Brain & Development 20 (1998) 543-546
Around the world
Andrey Petroukhin
Child Neurology Department. Russian Slate Medical University 7 Pozfuirskv pvreulok, 119034 Moscow, Russia Received 1 May 1998: accepted 7 May 1998
The child is the future of society because he is the parent of the children of tomorrow. However, children are also the products of our societies. This is why the United Nations promulgated in 1989 the International Convention on the Rights of Children. Health-care for children, and especially child neurology, plays a very important role in promoting their harmonious development. According to the data of World Health Organisation, every 20th child has some disturbances in his development, requiring special medical and educational measures.
Child neurology in Russia has long lasting history and iraditions, dating back to the end of the 19th century. The outstanding neurologists V.M. Bekhterev, A.Y. Kojevnikov, V.K. Rot, S.I. Rossolimo, V.A. Muratov, L.S. Minor, and others, made their contribution to the foundation of child neurology in Russia. Some famous pediatricians, including N.F. Filatov and others, also promoted the development of child neurology. It is difficult to overestimate the contribution of the world-famous psychologists and physiologists L.S. Vygotsky, A.R. Luria, P.K. Anokhin, I.A. Arshavsky. A.A. Volokhov, and others, to the research of nervous-system functioning in children and higher cortical functions development in ontogenesis.
At the end of the last century, neurology experienced an early period of accumulation of separate facts and observations and description of the separate forms of nervous diseases. It was found that the main forms of nervous-system diseases also affected children, and some illnesses were linked mainly to childhood. The epidemics of poliomyelitis and encephalitis in Russia affected many children, often leaving irreversible neurological deficits. It became obvious that nervous-system diseases in children require closer attention by virtue of their peculiarities and consequences.
There were two large neurological schools in Russia by that time, in Moscow and St. Petersburg. The Moscow Neurological Center was organized under Alexey Kojevnikov's (1836-1902) management; V.K. Rot, G.I. Rossolimo, V.A. Muratov, L.S. Minor and others were among its representatives. Kojevnikov graduated from the Medical Faculty of Moscow University - where nervous-system diseases in children constituted a large part of a special course on child diseases - in 1858, and began to work in obstetric clinic. Then he worked as an assistant in a therapeutic clinic, where, in 1866, he defended the doctoral thesis on his clinical and anatomical investigations on Duchenne disease and received the Doctor of Science degree. In 1869, Kojevnikov became the director of the specialized neurological clinic, which was the first not only in Russia, but in the world. A new clinical campus, which was attached to the Moscow University and consisted of a psychiatric clinic, a clinic for nervous diseases, a shelter for chronic patients and a neurological museum, was arranged under his management. Kojevnikov was interested in clinics and pathomorphology of poliomyelitis and expressed his opinion about its inflammatory nature in 1883. In 1894. Kojevnikov distinguished a special form of epilepsy, characterized by constant clonic convulsions in the hemiplegic hand and leg. They were observed in patients without any progression, but periodically transformed into extended epileptic fits. This form of epilepsy was named 'epilepsia partialis continual Kojevnikov considered encephalitis to be its cause.
Grigory Rossolimo (1860-1928) from 1890 to 1911 managed the neurology department of Novo-Ekaterininsky Hospital, simultaneously being the Assistant Professor of Moscow University. In 1917, Rossolimo was elected Head of the Neurology Department and Director of the Neurology Institute in Moscow University. His scientific investigations were devoted to nervous-system anatomy and physiology, clinical and surgical treatment of nervous diseases, child psychoneurology, and medical psychology. Rossolimo studied thoroughly a special form of relapsing hypertrophic polyneuritis in children (1899) which was given his name. Rossolimo was among the founders of child psychoneurology. He was especially interested in diseases of nervous system in childhood, education and peculiarities of mental activity of children, psychotherapy for sick children, disturbances of intellectual development in children with neurological diseases. Feeling the necessity of more detailed study of manifestations and approaches to treatment of neurological diseases in children, in 1911 Rossolimo organized, using his own funds, the Research Institute of Child Neurology and Psychology, which became the first child neurological clinic in Europe. Thus, the historical date of child neurology birth in Russia can be considered to be 1911. In 1918, Rossolimo opened another division for children in neurological clinic. The first child neurologists in Russia were educated under his supervision.
The outstanding pediatrician. the founder of Russian pediatrics, Nil Filatov (1847-1902), realizing the importance of problems in child nervous-system physiology and pathology, involved Moscow neurologists in the daily clinical and educational work of the Child Doctor's Scientific Society. This cooperation was very fruitful. The accumulated experience on study of nervous-system pathology in children formed the basis for the development of important points of semiotics, and clinics of nervous disorders in children. This experience was presented by Filatov in his book 'The semiotics of Child Diseases' (1890) in which the descriptions of palsy, ataxia, meningitis, chorea and hydrocephalus occupied the significant place. Filatov was tirst in Russia in popularisation and introducing into routine practice the method of lumbar puncture.
