History of Machado-Joseph Disease.

Machado-Joseph disease (MJD), or spinocerebellar ataxia type 3, was originally described in members of the families of Machado, Thomas, and Joseph from São Miguel Island, Azores, Portugal, in 1972. The purpose of this article is to present previous descriptions of hereditary ataxia resembling the heterogeneous phenotypic intra-familiar presentation of MJD. We suggest that the condition would best be called dominant spino-pontine atrophy.

Italian neuropsychology in the second half of the twentieth century.

Since the early 1960s, human neuropsychology, the study of brain-behavior interrelations, mainly based on the analysis of their pathological variations, brought about by brain damage, has had a remarkable systematical development in Italy. All this started in Milan, with the neurologist Ennio de Renzi, and his collaborators (Luigi Vignolo, then Anna Basso, Pietro Faglioni, Hans Spinnler, François Boller, and, more autonomously, Edoardo Bisiach), in the Clinic of Nervous and Mental Diseases. Scientists of the "Milan group" investigated several neuropsychological deficits caused by focal hemispheric lesions in large series of left- and right-brain-damaged patients, and control participants, comparable for cultural and demographic variables. Standardized tests and advanced statistical methods were used, which also applied to the diagnosis and rehabilitation of aphasia. Subsequently, neuropsychology developed in Italy extensively, reaching high international reputation. Leading neuropsychologists have been the neurologists Guido Gainotti (Rome), and Franco Denes (Padua), the physicians and psychologists Luigi Pizzamiglio (Rome), and Carlo Umiltà (Parma, with fruitful interactions with the neurophysiologists Giovanni Berlucchi, Giacomo Rizzolatti, and Carlo Marzi, from the school of Giuseppe Moruzzi in Pisa) A second scientific generation of neuropsychologists has then developed in the 1970s, trained by the abovementioned scientists, further boosting and spreading high-level basic and applied research (diagnosis and rehabilitation of neuropsychological deficits of patients with brain damage or dysfunction throughout the life span, from childhood to the elderly). Available techniques include structural and functional imaging (CT, PET, SPET, MRI and fMRI Scans, DTI), electrophysiological recording (EEG, ERPs), non-invasive brain stimulation (TMS, tES), and their combined use.

Charcot's international visitors and pupils from Europe, the United States, and Russia.

The foundation by Jean-Martin Charcot (1825-1893) of the Salpêtrière School in Paris had an influential role in the development of neurology during the late-nineteenth century. The international aura of Charcot attracted neurologists from all parts of the world. We here present the most representative European, American, and Russian young physicians who learned from Charcot during their tutoring or visit in Paris or Charcot's travels outside France. These include neurologists from Great Britain and Ireland, the United States, Germany and Austria, Switzerland, Russia, Italy, Spain, Belgium and the Netherlands, Scandinavia and Finland, Poland, Bohemia, Hungary, and Romania. Particularly emblematic among the renowned foreign scientists who met and/or learned from Charcot were Charles-Edouard Brown-Séquard, who had interactions with Paris University and contributed to the early development of British and American neurological schools; John Hughlings Jackson, who was admired by Charcot and influenced French neurology similarly as Charcot did on British neurology; Silas Weir Mitchell, the pioneer in American neurology; Sigmund Freud, who was trained by Charcot to study patients with hysteria and then, back in Vienna, founded a new discipline called psychoanalysis; Aleksej Yakovlevich Kozhevnikov and almost all the founders of the Russian institutes of neurology who were instructed in Paris; and Georges Marinesco, who established the Romanian school of neurology and did major contributions thanks to his valuable relation with Charcot and French neurology.

Shining a Light on Some of the Most Famous 19th and 20th Century's Neuropsychologists.

This chapter pays homage to the masters who made neuropsychology an esteemed and legitimate field in the 19th and 20th centuries. Here we offer a brief biography for each of them and an analysis of their discoveries: Théophile Alajouanine (1890-1980), Henry Charlton Bastian (1837-1915), Arthur L. Benton (1909-2006), Julian de Ajuriaguerra (1911-1993), Ennio De Renzi (1924-2016), Norman Geschwind (1926-1984), Kurt Goldstein (1878-1965), Henry Head (1861-1940), Henry Hécaen (1912-1983), Pierre Janet (1859-1947), François Lhermitte (1921-1998), Jean Lhermitte (1877-1959), Hugo Karl Liepmann (1863-1925), Heinrich Lissauer (1861-1891), Alexander Romanovich Luria (1902-1977), Brenda Milner (1918-), Théodule Ribot (1839-1916), Charles Richet (1850-1935), Paul Sollier (1861-1933), and Carl Wernicke (1848-1905).

