Pediatric Neurology 53 (2015) 105e107

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Obituary

In Memoriam: Bengt Arthur Hagberg, MD, PhD August 9, 1923-April 12, 2015

“If you do not know the names, your knowledge of things will disappear as well.” Carl von Linné (Linnaeus) 1707-1778 Professor of Medicine, Uppsala University

Bengt Hagberg (Fig 1) peacefully departed this life on April 12, 2015. Regarded as the “father of child neurology” in Sweden, he was an exceptional physician and clinical scientist and a remarkable human in all aspects of life. Throughout his career replete with many successes and appropriate accolades, he remained a strong, but modest leader, always seeking the correct blend of humility and excellence at every level of life. His friendship was regarded most highly by all who knew him. Bengt, the eldest of three children, was strongly influenced by his parents, who were themselves accomplished professionalsdhis mother an artist and his father among the first radiologists in Sweden. Growing up on the west coast of Sweden, he excelled in school and in athletics, at one time winning the 100-meter track event among Swedish high school students. During summertime, he and his family frequented their grandparents’ home on the archipelago near Göteborg where he enjoyed hiking and sailing, among other adventures. Following his graduation from high school, he entered medical school in Uppsala during the height of World War II, completing his education there in 1950, and immediately began training in pediatrics under the guidance of Professor Bo Vahlquist, completing this in 1953. At that time, he successfully finished his PhD studies in pediatric hematology. However, he was most attracted to the many problematic issues related to the diagnosis and treatment of disorders of the developing nervous system and quickly immersed himself in neurology completing a further three years of training in adult neurology and internal medicine in 1956. His first academic position was in Uppsala as Assistant Professor of Pediatrics. During his neurology training, he Reprinted with permission of the Child Neurology Society, St. Paul, MN.

* Communications should be addressed to: Dr. Percy; University of Alabama at Birmingham; Birmingham, AL 35294. E-mail address: [email protected] 0887-8994/$ e see front matter Ó 2015 Elsevier Inc. All rights reserved. http://dx.doi.org/10.1016/j.pediatrneurol.2015.04.014

FIGURE 1. Bengt Arthur Hagberg, August 9, 1923-April 12, 2015.

had spent time throughout Sweden with faculty in Uppsala, Lund, Malmö, and Göteborg, but he was most influenced by the experience with Professor Sven Brandt in Copenhagen. Professor Brandt was the original and only child neurologist in Scandinavia. As such, his initial years on faculty in Uppsala represented a period of self-teaching and learning in child neurology. It was during this period that he became engaged in a range of studies on diverse subjects, from cerebral palsy and infantile hydrocephalus to an emerging interest in the progressive neurometabolic disorders,

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Obituary / Pediatric Neurology 53 (2015) 105e107

among these the sphingolipidoses including metachromatic leukodystrophy and Krabbe disease. It was at this time, too, that he formed a strong working relationship with Lars Svennerholm and Patrick Sourander at Göteborg University. These threedBengt as a child neurologist, Lars as a neurochemist, and Patrick as a neuropathologistdrepresented a formidable triumvirate of physician-scientists who advanced our understanding of a number of progressive neurometabolic and neurodevelopmental disorders throughout the second half of the last century. Progressing through the academic system, Bengt ultimately was named Professor of Pediatrics at Göteborg University in 1971, where he continued for the remainder of his career, assuming emeritus status in 1990. It was here in Göteborg that he was able to make his mark on child neurology and neuropediatrics throughout Sweden and indeed throughout the world. Among his many accomplishments, he was perhaps the proudest of the creation of a network of pediatric neurologists and neuropediatricians throughout Sweden, representing a broad system of providing excellence in care and for securing a pathway for evaluation of the most challenging problems at the major centers throughout the country. Sweden is actually a very long country extending above the Arctic Circle, and although most of its population is located in the lowest or most southern third, its population extends far to the north. Thus, this system of regionalized medical care was crucial for the efficient delivery of services to even the most remote villages in the northernmost areas. Hagberg’s continued studies in hydrocephalus and cerebral palsy, the lysosomal storage diseases including metachromatic leukodystrophy, Krabbe, the very special Finnish-dominated infantile neuronal ceroid lipofuscinosis, and, most recently, Rett syndrome (RTT), enriched the international community. His myriad successes were accomplished by his ability to create strong collaborations with many investigators, but perhaps the most important resource was his wife, Gudrun. They met as medical students, he being her instructor in anatomy and histology. They married shortly thereafter and began a family eventually reaching five children, 14 grandchildren, and 14 great-grandchildren. Their children have also been quite successful, with three daughters: an architect, a teacher, and an associate professor in Medical and Health Sciences in Göteborg; and twin sons: one a professor in infectious disease in Göteborg and the other a professor of oncology in Uppsala. Gudrun was a leading tennis competitor and it carried over to the family, especially the boys, who continue to challenge each other in this regard. Despite side-tracking her medical education, Gudrun subsequently became an expert in epidemiology and biostatistics. In this capacity, she coauthored many papers on cerebral palsy over the years and assisted many other faculty within the university with their work. Theirs was a rich marriage that combined so many wonderful times together and persisted into their retirement years. Bengt’s academic excellence was truly remarkable and brought him broad recognition both in Sweden and throughout the world. His more than 400 peer-reviewed papers covering the child neurology landscape from cerebral palsy to neurometabolic disorders reflect not only his broad vision but also his strong commitment to fostering