In St. Petersburg the course of nervous diseases was lectured on from 1857 by the Chair of Psychiatry, managed by professor I.M. Balinsky (1827-1902). His disciple, and successor of the chair, I.P. Merjeevsky taught nervous diseases even more systematically. He created a remarkable school of Russian neurologists, which world-famous representative was Vladimir Bekhterev (1857-1927).
Bekhterev created a number of new laboratories (pathological, experimental physiological) inside the clinic and organized the hospital for patients with nervous diseases in 1898. There was an operation room at the clinic where surgical methods were applied to the treatment of nervous diseases. The first Neurosurgical Department in Russia, and in the world, was opened at the hospital in 1907. In 1897, Bekhterev received the Chair of Nervous and Mental disorders in the Women's Medical University, where he supervised until 1913. The idea of the association of the different fields of neurological science into a united scientific complex led Bekhterev to the creation of the Psychoneurological Institute in 1907. Bekhterev also made a great contribution to the development of child neurology and psychology, by the foundation of the Child Research Institute, the Medical and Pedagogic Institute for children with antisocial bents, the Otophonetical Institute for the study of speech and hearing disorders in children and the Central Auxiliary School for mentally-retarded children, having offered a number of methods for investigation of child psychological development.
After World War II, scientific researches in child neurology concentrated on the most urgent infectious diseases of the time: poliomyelitis, enteroviral meningitis end encephalitis and tuberculous meningitis. Advances in the treatment of these diseases were closely connected to achievements in pathological, physiological, biochemical and immunological studies. The efforts of Russian scientists and physicians resulted in 1955 in the introduction of the efficient system of vaccination prophylaxis. Very soon, poliomyelitis disappeared as an epidemic disease and the frequency of other acute infectious diseases significantly decreased. The problem of perinatal neurological complications was put forward that time as one of the most important in Russian child neurology. It became apparent that neurological evaluation of infants is of great importance in their overall assessment. P.S. Babkin described the palmar-oral reflex, which is everywhere included in neonate neurological examination:pressing the neonate's palm causes the mouth to open, the head to turn sideward, and the eyes to close. In 1962, the first specialized neurological department for newboms was opened in D.S. Futer's clinic. In 1976, Y.A. Yakunin et al. developed the clinical classification ofCNS damage in newborns and infants, which is still used, with some modifications.
At this time, the problem of hereditary diseases of the nervous system came to be considered a new challenge, due to achievements in cytogenetics. The founder of clinical neurogenetics in Russia was Sergey Davidenkov (1880-1961). Davidenkov was one of the first to indicate the genetic heterogeneity and polymorphisms of hereditary diseases, and formulated a concept of the anticipation phenomenon. Davidenkov described a new form of progressive scapuloperoneal muscular atrophy (1928). He was the first to propose the compilation of a systematic catalogue of hereditary diseases in man, but this brilliant idea could not be realized in the Soviet Union in the 50s because of political suppression of genetic studies.
Child neurology became an independent medical discipline in Russia in 1963 when the first chair of child neurology was organized by Levon Badalian (1929-1994) at the pediatric faculty of the Russian State Medical University. Levon Badalian developed the best traditions of Russian child neurology. He worked out the problems of developmental neurology, perinatal damages of the nervous system, neurological manifestations in heart disease, neurogenetics, neurophysiology and many others. His clinical and genetic studies of hereditary disorders of the nervous system resulted in creation their classification and registration in the Soviet Union, included more than 1000 nosological forms. Due to his significant contribution perinatal neurology was developed as a special field of medical science. The study was started of psychological functions development in normal and neurologically-sick children and many peculiarities and pathogenic mechanisms were revealed at different stages of development. In 1975 Badalian issued the first textbook on child neurology in the Soviet Union for students of medical universities. Several generations of child neurologists, researchers and teachers in child neurology weregraduated from the chair of Levon Badalian, who are actively working now not only in Russia, but also in many other countries.
Recently the Health Ministry of Russian Federation had analyzed current state and problems of the neurological care for children in all 89 administrative regions of the country. According to recent reports, the number of child neurologists in Russia is more, than 2000 (for a child population, up to 14 years of age, of 31 400000, according to the demographic data of 1996, which is 21 % of the overall population of the Russian Federation).
