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|The History of Sturge-Weber Syndrome|
Dr. William Allen Sturge (1850-1919)
William Allen Sturge was born in 1850 in Bristol , England , the eldest son of William and Charlotte Sturge. William's parents were members of the Religious Society of Friends (Quakers). His father was a wealthy land surveyor.
He attended local schools until 1865 when he was sent to a Quaker school in London . There, while playing soccer, he injured his knees. He stayed with his uncle while he was recuperating from the knee injury. His uncle, a physician, had sons studying medicine and it was during this stay that William developed an interest in medicine. This was contrary to his father's wishes; who wanted his son to take up the family business. Eventually, William's father relented and allowed him to pursue his interest in medicine.
He entered Bristol Medical School in 1868 and during this time he did practical work at Bristol General Hospital . He was a very industrious young man who, in the pursuit of his interests, neglected his own health. He fell ill of diphtheria in June, 1869. In August, after recovering, William went on vacation in Switzerland with his family. While there he developed rheumatic fever.
After recovering, he resumed his studies and passed the Primary Examination of the College of Surgeons in 1870. He continued his studies at University College in London in 1871. He had a second attack of rheumatic fever in 1872 which forced him to take a prolonged rest. His father sent him on a tour in the East in 1873. As a medical attendant, William accompanied a wealthy young man named Lukas to Egypt. After returning from Egypt he resumed his medical studies and completed his M.D.in 1875 in London.
He became a resident Medical Officer and subsequently Registrar of the National Hospital for Paralysis and Epilepsy. It was there that he laid the foundation of a wide and thoughtful survey of neurological diseases. The years 1876 and 1877 were spent in Paris on his post-graduate studies. During this period, neurologists were attracted to the work of Charcot in a clinic in Salpetriere.
William did not limit his studies to neurology. He worked in general pathology and in medicine and followed closely the practice of Fornier. Both Charcot and Fornier were highly impressed with William's intelligence and originality. It was in Paris that he met his wife, Emily Bovell, who was also a physician. They married in September 1877 and returned to London to set up a practice together. He was appointed physician and pathologist to the Royal Free Hospital, and a lecturer to the Women's Medical School.
It may be of interest to note that Emily Bovell was one of the original half dozen women who gained admission to the Medical School of Edinburgh University, only to be physically ejected by the male students and faculty. All of these women eventually completed their medical training elsewhere and all achieved distinction in their own particular field. Emily was older than William.
She developed tuberculosis, a circumstance that prompted the couple to move to Nice in France (French Riviera) in order to live in a milder climate. There they set up practice treating the wealthy and famous English and American visitors. During this time he took medical care of Queen Victoria and her family. In recognition of this service, Queen Victoria awarded him gifts and an MVO, an order and decoration reserved for those who have rendered service to the Royal Family of a personal nature. William stayed in Nice for 27 years. Emily Bovell died in her early 40's in 1885.
The following year William married Julia Sherriff, who was his nurse in Nice. Julia was the daughter of a wealthy ironmaster in the north of England . Because of the summer heat in Nice, the couple used to take their vacations at this time of the year. William was very fond of traveling. It was during his travels that he became greatly interested in archaeology and began to collect Greek vases and Paleolithic and Neolithic flint implements. After his retirement in 1907, William returned to England and settled at Icklingham Hall, Suffolk , and area that contained many Neolithic flints which satisfied his passion for archaeology.
In the winter of 1918 he contracted influenza and then nephritis and subsequently died in March 1919. William left no children to bear his name.
In 1879, Dr. Sturge described the case of a patient whom he originally saw at the age of 6 ½ years. This child had enjoyed good health until she was 6 months old. At this age she started to have twitching on left side of her body. Later the attacks became stronger but the child would not lose consciousness. With time the twitching started to spread to the other side and she would lose consciousness. She benefited from potassium bromide. Of particular interest was that the child had what was described as a "mother’s mark" on the right side of the head and face. The skin lesion was accurately demarcated in the midline and involved the upper lip, nose, forehead, scalp, and back of the neck extending a little beyond the midline on the chin and on the upper part of the sternum. It extended as low as the third or fourth dorsal vertebra behind and the second costal cartilage in front. The lips, gums, tongue, roof of mouth, floor of mouth, uvula, and pharynx were all similarly affected, to a greater or less extent, on the right side. The right eye was larger (buphthalmos; congenital glaucoma) and the sclera, choroid and retina were all affected by a vascular malformation. In addition there was a patch about the size of the palm of the hand over the left eye, frontal and temporal regions.
