Désiré-Magloire Bourneville
Tuberous sclerosis complex, composed of the Latin tuber (swelling) and the Greek skleros (hard), refers to the pathological finding of thick, firm and pale gyri, called “tubers,” in the brains of patients postmortem. These tubers were first described by Désiré-Magloire Bourneville in 1880; the cortical manifestations may sometimes still be known by the eponym Bourneville's disease 1).
The first case of tuberous sclerosis that Bourneville attended was a 15-year-old girl named Marie. Marie had severe epilepsy, neurodevelopmental delay, and a papular eruption on her nose, cheeks and forehead. She had presented with seizures since infancy, had learning difficulties, and she could neither walk nor talk. She was treated with multiple strategies including: quinine, bromide of camphor, leeches behind the ears, and amyl nitrite. None of them seemed to work and her seizures became progressively worse. At the age of three, she was declared “a hopeless case”. Marie died in May 1879, and a postmortem study revealed dense tubers in the cerebral gyri, henceforth known as sclérose tubéreuse des circonvolutions cérébrales, determining them to be the cause of the seizures. In addition to these findings, white-colored hard masses “the size of a walnut” were found in both kidneys. Not long afterwards, in 1908, Heinrich Vogt (1875-1957) described the classic triad, that included epilepsy, mental retardation, and sebaceous adenomas 2). His work was based on the study of epilepsy and idiocy, and he was also an advocate of public health and social medicine education; creating day hospital programs for children with this type of neurologic disease 3).
The condition was also studied by the British dermatologist, John James Pringle (1855-1922), leading some historical texts to refer to it as “Bourneville-Pringle disease”.
Bourneville was skeptical of mystical and supernatural claims. Between 1882 and 1902, he published a series of volumes known as La Bibliothèque Diabolique, in these he re-evaluated historical cases of possession and witchcraft in favor for pathological explanations
Désiré Bourneville was one of Jean-Martin Charcot's most important disciples. His previous works as an alienist allowed him to influence his master's interest in hysteria, which led to the creation of a service regarded as a neurological mecca. During his time under Charcot, Bourneville, a passionate left-wing radical, had to coexist with characters representative of the conservative, bourgeois Parisian society 4).
Louis Delasiauve developed a novel specific form of pedagogy to deal with delays in cognitive development. This made him one of the initiators of institutional pediatric psychiatry. His ideas would be carried forward by his favorite student, Désiré-Magloire Bourneville (1840-1909). Committed to social welfare, Delasiauve worked relentlessly to improve access to healthcare for the least fortunate throughout France 5).
Désiré-Magloire Bourneville at the Hospital Bicêtre in Paris, based on his observations of children and adolescents who had been labeled “abnormal” and placed in medical and educational institutions. In the early twentieth century, elaborating on the observations of Bourneville, Jean Phillipe and Georges Paul-Boncour showed the presence of a subgroup of “unstable” children who suffered from a disease entity in its own right within the population of “abnormal” schoolchildren (the terminology of the time). This new pathological entity included symptoms of hyperactivity, impulsivity and inattention, corresponding to today's classic triad of ADHD symptoms. While noting the lack of behavioral inhibition, clinical descriptions of Bourneville, Philip and Paul-Boncour also considered the notion of “moral disorder” which at that time played an important role in psychopathology. This resulted in some degree of confusion between impulsive symptoms and major behavioral disturbances often associated with ADHD 6)
He studied medicine in Paris, and worked as interne des hôpitaux at the Salpêtrière, Bicêtre, Hôpital Saint-Louis and the Pitié. During the Franco-Prussian War, he served as both a surgeon and an assistant medical officer. From 1879 to 1905 he was a physician of pediatric services at Bicêtre. In Paris, he founded a day school for special instruction of children with mental disability.
In 1866, during a severe cholera epidemic in Amiens, he volunteered his services, and after the siege had passed, was presented with a gold watch as an expression of the city's gratitude. During the Paris Commune (1871), when revolutionaries wanted to execute their wounded enemies, Bourneville intervened and saved the prisoners' lives.
He was elected to the Paris city council in 1876 and to the French Parliament in 1883, where he served as a deputy until 1889. In both positions he advocated reforms of the health system. As a politician, he spearheaded efforts to train professional, secular nurses to replace the religious sisters who staffed most of the nation's hospitals at the time 7).