The prevalence of neurological diseases among the children in Russia is about 30 cases per 1000. The most common causes of neurological morbidity are the following:
Prenatal and perinatal damages, including CNS malformations: 36%
Cerebral palsy: 24%
Epilepsy and seizure disorders, caused by early neonatal damage: 19%
Infections of the nervous system: 10%
Hereditary diseases of nervous and neuromuscular systems: 4.8%
Tumors of the CNS and vascular malformations: 3%
In total, neurological diseases represent the most common cause of disablement of children in Russia - almost 60%.
The system of neurological care for children has a differentiated and staged structure. This is provided by health-care institutions (in-patient and out-patient services, specialized medical centers) and by social and educational institutions. At present, there are 9000 places for neurologically-ill children in hospitals (as well as places in the newborn and infants units).
The highest weight in the structure of neurological morbidity among the children in Russia belongs to CNS prenatal and perinatal damage (36%) and residual neurological deficits due to this damage (24%). This is why the most important early stage of child neurological care is represented by the maternity homes. This stage can be regarded as a prophylactic one, because the severity of neurological disorders and the degree of further disability depend on timely and rational care of newboms. In the maternity homes, there are intensive care units for children with neurological complications of the perinatal period and inherited diseases. Depending on the severity of the neurological disturbances, newboms are transferred from maternity homes either to neonatal centers or to neurological departments for new-borns and babies in the child hospitals. Complex investigations are performed there, aimed at revealing the main neurological syndromes and associated disorders, which is important for the early diagnosis of diseases of different lorms, and the treatment and training of age-appropriate motor, speech and mental functions. In these hospitals, parents are taught the main skills of training and rehabilitation for training at home. Later on rehabilitation is continued in the local out-patient departments and half-day hospitals.
Specialized child neurological clinics are functioning in half of the Russian Federation administrative regions and in many others, special places for neurologically-ill children are reserved in general hospitals. Twenty-seven administrative regions possess Consulting Child Neurological Centers. providing neurological out-patient care. Most of them have all the necessary diagnostic facilities and modem equipment. In the latter administrative regions, neurological assistance is provided by local child polyclinics.
Specialized medical centers are arranged for neurosurgical treatment of CNS tumors, malformations or injuries, craniofacial surgery, rehabilitation of patients with cerebral palsy or other motor disorders and problems of speech, vision and hearing. These centers are working in at least eight of the biggest cities, with populations greater than 500000.
The sphere of epileptology is shared in Russia between neurologists and psychiatrists. Consequently, a unified system of registration and treatment is lacking. Now, some specialized centers for children with epilepsy are being arranged, as epilepsy and seizure disorders constitute 19% of neurological morbidity among children in Russia. The problem of epilepsy is first of all the problem of definite diagnosis, and efficiency of epilepsy treatment depends on its precise diagnosis. Epileptic-patient assessment and treatment in Russia is faced with many difficulties, in particular inadequate provision with modern antiepileptic drugs (only 16% of patients are able to get the necessary drugs in the required amounts) and insufficient technical equipment for neurophysiological investigations and neurosurgical treatment. Much effort is now being undertaken to improve this situation.
Rehabilitation services are arranged in 27 administrative regions, mostly in the central regional child hospitals. Besides that, for children with neurological disorders, 26 regions have sanatoria and so-called 'forest schools,' and 29 regions have specialized kindergartens. Together with special treatment, children are included here into cultural, sporting and social activities and thus are better adapted to their life in society. Climate and natural factors are widely used in the sanatoria, situated in the resort areas (Black Sea coast. Northern Caucasus, etc.).
Much attention is paid to genetic counseling, which is performed in the centers of medical genetics. The three biggest research centers of medical genetics, in Moscow, St. Petersburg and Tomsk, are preoccupied with DNA analysis in a number of nervous-system hereditary diseases for precise diagnosis, carrier-detection and prenatal diagnosis. However, the prevalence of hereditary diseases in Russia (4.8%) is underestimated, which can be explained only by their insufficient diagnosis. As for early diagnosis of inborn errors of metabolism, there is still the problem of the absence of many technical facilities for biochemical assays.
Currently, the system of neurological care for children in Russia is under transformation. Some health-care authorities support the principles of general-practitioner education, but the others insist on keeping the former educational subdivision in the universities in medical and pediatric faculties. The system of medical insurance is being introduced, which has yet to reach perfection. Nevertheless, it is obvious already that child neurological care must be provided to a great extent locally, on a residential basis. However, for more sophisticated and technologically-difficult diagnostic and treatment procedures, federal centers must be available, including centers for neurosurgical, epileptic patients or children with metabolic and hereditary disorders. A system of regular professional training for child neurologists and exchange of experience has to be developed. For instance, some neurological schools are being arranged for professional training in the most important fields of child neurology.
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