The mark was of a deep purple color, the color partially disappearing on firm pressure. The affected parts were distinctly larger than the corresponding parts on the other side. This mark was termed a "port-wine" by him. Dr. Sturge had no proof (for which he was greatly criticized by his peers when he presented his case to the Clinical Society in London in April 18, 1879 ) but he postulated that the patient’s neurological deficit was explained by a lesion that existed on the surface of the same side of the brain.
The reason he did not suspect that the lesion could have arisen from the brain parenchyma itself was that, in his view, if there had been a parenchymal. lesion to begin with the patient would have started with generalized seizures from the beginning since the immature brain has great "instability of grey matter" and thus allows for widespread dissemination of the "nerve-discharge".
It is a great tribute to Dr. Sturge that he had such an incisive intellect to correctly postulate the underlying basis of the patient’s disease. It was not until 1901 that S. Kalischer provided a pathological proof of such an association. The radiographic findings of such a condition were first described by F. Parkes Weber of England in 1922 and then by V. Dimitri of Argentina in 1923. It should be noted that Dr. Sturge contributed greatly toward the understanding of muscular diseases in recognition of which he was awarded a silver medal by the Royal Society of Medicine for his dissertation on Spinal Muscular Atrophy.
It is of great interest that Drs. Sturge and Weber had common interest not only in medicine but also in extra-curricular pursuits. Both had immense interest in antiquities. It was during his sojourn in the South of France that Dr. Sturge started a collection of Greek Amphora that are now housed in the Toronto Museum ( Canada ) as the Sturge Collection. After his return to England , he lived in Suffolk where he amassed an impressive collection of early prehistoric flint implements that are now housed in the British Museum.
Dr. Frederick Parkes Weber(1863-1962)
Frederick Parkes Weber was born in May, 1863, in London. His father, Sir Hermann David Weber, (1823 – 1918) had come to England from Germany and served as physician to Queen Victoria. The middle name "Parkes" was derived from his father's great friend, Sir Edmund Parkes and in time this forename became coupled with his surname, so that he was generally known as "Parkes Weber". He was educated at Charterhouse School, Cambridge University and studied medicine at St. Bartholomew's Hospital, London, as well as in Cambridge, Paris, and Vienna.
Weber and his family retained their German connections, emphasized by his persistent pronunciation of his surname with the continental “V” sound, now long forgotten since his death.
Weber obtained his doctorate at Cambridge in 1892 and held resident posts at St. Bartholomew's Hospital as House Surgeon and House Physician, and at the Brompton Hospital for Chest Diseases as house physician, before being appointed as honorary physician to the German Hospital , Queen Square , London , in 1894. In this capacity he carried on with his duties until he reached his 80th year. He was also physician at the North London Hospital for Consumption. From 1899 to 1911 he was at the Mount Vernons Hospital for Chest Diseases.
In 1921 Parkes-Weber was the first Mitchell Lecturer at the Royal College of Physicians. In 1959 he founded his own triennial prize and medal to promote advances in dermatology.
Weber was legendary in the Royal Society of Medicine for his remarkable knowledge of rare disorders, for his clear and meticulous diction, and for his prolific contributions of more than 1200 medical articles over a span of 50 years of active medical practice. He was a prodigious describer of new or unique entities, many of them dermatologic, and his name has been attached to several disorders, including Klippel-Trenanay-Weber syndrome, Hutchinson-Weber-Peutz-Jaghers syndrome, Osler- Rendu-Weber syndrome, Pfeifer-Weber-Christian disease and Weber-Cockayne syndrome as well as Sturge-Weber syndrome.
In 1922, Weber reported the first radiologic features of brain “atrophy” in the Sturge-Weber syndrome, but did not mention the now classic intracranial calcifications. In a later account of the same patient, however, he amended the description with a much better film of the skull.
This erudite and remarkable man lived to a great age, narrowly missing his century. He continued to attend the Section meetings at the Royal Society of Medicine into his ninetieth year - this in spite of deafness, failing vision and unsteadiness of gait. He was ably supported by his devoted wife who acted as nurse, secretary, stabilizer and general factotum. He would often contribute to the discussions, quietly yet revealing in a modest way his astonishing erudition. He had the kindliest disposition and when giving a second opinion would never let the referring physician lose face over a faulty diagnosis, so subtle would be his remarks and so gentle his correction. Mention of a relevant reference would cunningly give the game away.
Like his father he was a keen alpinist and collector of coins and vases, which towards the end of his life he donated to museums. Among his many fascinating articles was one on death in the arts, 1910, and he also wrote books on the philosophy of medicine.
Geoffrey Dowling in 1962 wrote - "For about as long as any living physician is able to remember, it was customary to consult Parkes-Weber on any case of exceptional rarity or obscurity. He was the kindest consultant imaginable but had sometimes the fault of crediting his colleagues with more knowledge than they possessed".
He died in 1962.