Acute necrotizing encephalopathy of childhood (ANE) is a clinicopathological entity recently separated from acute encephalopathy of unknown etiologies. The hallmark of ANE is multiple, bilateral symmetric brain lesions showing edema and necrosis which occur in the bilateral thalami and other specific regions. Since its establishment in 1993-1995, data have further accumulated and have provided additional insight into its pathogenesis. This review summarizes recent achievements on ANE, with reference to issues to be clarified by future studies.

View full abstract

Cerebral palsy is associated not merely with impairments of posture and locomotion, but also with various other problems. Medical illnesses should be treated by an adequate system. Emotional disturbances require a psychological support. Communication disorders may be overcome with proper modern equipments. Perceptional deficits and sensory impairments should also be addressed.
Care systems for physically handicapped children in Japan were established by the concept of ryoiku, a term in Japanese created about fifty years ago by K. Takagi, Professor of the Department of Orthopedics, University of Tokyo. This term can hardly be translated into English, bot in my view stands for a comprehensive approach comprising orthopedic treatments, educational programs and vocational trainings. Based on this concept, Dr. Takagi founded an institution for the physically handicapped in 1942, followed by establishment of similar institutions in all areas of Japan after World War II.
Despite its historical role, ryoiku does not meet any more the current standard of care for cerebral palsy. We need now a new comprehensive term and management systems. A novel medical subdivision “medicine for developmental handicaps” may also be necessory.
In this paper I made an overview of current problems of children and adults with cerebral palsy. I proposed a new concept of ryoiku and emphasized the need for the new medicine to treat developmental handicaps.

View full abstract

We investigated the relationship of the infants admitted to the neonatal intensive care unit (NICU) of Anjo Kosei Hospital, the outpatients of the pediatric neurology division of the same hospital and the patients in Daini-Aoitori Gakuen, A ichi prefecturalh ospitala nd Rehabilitation Center for Disabled Children.
Eighty-six infants (2.9%) disclosed perinatal brain injury, and seventy infants (2.3%) had cerebral palsy in a total of 2, 985 patients who were admitted to the NICU in Anjo Kosei Hospital. Forty-seven percent of the outpatients of pediatric neurology division had been admitted to NICU. Eighty-four percent of the infants with cerebral palsy had been referred to the Rehabilitation Center for Disabled Children. MRI disclosed perinatal brain injury in 72% of patients with cerebral palsy.
We stress the importance of an information network among NICUs, pediatric neurology divisions and rehabilitation centers for disabled children.

View full abstract

In children with severe cerebral palsy, the daily use of naso-pharyngeal airway, daily chest physiotherapy and adequate posture control markedly improve respiratory disorders and their consequent complications. Non-invasive ventiratory support methods such as nasal IPPV and nasal CPAP can be also effective. Enteral feeding through a naso-jejunal catheter can successfully manage feeding difficulty due to gastroesophageal reflux when surgical treatment is difficult. Exact assessment of aspiration by adequately performed video-fluoroscopic swallowing examination leads to rational management of dysphagia. Tube feeding by intermittent oro-gastric catheterization combined with oral feeding is useful way in many dysphagic children. Surgical treatment for prevention of aspiration markedly improves the general condition. These treatments and managements have markedly improved the QOL of children with severe cerebral palsy in many aspects. Cooperation of pediatricians, parents, school teachers and other community staffs is neccesary for appropriate daily management of medical problems of these children.

View full abstract

Preterm diplopic children (SD) frequently suffer pedagogical difficulties. They showed visual perceptual impairments end also constructional dysphasia, neuropsychologically. Multiple regression analysis proved that the constructionald yspraxia was independent from the impairments of visual acuity, stereoacuity, depth perception and visual perception. EEG coherence analysis in SD patients disclosed lower interhemispheric coherence at alpha band frequency in occipital derivations. The lower coherence value may correspond to thinning of posterior body and splenium of corpus callosum in SD patients. A adequate ophthalmological treatments, books with enough space between the lines and three dimensional models to facilitate the image of stereography are recommended.

View full abstract

During the last decade, the idea of self decision have become a very important concept for the independent life of children with cerebral palsy. In association with this, AAC (augmentative and alternative communication) researches became more active to support their communication. Especially, high- tech communication aids have enabled speechless cerebral palsied children to speak. However, in Japan, there are negative attitudes toward the use of aids in the parents, therapists, physicians, and teachers. Consequently, aids are not popular among young Japanese children at present. In contrast, there are in US some reports showing positive effects of aids. It does not only compensate their disabled functions, but also make a chance to communicate with other people. In this paper, how communication aids affect habilitation of children with cerebral palsy was discussed.

