Gilbert, dit Gilles (Camille) LYON
(b. 1921) was born in Mulhouse, a city of the
Haut-Rhin, an Alsatian department in France. His father had been
a lawyer and legal
counselor of the French Ministry of Foreign Affairs. His mother
belonged to an Alsatian family of
industrialists. He attended high school
in Paris.
Lyon started medical school in Paris
(which includes college in continental Europe) in 1940 and two years
later
attempted to apply for the Externat des Hôpitaux, a rotating
internship for the
best young medical students, awarded on a competitive basis.
However, he was prevented from
applying to the Externat by the "laws of exception" promulgated by
the Vichy government against those of Jewish origin. In February
1943 he crossed the Spanish
border and, upon reaching England, volunteered for the Forces
Françaises Libres
(FFL) of General Charles de Gaulle. He
spent one year at Ribbesford in the military school of the FFL. As a
lieutenant, Lyon served in the Second
Armoured Division (the famous "Seconde D.B." of General Leclerc)
until the Liberation and received the French War Cross.
Lyon
resumed his medical training after the war.
As was the custom in France for the best students, medical school was
combined with an internship (« externat ») and afterwards,
a residency program
(« internat »). Among the chiefs who had
an important impact on his training and career were Raymond Garcin at
the Hôpital
de la Salpêtrière, Julien Marie and Robert Debre at the
Hôpital des Enfants
Malades, and Stéphane Thieffry at the Hôpital des Enfants
Malades and later at
the Hôpital des Enfants Assistés (also called the
Hôpital Saint Vincent de
Paul). He graduated with a doctorate in
medicine and at the same time as a licensed pediatrician in 1955 from
the
University of Paris. It was the
tradition in France to delay the official graduation as M.D. until
completion
of both residency and specialty training.
Lyon graduated three years later than many of his high school
classmates, due to his service in the army during the war.
<> Several persons familiar with
events at that
time led me to understand that the postwar medical establishment did
not treat
former young members of the Resistance with special attention and
sometimes
"penalized" them by not taking into account for the age limit of the
"concours," the time spent in the army. His thesis was entitled
"Contribution à
l'étude des hémiplégies survenant au cours des
premiers mois de la vie"
("Contribution on the Study of Hemiplegia Following the First Months of
Life"). This early publication
displayed many of the qualities of his subsequent works: a
comprehensive examination
of the material, deliberate in thought, original, and written in a
style that
was extremely concise and clear. After
publication, he disdained any effort to advertise his own publications
in the
professional community turned his mind to his current work. The quality
of his
thesis, a model of clinical research, was recognized by the 1956 Thesis
Award
of the French National Academy of Medicine and later by publication in
the
international literature (Lyon, 1961).>
<>>
<>
>
<>From 1955
until 1962, Lyon completed his training in neuropathology and occupied
several
positions as clinical and research fellow in Paris and in Boston :
1955-57; chef de clinique (in France, this term means clinical fellow;
in the
rest of
Europe, this term means associate chief of service), Hôpital des
Enfants
Malades, Paris; 1957-58: clinical and research fellow, Massachusetts
General
Hospital, Boston; 1959-62; Chargé de Recherches (research
associate), Institut
National de la Santé et de la Recherche Médicale
(INSERM), Paris. This period was crucial for his professional
development, especially his contact and neuropathology training with
Raymond
Adams and Edward P. Richardson, Jr. His
"classmates" in the Charles Kubik Laboratory of Neuropathology at the
Massachusetts General Hospital were Karl-Erik Astrom, Elliot Mancall,
and Henry
de F. Webster.
Due to organizational
problems at that time, the once-famous French school of neuropathology
was in
disarray, and young French scholars had to go to Adams's service at
Harvard,
Ludo van Bogaert's Institute at Antwerp, Belgium, or Harry Zimmerman's
laboratory in New York to complete their training in this basic
neurological
discipline. These obligatory trips had
excellent consequences and contributed to the development of a core of
French
scientists exposed to the international
scientific community, like Lyon and his friend Jean Lapresle.
This combination of clinical training in
France with international research exposure was especially
valuable. Research in Europe had not yet recovered from
the war and was still comparatively weak, but clinical training
remained
excellent, especially in Paris.
Recruitment of patients was unlimited and directed into a very
centralized health care delivery system.
