This symposium, discussed clinical approaches to improve the quality of life for severely disabled children including the management of feeding and breathing problems, schoolife and grief care. Pediatric neurologists should be health care professionals for them, consider improving their quality of life at both home and school, help them to decide which information from different professionals is appropriate. In order to improve their quality of life, feeding and breathing problems are often the most important. Furthermore, we have to promote discussion and co-operation with school teachers, especially about so-called medical care.

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With an increasing number of children with severe neurological impairment living in their houses, there is growing demand for medical care and support in school and community life. In such cases, respiratory disorder, gastro-esophageal reflux and dysphagia are closely related. To improve these disorders, appropriate rehabilitation and daily managements, such as posture control, are important as well as medical and surgical treatment. Social and educational support is also necessary for improvement of the QOL of these children and their family. For example, daily medical care such as tube feeding and sputum suctioning should be provided by school staffs. Pediatric neurologists should actively participate in such educational and social activities.

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In children and adults with severe motor and intellectual disabilities (SMID), management of nutrition is very important. We investigated the problem of long-term use of tube feeding on which many of them depend because of swallowing dysfunction. Trace elements such as copper, zinc, selenium and long chain unsaturated fatty acid (ω-3fatty acid) were often deficient. To evaluate their nutritional condition, we calculated the body fat mass by the method of Bioelectrical Impedance Analysis and measurement of subcutaneous fat thickness. Patients with the athetotic type of cerebral palsy had a lower level fat mass than those with the spastic type. Patients with a low body fat mass tended to require more energy than those with a high body fat mass. To improve the quality of life of persons with SMID, it is important to find clinical dysfunction related to the nutrition and to improve the nutritional condition immediately.

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We present here the indication, procedure and results of surgical treatment of gastroesophageal reflux (GER) for neurologically impaired children. We decide its indication based on clinical symptoms and findings of upper GI series, esophageal pH monitoring and GI fiberscopy, respecting the QOL of the patient and family. Laparoscopic fundoplication has become the first choice in surgical treatment of GER because of its good results. However, an anti-reflux procedure is not effective in patients with intractable aspiration. A laryngotracheal separation procedure should be applied for these cases.

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Children with severe physical disabilities frequently have respiratory problems which affect their quality of life (QOL). They commonly stem from central nervous system dysfunctions and/or severe motor disabilities, and consist of various impairments deriving primarily from central and motor dysfunctions, such as dysmyotonia, deformation, dysphagia, and gastro-esophageal reflux (GER), which often influence each other and result in respiratory insufficiency without adequate interventions. Aging is also an important factor to worsen respiratory involvements and to change their underlying pathophysiology gradually, even if the primary cause of the disability is non-progressive. A key to effective treatment and improvement of their QOL is to understand the pathophysiology. Evaluation is primarily based on regular physical examination. Other examinations include blood sampling to examine inflammation, nutritional state and blood gas analysis, round-the-clock SpO₂ and TcPCO₂ or EtC0 ₂ monitoring, chest X ray and computed tomography, polysomnography, laryngoscopy, bronchoscopy, evaluation of swallowing function, and evaluation of GER. Postural control, relaxation, respiratory physiotherapy, and treatment of dysphagia are the most important and common therapeutic procedures. Treatment of GER, treatment of upper airway obstruction, oxygen therapy, tracheostomy, or mechanical ventilation may be needed. It is also important to take into account the possibility that the treatment procedures themselves could affect the patient's QOL. Counseling should be performed before and throughout the treatment process about how patient's and family's everyday life will be influenced positively and negatively by the treatment, and a multidisciplinary team should support all aspects of their ne

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The final report of the Collaborative Research Conference on Special Education in the 21th Century pointed out that supportive activities, such as education, welfare, medical care and labor should be strengthened to provide special support for disabled children, and that measures should be taken for school children requiring medical care in daily life, in response to an increasing number of children with severe and multiple disabilities and other social changes. Recent great progress in pediatric medicine has enabled these children to attend schools for the physically handicapped which provide more specialized educational support. At present, support for these children is still insufficient. This symposium discusses these problems as well as future prospects of disabled children's schools.