View full abstract

A variety of Technical Aids have been developed and adapted for children with cerebral palsy. The most important objects of Technical Aids are positioning, locomotion and communication.
Essencially, Technical Aids are based on assistive technologies, and must be applied to the activities of daily living and to habilitation for children with cerebral palsy by themselves, their family and the professional staff in institutions. It is necessary not only to develop new Technical Aids, but also to extend each adaptive measures and applied technologies for all local areas.

View full abstract

We studied functional deterioration in adults with cerebral palsy working at community workshops throughout Japan by interviews and direct examinations. The incidence of functional deterioration was 36%, being higher among those with involuntary movements of the head and neck, abnormal movement patterns when moving and those with reported in poor work environment. In more than one-half case, deterioration was associated with inadequate in noise control, lighting, air circulation from the cooling and heating systems, height of the chairs and desks, and height of VDT screens. Though the factors intrinsic to cerebral palsy were often responsible for the functional deterioration, environmental factors were also significant. Studies on the work environment as well as medical evaluations are needed.

View full abstract

The child with cerebral palsy becomes the adult with cerebral palsy. In Japan early detections and treatments of CP have enthusiastically brought into practice over the last twenty years. But cerebral palsy could not be cured. Children/adults with CP should be provided comprehensive treatments and cares so that they can perform their daily activities and fulfil their social roles expected during their lifetime.
There are two concepts needed to provide comprehensive management of CP. The first is a medical model that assesses CP in the following manner: etiology-pathology-manifestation. The other is a disability model based on three seperate categories suggested by WHO: impairment-disability-handicap. Keeping these in mind, total effective approaches to children/adults with CP were discussed. Neuropaediatricians are asked to play important roles which contain epidemiologic activities, early detections, and medical treatments. They should refer clients and their families to related professionals if necessary and have also knowledges of assisstive equipments to enrich their lives.

View full abstract

The findings on magnetic resonance imaging (MRI) or computed tomographic scan in 35 children with acute encephalopathy (AE) induced by infections were categorized into five groups:(1) normal findings (11 cases), (2) normal findings in the acute phase followed by mild brain atrophy (1 case), (3) severe brain edema which developed within 48 hours after the onset of AE (7 cases), (4) diffuse cortical necrosis which appeared around 4 days after the onset of AE (9 cases), and (5) symmetric thalamic lesions (7 cases). Their outcomes were as follows: three cases developed epilepsy in (1) and (2); six died and one had a sequel of severe brain damage (SBD) in (3); all cases survived but had SBD in (4), and four died, one had SBD, and 2 recovered in (5). Serum AST were elevated in 7, 7 and 6 cases in (3), (4) and (5), respectively. In all of them, there were more than one case having liver histologies consistent with Reye syndrome.

View full abstract

We report a 4-year-old boy with congenitalc entral hypoventilations yndrome (CCHS) successfullyt reated with home mechanical ventilation with nasal intermittent positive pressure ventilation (NIPPV) during sleep hours. He had had frequent severe apneic attacks from the neonatal period. At 8 months, he was treated with positive pressure ventilation following a tracheostomy. At 4 year and 2 months, NIPPV was attempted because of recurrent respiratory tract infections and cor pulmonale. The tracheostomy was successfully abandoned 6 months later. Adequate ventilation has been maintained for more than 3 years without troubles. NIPPV is an effective and non-invasive treatment of CCHS that it significantly improves the quality of life during daytime.

View full abstract

We report a 7-year-old girl with severe Guillain-B arre syndrome (GBS) who showed dramatic improvementa fter immunoabsorptionth erapy. She had progressivem uscle weaknessw ith resultant respiratory failure. On the 7th day of the illness, she was nearly quadriplegic and dependent on mechanical ventilation. In addition, she had autonomic manifestations, areflexia, abolished or delayed peripheral nerve conduction, and increased CSF protein. Administrationo f a high dose of gammaglobulin failed to improve the symptoms. Immunoabsorptionth erapy using a tryptophan column was performed9 times during 17 days. After 5 times of immunoabsorption therapy, the muscle weakness improved dramatically ; she was extubated 16 days after the institution of the therapy and walked without assistance on the 41st day. She regained normal muscle power without any sequelae. The immunoabsorption therapy caused hemolysis and decrease of serum fibrinogen, but no serious complications. The clinical course of this patient suggests the efficacy of immunoabsorption therapy in GBS.

View full abstract

[in Japanese]

View full abstract