In addition, the faculty, who had previously sustained difficult
clinical competition (« les concours ») until age forty,
were not pressured to
publish superficial and repetitive papers but were allowed to
concentrate fully
on their clinical activities. Thus, the
French academic system had several weaknesses but also a number of
advantages.>
<>
>
<>After such a long period of
preparation, Lyon was appointed pediatric neurologist,
neuropathologist, and «
professeur agrégé » (associate professor)
at the Hôpital des En fants Malades and the Hôpital Saint
Vincent de
Paul. These hospitals, which belong to the Assistance Publique de
Paris, are
affiliated with the University of Paris Medical School and jointly, as
extramural facilities, with the Institut National de la Santé et
de la
Recherche Médicale. From the mid 1950s
until the mid 1970s, Gilles Lyon, Jean Aicardi, Stéphane
Thieffry, and Michel
Arthuis were associated with the same service in Paris and had almost a
complete monopoly on pediatric neurology in France. This included
the availability of both large
numbers of children with neurological disorders and fellows interested
in
training, who were predominantly from French and Spanish-speaking
countries. Further, both Lyon and
Aicardi had excellent international contacts with child neurologists in
the
English-speaking countries.>
During the
decades following World War II, the new French pediatric establishment,
founded
by Robert Debre, reorganized pediatrics and made important
contributions in
general pediatrics, public health, genetics, metabolic disorders,
social
pediatrics, and several pediatric subspecialties. Pediatric
neurology, after an initial period
of growth in Paris under Thieffry’s chairmanship, did not remain a
priority. This occurred partly because pediatrics
and neurology were separated by an "iron curtain," partly for local
personai
and organizational reasons and partly because certain powerful French
pediatricians thought pediatric neurology could be indirectly covered
with less
disruption to the pediatric establishment by a combination of
neurosurgery,
genetics, metabolic diseases, and child psychiatry. As leaders of
world pediatric neurology, Lyon
and Aicardi found it difficult to obtain funding and space to see
patients and
engage in research at a time when their clinical, tesearch, and
teaching needs
were dramatically increasing. More
recently, however, the attitude towards pediatric neurology has
improved and
the importance of this field and the need for its development are
better
appreciated.
The
Université Catholique de Louvain (UCL), with two campuses in
Belgium (the
Medical School in Brussels and the nonmedical faculties in
Louvain-la-Neuve),
is among the oldest European Universities. During the past several
decades,
this institution, located at the center of the European community, has
enjoyed
excellent support, organisation, and superb academic freedom. In
1969 1 was appointed to develop the
Pediatric Neurology Unit at the UCL, and by 1975 this service had
expanded
substantially. Transformation into an
international center with new research facilities, to allow colleagues
from
clinical pediatric neurology and the developmental neurosciences to
collaborate
was proposed by myself. At my request,
Lyon was approached by the « president » (Recteur Edouard
Massaux) of the
university and by the dean of the medical school (Michel Meulders) to
become
director and chief of service of this new center. Under my
insistance, Gilles Lyon accepted
this position, arriving in Brussels in 1976.
The service grew rapidly and now includes twenty-eight beds of acute
pediatric neurology and eighty beds of pediatric neurology
rehabilitation. With our associates, we are responsable for
sections on pediatric neurology, neuroanatomy, pediatric and
developmental
neuropathology, pediatric neuropsychology, and metabolic disorders at
the medical
school and academic hospital. There are
also programs in preventive medicine in child neurology, a section on
developmental and fetal neuropathology, and a positron emission
tomography
program oriented towards pediatric neurology.
A general overview of the clinical and research contributions of this
center reflecting the integration of clinical pediatric neurology and
neuropathology is available in a recently published textbook (Lyon and
Evrard,
1987).
It is
probably of interest when reading these biographies on the founders of
child
neurology to compare the differences in pediatric neurology in
different epochs
and continents. In this respect, one
should mention that in most European pediatric neurology services,
general care
of the patient is provided by the child neurology staff. Most of
us were trained in general pediatrics
and keep current in order to provide care for the child, infant, and
newborn. Lyon, like most child
neurologists in Western Europe, has this daily responsibility.
This type of organization of a pediatric
neurology service is burdensome but keeps us in close contact with the
patients
and their parents and provides other advantages for the development of
pediatric neurology. The appointment of
the Frenchman Lyon as chairman in a Belgian university has been
remarkably
successful and is often quoted as an example to be followed at a time
when the
European community is officially trying improve the circulation of
academic
personnel among the different universities in Western Europe.
Lyon's
major research contributions relate to the prenatal encephalopathies,
perinatal
neuropathology, viral infections of the central nervous system, and
several
clinicopathological correlations. Among his 150 publications, I shall
cite only
a few.