the careers of the members of his department. Quite in parallel, he developed broad-ranging international associations, already in the 1950s, strongly encouraging the exchange of knowledge among colleagues, particularly with the European Federation of Child Neurology Societies. Indeed, it was at the meeting of this group in 1980 that he presented his series of individuals with what he had called Vesslan disease. This created a rich and vibrant discussion, particularly from Jean Aicardi and Karin Dias (Portugal), which resulted in the first widely read English language paper on RTT (see the following section). He also developed a remarkable collaboration of exchange programs with Folker Hanefeld in Germany beginning in the 1960s; presented at conferences in the United States, Canada, and Japan; and collaborated widely in international studies on cerebral palsy. He was the recipient of numerous awards, not the least of these being the Child Neurology Society Hower Award in 1993. One of us (AP) first met Bengt in 1964 as a medical student while spending a 6-month rotation in the neurochemistry laboratory of Lars Svennerholm investigating sphingolipid deposition in metachromatic leukodystrophy. Bengt was still in Uppsala, but graciously agreed to meet there and discuss a variety of topics in child neurology. Little did I know that a mere 20 years later, Bengt’s publication on

FIGURE 2. Bengt Hagberg, MD, PhD, interacting with a patient at a Rett syndrome conference in Japan.

Obituary / Pediatric Neurology 53 (2015) 105e107

RTT in the Annals of Neurology1 would result in a close partnership as we both sought to unravel the mysteries of this unique neurodevelopmental disorder principally affecting young girls. We worked together on a number of projects related to RTT, including revising the diagnostic criteria, marshaling a series of research studies both before and after the establishment of mutations in MECP2 as the causal link, and guiding several international conferences. One particularly poignant moment occurred following an international consensus conference on diagnostic criteria in Baden Baden. It was 9/11/2001. Following the meeting, I met with Bengt and Ola Skjeldal (Norway), and they broke the news to me regarding the World Trade Center attack, a most difficult message to deliver as well as to receive. Bengt had begun recognizing RTT in 1960 before our first meeting, but it was not until decades later that it became quite clear how the early observations of Bengt and Andreas Rett were linked and how his seminal publication in Annals of Neurology firmly established the disorder as RTT. Andy Rett had made the first published observations, albeit in German, in a weekly newsletter in Vienna. Bengt had become aware of this and felt the credit properly belonged to Andy. Such was his sense of honor and decency, qualities that were valued most highly by all who knew him. Among our many collaborations, we participated in a meeting on RTT in Japan where he already had important contacts with Masaya Segawa, perhaps best-known for Segawa disease, and Yoshiko Nomura. Sadly, Masaya Segawa also recently passed away. It was there that Bengt demonstrated for all his familiar habit of examining small children while seated on a mat on the floor (Fig 2), a behavior that is well-known to all who have been to his clinical rounds in Göteborg. The other author (MK) first met Bengt when, as a medical student on pediatrics in 1969 under Professor Vahlquist at Uppsala, Bengt lectured on child neurology. His lecture was conducted in his characteristic and rapid fashion for exactly 45 minutes. His well-constructed and distinct presentation attracted many of us. In 1972, we met again, this time in

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Göteborg, and he quickly became my mentor and a good friend. His clinical demonstrations, “Hagberg’s corner,” were carried out in the wide hall of physical therapy, a ring of students and physicians surrounding an interesting young patient with parents all in their own way taking part in what became a neuropediatric seminar with a sharing of new ideas and associations. As a tutor, he was keen to find the exact presentation and to ensure precise writing style. One cherished memory was his demonstration of three RTT females from young childhood age to adulthood in different stages of the disorder. All of us worked hard and we had a great time doing it. Bengt was a remarkable human who loved all that he did. In turn, he was highly respected and appreciated, even loved, by those who worked with him and by patients and parents alike. He approached all of life with the same excitement and enthusiasm, whether it was sailing with his family on his beloved archipelago, meeting friends at home or away, or trying to enlighten us about the most complex issues of child neurology. A giant has moved on. We will never forget him for his great accomplishments and for his remarkable spirit. Reference 1. Hagberg B, Aicardi J, Dias K, Ramos O. A progressive syndrome of autism, dementia, ataxia, and loss of purposeful hand use in girls: Rett’s syndrome: report of 35 cases. Ann Neurol. 1983;14:471-479.

Alan Percy, MDa,* Mårten Kyllerman, MD, PhDb,c a Pediatrics, Neurology, Neurobiology, Genetics, and Psychology University of Alabama at Birmingham Birmingham, Alabama b

Sahlgrenska University Hospital Gothenburg, Sweden

c

Formerly of Gothenburg University Gothenburg, Sweden

In Memoriam: Bengt Arthur Hagberg, MD, PhD August 9, 1923-April 12, 2015.

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