Status paralyticus

present in the nervous system. Movements are considered to be secondary consequences only. The Institute works in an open system. Approximately 1,000 Hungarian and foreign patients with motor dysfunctions are assessed per annum. Integration through CE is primarily achieved in a significant proportion of cerebral palsy (CP) patients (pursuant to the European definition). The required duration of CE is the shortest in the so-called pure hypotonic and diplegic CP. Long-term CE is needed in clinical pictures with athetosis. In other systems, however, the latter can be less successfully influenced, regardless of the duration. Since CE is not obligatory in any disease and was applied in various other clinical pictures by its founders (Pet6 and H~iri), the demand for this is still present. In other diseases (e.g., HMSN, SNE, Kugelberg-Welander) representing about 10% of the Institute's turnover, in cases where the medical solution is lacking, CE achieves a different, human specific quality of life. We find it necessary to take into account the theoretical considerations to permit development of the necessary conditions to achieve maximum possible performance. The result will not depend on the effectiveness or suitability of CE but on the grade and nature of the progress of underlying disorders. It is important both for the child and parents that the child acquires certain skills, even if these are limited or the child may later on lose them as if he has never had them before. The child's quality of life is much better and his personality develops in a more favorable way with CE than would be without it. If there is a better, more effective, and more accessible possibility, CE should not replace it.

92. CHANGING PANORAMA OF CEREBRAL PALSY IN HUNGARY (1952-1992) Ildid6 Kozma and Erzs6bet Balogh, Budapest, Hungary The nearly stable population of Hungary is about 10 million. In the assessed period, the number of live births has declined, amounting to approximately 120,000 per annum at present. Despite all medical efforts the incidence of low birth weight (< 2,500 gm) has been between 9-15%, currently about 10%. In the past decade the morbidity of cerebral palsy (CP) among infants with a birth weight under 1,500 gm with no other risk is 1.5%. The incidence of CP is otherwise 0.2-0.3%. In order to assess the situation, we monitored CP patients in Hungary. At the beginning of the period, the prevalence of the main spastic diseases (double hemiplegia, hemiplegia, diplegia) took up 1/3 of all CP patients, while other mixed or athetoid clinical pictures came to 2/3 of patients. Between then and the past few years the prevalence of tetraparesis (double hemiplegia = total body involvement) increased gradually; most recently it amounts to 2/3 of the morbidity of CP, while other diseases share the remaining 1/3. This is attributable to the following: (1) we have been unable to properly prevent intrauterine lesions; (2) rate of premature births has not been diminished but the proportion of survivors has grown; (3) perinatal traumas have been successfully reduced and kernicterus has been eliminated; and, (4) in some instances we are still unable to control chronic hypoxia. As regards our tasks, the new situation does not necessarily mean a change for the more difficult. In many cases, disturbances in personality development, intellectual deficit, perception deficiencies or epilepsy as concomitant diseases are more determinative for the social burden than the primary disease. In Hungary motor dysfunctions are influenced by early developmental neu-

368 PEDIATRIC NEUROLOGY Vol. 8 No. 5

rologic habilitation and by the typically Hungarmn (7t.; (Peti~ method and system).

93.

STATUS PARALYTICUS

Erzsrbet Batogh and Adrienne Halzisz, Budapest, Hungary Conductive education (CE), practiced in a system, achieves significant successes in the rehabilitation and social integration of cerebral palsy (CP) children. Approximately 1,000 candidates per annum are examined and assessed for suitability and then provided with CE. As a rule, motor dysfunction (CP) is only one and not always the most serious disability of these individuals. Besides the severity of the primary disease, success is influenced most significantly by the severity of the concomitant diseases, apart from the quality of CE. Epilepsies with a standard appearance are diagnosed according to the general professional rules of child neurology. The judgement of "status paralyticus" conditions, however, represents a special problem, and in the literature only little data are of help. Under status paralyticus we include a convulsive equivalent paresis or plegia of the sensorimotor system, the onset and the end being easily definable, persisting for hours, maybe days, with fluctuating intensity, deviating in extent from the CP symptoms otherwise present in the child. As a rule, the minus symptoms of the palsy (decrease in tone, more marked decrease of muscle power, loss of skill, more marked neglect, general or partial deficiency of motion) are characteristic of this condition. Ictal and interictal EEG, and symptoms becoming more serious, separable from the child's clinical paresis which is of a status character, suggest a suspicion. Standard convulsion phenomena are not present and the split-screen is of no help because poor motion is more characteristic than recordable movement. We may think of a certain appearance of perinatal convulsions and post-convulsive pareses for analogy. Its treatment lege artis is preventive anticonvulsive therapy.

94. SEQUENTIAL MOTOR PROGRAMMING DETERIORATION IN RETT SYNDROME: C L I N I C A L F O L L O W - U P STUDY O F 13 PATIENTS Juan Narbona and M. Asunci6n Garcia P6rez, Pamplona, Spain Sequential features concerning motor deterioration have been collected from clinical examination and from retrospective and prospective video recordings in a series of 13 Rett syndrome patients covering an age range of 1.5 to 14 years. The duration of direct clinical follow-up has ranged from 4 to 9 years (mean: 7 years, 2 months). The hallmark motor features can be grouped in 3 main successive periods: first (age 1-4 yearst, manual praxic capacities (ideomotor and myelokinetic) deteriorate until near total inability of purposeful actions involving external objects; second (age 5-8 years), abnormal approach-to-own-body gesturing raise progressively finger-to-finger, finger-to-hairmouth, and hand-to-hand ("hand washing" stereotype), contemporaneous with active avoiding reactions to external objects put in contact to the hands; and third (age 9 to 14 years), progressive dystonia in distal parts of lower limbs, mimical freezing, deficits in rapid postural axial reactions, and "pirtinement," this constellation of motor control deficits contributing to progressive loss of locomotion. The sequential deterioration of motor programming we have described- loss of manual praxias, subsequent raising of approach avoiding pathologic gestures and develop-