Lyon was among the first to demonstrate
convincingly the role played by circulatory disturbances arising in the
second
half of pregnancy as the origin of prenatal brain disorders (Lyon and
Robain,
1967). He was the first to show that
cytomegalovirus infection affects the fetal brain, particularly by
creating a
perfusion failure, not an arrest of neuronal migration (Marques-Dias et
al,
1984). His description, with Philippe
Evrard and Marc Tardieu, of the pathophysiology and management of the
progressive expanding porencephalies is widely accepted (1981).
In a seminal paper, Lyon established the
existence of several "developmental microcephalies" (Robain and Lyon,
1972). With the joint efforts of
Philippe Evrard, Verne S. Caviness, Olivier Robain, and Roger Williams,
during
the past fifteen years he has established a framework for the
pathogenetic
classification of prenatal malformations.
More recently, Jean-François Gadisseux joined this international
collaborative team. For reviews
explaining the evolution of the ideas in this field, to which Lyon has
been a
major contributor, see Evrard, Gadisseux, and Lyon, 1982; Caviness and
Williams, 1984; and Evrard et al, 1989.
In
perinatal neuropathology, Lyon first described the chronic lesions of
periventricular leukomalacla as early as 1959 in a paper for which he
was
responsible for the neuropathology (Marie, Lyon, and Bargeton,
1959). This original study has not been given
sufficient recognition because it was published only in a French
journal.
In neurovirology,
he first described chronic rubella encephalitis (Lebon and Lyon, 1974)
and the
delayed type of acute measles encephalitis in immunosuppressed children
(Lyon,
1972) and in immunocompetent children (Lyon, Ponsot, and Lebon,
1977). He contributed to the knowledge of chronic
encephalitis in X-linked hypogammaglobulinemia (Lyon, Griscelli, and
Lebon,
1972; Lyon et al, 1980). In a classic
paper with Raymond Adams and Philip Dodge, he drew attention to the
heterogeneous
group of postviral noninflammatory acute encephalopathies of infancy
(Lyon,
Dodge, and Adams, 1961). Some of these
patients, but not all, were retrospectively found to be affected with
Reye
syndrome.
Among
Lyon’s more general contributions, aside from his most recent textbook
(Lyon
and Evrard, 1987), he published with Raymond Adams the Neurology of
Hereditary
Metabolic Diseases of Children (Adams and Lyon, 1982). Alone or with
other
coauthors, Lyon contributed new information on many other
clinicopathological
problems. He first described the in vivo
diagnosis of metachromatic leukodystrophy by nerve biopsy (Thieffry and
Lyon,
1959) and the importance of peripheral nerve involvement in this
disease
(Thieffry et al, 1964). He described a
new form of sialidosis (Le Sec, Stanescu, and Lyon, 1978), and with
Thieffry,
Arthuis, and Aicardi first reported the IgA deficiency in
ataxia-telangiectasla
(Thieffry et al, 1961). He has also
contributed to the description of the congenital hypomyelinating
neuropathies. The characteristic ultrastructure of these
disorders is now frequently referred to as the "Lyon type" of onion
bulbs (Lyon, 1969; Guzzetta, Ferrière, and Lyon, 1982).
Lyon has
been a pioneer and a major contributor in the field of pediatric
neurology. He combines an outstanding
knowledge of child neurology and neuropathology. This now rarely
combined competence has given
him a strong and exceptional position.
He is an internationally recognized authority in developmental
neuropathology, the continuing importance of which is now emphasized by
all
concerned in the developmental neurosciences.
With his powerful but liberal leadership, his influence has been
effective, and he is among the creators of modern pediatric neurology
in
Western Europe.
Lyon was
one of the founders of the Société Européenne de
Neurologie Pédiatrique, a
branch of the European Federation of Child Neurology Societies.
He has been, and remains, an inspiring master
for dozens of pediatric neurologists throughout the world, who now have
great
responsibilities and internationally recognized positions. In
addition to his outstanding research
contributions, he is well versed in literature, art, and
architecture. He has an extraordinary "internationalist
charm," being one of the best symbols of French medicine for American
colleagues, a lost pearl for the French brain hunters, a "French
pediatric
neurologist drinking no wine and no Scotch" for the British, and just a
Belgian pediatric neurologist for us. Something else has made life
easier and
more meaningful for him; he has a marvelous and generous wife, trained
first in
law and afterwards in classical dance. She is a woman of culture and
sophistication, and their marriage has been a source of happiness and
affection
for both of them.
For me, as
for many other pediatric neurologists, Gilles Lyon is the best and most
generous friend, always delighted to promote and eager to delegate,
while
remaining, for the important concerns, the most solid and final
reference for
his friends, colleagues, and associates.