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Terminal care for the handicapped newborns and the grief care for their families have recently emerged as a medical problem. Questionaires to 13 parents who lost their children showed that they were unsatisfied withthe care from doctors and nurses before and after the child's death.
Medical approach to grief care includes changes in the quality of medicine for handicapped newborns. A system should be created to improve the relationship between the NICU pediatricians/nurses and the staffs engaged in early intervention prior to the newborns' death, which in turn strengthen the ties with a network supporting the families who have lost their newborns.

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[in Japanese]

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Mollaret's meningitis is a rare disease of unknown etiology, characterized by repeated aseptic meningitis with transient neurological symptoms and quick recovery. The patient, a 16-year-old boy, had episodes of acute encephalitis followed by complete recovery every year from the age of 13 to 16 years. The symptoms at onset were loss of consciousness, a generalized tonic clonic seizure and pyramidal signs. He was first admitted to our hospital at the age of 15 years. EEG in the early stage showed diffuse low-voltage rhythmic fast activity. He recovered consciousness after a week. At the age of 16 years, he was admitted again because of high fever (39°C). Soon after admission, he had generalized tonic-clonic seizure and was accompanied by alternating deep coma and delirium. Cerebrospinal fluid examination during early stage revealed mild leukocytic pleocytosis, elevation of protein and a high level of IL-6, although brain CT and MRI showed no abnormal findings. EEG showed intermittent, diffuse, middle-voltage, rhythmic fast activity. After 5 days, the neurological symptoms completely disappeared with EEG normalization. To our knowledge, this is the first report of rhythmic fast activity on EEG in Mollaret's meningitis. The clinical pictures of this case suggest a transient functional disorder of brainstem or cerebrum may occur in this condition.

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Benign myoclonus of early infancy (BMEI) is a non-epileptic paroxysmal phenomenon. Some patients with BMEI were mistakenly treated as infantile spasms, because the fits resemble to tonic spasms in infantile spasms and they occur in cluster. However, the patients have normal development and no abnormal electroencephalograms (EEG), and the fits spontaneously subside without sequelae. There are only a few reports on BMEI, and it is not widely recognized in Japan. We report three cases of BMEI. All the cases were suspected to have infantile spasms fromthe characteristic features of paroxysmal events, and the parents had strong anxiety because of recurrent fits.However, the fits decreased dramatically in about three months, and spontaneously disappeared within one year without any sequelae. BMEI might be included in cases of suspected infantile spasms, and such patients should be followed by monthly EEG examinations and close observation for other seizure phenomena. To avoid unnecessary treatments, such patients should be observed without any therapeutic trials including antiepileptic drugs.

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We report an 11-year-old girl with Turner syndrome with 45, X/46, X, mar (X) who had mental retardation. EEG showed remarkable provocation of paroxysmal activity by photic stimulation. Diffuse irregular poly-spike and wave bursts werelicited by photic stimuli, without accompanying by clinical seizure

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We report here a boy with infantile-onset fibromuscular dysplasia (FMD). At 8 months of age, he had the first cerebrovascular attack presenting with left facial palsy, followed by recurrent attacks of alternating hemiparesis. Involvement of systemic arteries was manifested by variable somatic symptoms: retarded growth of the right arm due to stenosis of the brachial artery, constipation and recurrent abdominal pain due to intestinal ischemia, and renovascular hypertension. Cerebral angiography disclosed stenosis of the bilateral internal carotid arteries and right vertebral artery, with development of moyamoya disease like collateral vessels. Systemic arteriography revealed stenosis of the right brachial and left femoral arteries, which was either tubular or mimicking a string of beads. This case was characterized by the early onset and involvement of many cerebral and systemic arteries resulting in severe and variable symptoms.

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A case of forced normalization in childhood is presented. When zonisamide was administered to a five-year-old girl with intractable epilepsy, disappearance of seizures was accompanied by severe psychotic episodes such as communication disturbance, personal relationship failure, and stereotyped behavior, which continued after the withdrawal of zonisamide. These symptoms gradually improved by administration of fluvoxamine, however epileptic attacks reappeared. Although most patients with forced normalization are adult and teenager, attention should be paid to this phenomenon as adverse psychotic effects of zonisamide even in young children. Fluvoxamine may be effective for the symptoms.

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[in Japanese]

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