Helicobacter pylori, Vascular Risk Factors and Cognition in U.S. Older Adults.

Previous studies suggested that Helicobacter pylori infection could be a risk factor for stroke, dementia, and Alzheimer's disease (AD). The authors examined data from participants, 60 years old and older in the Third National Health and Nutrition Examination Survey (NHANES-III) to assess the relation between Helicobacter pylori infection and results of the Mini-Mental State Examination (n = 1860) using logistic regression analysis controlling for age, gender, race/ethnicity, education, poverty and history of medically diagnosed diabetes. Moreover, we examined performance on the digit-symbol substitution test (DSST) of 1031 participants in the 1999-2000 NHANES according to their H. pylori infection status controlling for potential confounders using multiple linear regression analyses. In 1988-1991, older adults infected with CagA strains of H. pylori had a 50% borderline statistically significant increased level of cognitive impairment, as measured by low Mini-Mental State Examination (MMSE) scores (age-education adjusted prevalence ratio: 1.5; 95% confidence interval: 1.0, 2.0). In 1999-2000, older US adults infected with H. pylori scored 2.6 fewer points in the DSST than those uninfected (mean adjusted difference: -2.6; 95% confidence interval -5.1, -0.1). The authors concluded that H. pylori infection might be a risk factor for cognitive decline in the elderly. They also found that low cobalamin and elevated homocysteine were associated with cognitive impairment.

Thomas Mann's depiction of neurosyphilis and other diseases.

Thomas Mann (1875-1955), a Nobel Prize recipient rightly considered one of the great novelists of the twentieth century, was one of the most medically perceptive writers of recent times. His novels take place against the background of the different plagues (tuberculosis, cholera) that characterized the eighteenth and nineteenth centuries. One of Mann's later novels, Doctor Faustus, is set against a background of syphilis. In the 500-page book, which is subtitled The Life of the German Composer Adrian Leverkühn as told by a Friend, we see the theologian turned composer make a pact with the devil. He "voluntarily" contracts syphilis and, as a result of the pact and despite (or because of) the disease, Leverkühn starts a brilliant 24-year career, becoming the greatest German composer of his time. While it is widely thought that Arnold Schoenberg (1874-1951) is the model for Leverkühn, we will show that other composers of the time also inspired the fictitious musician's life and works. We will also illustrate the parallel between Leverkühn's disease progression and political events in Germany in the 1930s and 1940s and their similarity with current political events.

Thomas Mann and Neurology.

Thomas Mann (1875-1955) is considered one of the most influential writers of the late nineteenth and early twentieth century. In addition to his novels and essays, he was well known for his criticisms of the Nazi party, and particularly against the racial nationalism promoted by Adolf Hitler after the First World War, as well as for his depiction of diseases. Here, we provide a quick sketch of Mann's life and his relationship with nineteenth to twentieth century German society. We then proceed to describe how Mann became interested in diseases, how he used the diseases as metaphors, and his specific contribution to the field of neurology. We describe some of the neurological cases portrayed in Mann's work, and particularly epilepsy described in The Buddenbrooks, Felix Krull and The Magic Mountain, meningitis, neurosyphilis and migraines depicted in Doctor Faustus, and essential tremor described in The Magic Mountain and Doctor Faustus. We conclude with reflections about Mann's interest in diseases and particularly in neurology.

Charles Bonnet Syndrome and Other Hallucinatory Phenomena.

Descriptions of hallucinatory phenomena have figured prominently since the beginning of recorded history. Jean Etienne Esquirol (1772-1840) is usually credited for having introduced the term in 1817, differentiating between hallucinations and illusions. Both are wrong perceptions, but in illusions, an external stimulus is always present whereas hallucinations are perceptions that occur in the absence of corresponding sensory stimuli. They occur in a variety of conditions but more often in the mentally ill, especially in schizophrenia where hallucinations, particularly auditory hallucinations represent for many, such as Henri Ey one of the cardinal features. This chapter, however, deals with visual hallucinations as found in individuals who are not necessarily mentally ill: the Charles Bonnet syndrome and autoscopy.