Philippe
Evrard (written in 1989)
REFERENCES
- Adams, R.
D., and G. Lyon. 1982. Neurology of
Hereditary Metabolic Diseases of Children.
New York: McGraw-Hill.
- Caviness,
V. S., and R. Williams. 1984. Normal and
Abnormal Development of the Brain. In
Advances in Clinical Neuropsychology.
Vol. 2, 1-62. Ed. R. E. Tarter and G. Goldstein. New
York: Plenum.
- Evrard, P.,
et al. 1989. Pathology of Prenatal
Encephalopathies. In Child Neurology and
Developmental Disabilities, 153-76.
Ed. J. French et al. Baltimore: Paul H. Brookes.
- Evrard, P.,
J. F. Gadisseux, and G. Lyon. 1982. Le
développement prénatal du système nerveux et ses
perturbations. Prog néonat 4:63-106.
- Guzzetta,
F., G. Ferrière, and G. Lyon. 1982.
Congenital Hypomyelination Polyneuropathy. Pathological Findings
Compared to
Polyneuropathies Starting Later in Life.
Brain 105:395-416.
- Lebon, R,
and G. Lyon. 1974. Non-Congenital Chronic
Rubella Encephalitis. Lancet 2:468.
- Le Sec, C.,
R. Stanescu, and G. Lyon. 1978. Un
nouveau type de sialidose avec atteinte rénale: la
néphrosialidose. Etude anatomique. Arch fran
péd 35:83044.
- Lyon, G.
1961. First Signs and Mode of Onset of Congenital Hemiplegia. Dev Med
&
Child Neur 4 :33-38.
- Lyon, G.
1969. Ultrastructural Study of a Nerve Biopsy from a Case of Early
Infantile
Chronic Neuropathy. Acta Neuropath 13 : 131-142.
- Lyon, G.
1972. Action des immunosuppresseurs sur une encéphalite virale
(rougeole) :
inhibition de la réaction inflammatoire. Comptes Rendus
Académie des Sciences
274D : 1872-78.
- Lyon G, PR
Dodge, and RD Adams. 1961. The acute encephalopathies of obscure origin
in
infants. Brain 84 : 680.
- Lyon, G.,
and P. Evrard. 1987. Neuropédiatrie. Masson Publ., Paris, 1987,
436 pages.
- Lyon, G.,
et al. 1980. Chronic Progressive
Encephalitis in Children with XLinked Hypogammaglobulinemia.
Neuroped 11:57-71.
- Lyon, G.,
C. Griscelli, and P. Lebon. 1972.
Endothelial intracisternal Tubular Inclusions in a Case of Chronic
Encephalitis Associated with Immunological Deficiency. Neuroped
3:459-69.
- Lyon, G.,
G. Ponsot, and P. Lebon. 1977. Acute
Measles Encephalitis of the Delayed Type.
Ann Neur 2:322-27.
- Lyon, G.,
and 0. Robain. 1967. Etude comparative
des encéphalopathies circulatoires prénatales et
para-natales
(hydranencéphalies, porencéphalies et
encéphalomalacies kystiques de la
substance blanche). Acta Neuropath
9:79-88.
- Marie, J.,
G. Lyon, and E. Bargeton. 1959. La sclérose
cérébrale centro-lobaire (syndrome de Little).
Presse méd 67:2286-89.
- Marques-Dias,
M. J., et al. 1984. Prenatal
Cytomegalovirus Disease and Cerebral Microgyria. Evidence for
Perfusion Failure, not
Disturbance of Histogenesis, as the Major Cause of Fetal
Cytomegalovirus
Encephalopathy. NeuroPe,d 15:18-24.
- Robain, O.,
and G. Lyon. 1972. Les microencéphalies
familiales par malformation cérébrale.
Etude anatomo-clinique. Acta
Neuropath 20:96-109.
- Tardieu,
M., P. Evrard, and G. Lyon. 1981. Progressive
Expanding Congenital Porencephalies: A Treatable Cause of Progressive
Encephalopathy. Pediatrics 68:198-202.
- Thieffry,
S., et al. 1961.
I:ataxie-télangiectasie. Rev neur
105:390-405.
- Thieffry,
S., and G. Lyon. 1959 Diagnostic d'un cas de leucodystrophie
métachromatique
(type Scholz) par la biopsie d'un nerf
périphérique. Rev neur 100:425-56.
- Thieffry, S., et al. 1964.
L’atteinte du système nerveux périphérique dans la
leucodystrophie
métachromatique. Rev neur 110:5.
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