We studied swallowing movements of 8 patients with severe motor and intellectual disabilities and recurrent pulmonary infectious diseases. All the patients were orally fed with no problems, but had frequent episodes of pyrexia, mostly due to pulmonary infections. Videofluorographic studies revealed severe impairments in the transitional and pharyngeal phases. The patients aspirated liquids, most frequently during swallowing.
Prior to swallowing pooling of liquid barium was frequently observed in the hypopharynx, and that of purees in the pharynx after swallowing. The delay of cricopharyngeal relaxation was also frequently observed. Based on the videofluorographic findings, we made some efforts to provide patients with evident or latent aspiration with better management: changes in food textures and posture during drinking and eating.
These efforts significantly reduced the frequency of pyrexic episodes. Since latent aspiration is not uncommon among patients with severe motor and intellectual disabilities, swallowing disturbance should be evaluated in detail by videofluorographic examination to improve QOL.

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We studied swallowing movements in 14 patients with severe motor and intellectual disabilities and development of the impaired oral phase. The oral phase in the patients did not develop beyond the late weaning phase. They had impairment of the oral phase: poor or absent lip seal, infantile swallowing, slowed or stalled bolus transit, and oral residue after swallowing. Videofluorographic studies revealed severe impairment in the transitional and pharyngeal phases: pooling of liquid barium in the hypopharynx prior to swallowing, the delay of cricopharyngeal relaxation, aspirated liquids (silent aspiration 77.8%), aspirated purees, pooling of purees in the pharynx after swallowing. After videofluorographic studies we made some efforts to provide patients with better management; changes in food textures and posture during drinking and eating. These efforts eased their feeding difficulties.
Videofluorographic studies could be helpful in evaluating swallowing disturbance in the oral phase and thereby preventing chronic aspiration, malnutrition and feeding difficulties in patients with severe motor and intellectual disabilities.

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Thirteen children aged 2 years or less who had intractable epilepsy caused by cortical dysgenesis underwent functional hemispherectomy. The cerebral malformations were hemimegalencephaly in 8 cases and focal cortical dysplasia in 5 cases. Among 11 children who were followed for at least 6 months after the operation (6 to 54 months with a median of 26 months), 5 were seizure-free, 4 achieved>90% seizure reduction, and 2 achieved 50-90% reduction. Ventriculo-peritoneal shunt was placed in 3 children with hemimegalencephaly. After hemispherectomy, all the children showed improvement in psychomotor development. Development was accelerated in 3 seizure-free children. In children with cortical dysgenesis, functional hemispherectomy may result in remarkable seizure reduction and steady developmental progress.

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We reported serial neuroradiological findings of a 20-year-old male with subacute sclerosing panencephalitis, who deteriorated from Jabbour stage 2 to stage 4 rapidly in spite of oral administration of inosine pranobex. At the stage 2, brain magnetic resonance imaging (MRI), computed tomography (CT) and [^99mTc]-L, L-ethyl cysteinate dimer (^99mTc-ECD) single photon emission computed tomography (SPECT) findings were normal. As the disease progressed, brain MRI and CT revealed diffuse cerebral white matter lesions as well as cerebral atrophy. ^99mTc-ECD SPECT showed serially various perfusion of cerebrum. These findings in ^99mTc-ECD SPECT may reflect the acute inflammatory process and consequent destruction of brain tissue.

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To clarify the relationship between epileptic attacks and cognitive dysfunction, we examined the serial findings of ¹²³I-IMP single photon emission computed tomography (SPECT) in relation to the intelligence quotient (IQ), assessed by Wechsler Intelligence Scale of Children-Revised, in two female patients with focal cortical dysplasia (FCD) over a 10-year period. The age of patient 1 at the initial assessment was 2 years, and the age of patient 2 was 9 months. They developed complex partial epilepsy in infancy, and were treated with antiepileptic drugs, which remained effective until 11 years of age, when their epileptic attacks recurred.
Patient 1, a 14-year-old girl with FCD of the left parietal lobe suffered from dyscalculia, right-left disorientation, and finger agnosia even when she was free of epileptic attacks. Following the recurrence of seizures which occurred every night, she became unable to understand what was said to her. A hypoperfusion area detected by ¹²³I-IMP SPECT was restricted to the left parietal lobe during the seizure-free period, but spread to the temporo-parietal lobes following the recurrence. Her verbal IQ declined from 94 (at 9 years of age) to 63 (at 11 years and 8 months). After her seizures were controlled again (at 14 years and 4 months), the ¹²³I-IMP SPECT findings improved.
Patient 2, a 12-year-old girl with FCD of the left frontal lobe, showed cognitive dysfunction. Her verbal IQ declined from 91 (at 7 years and 5 months) to 76 (at 11 years and 8 months) following a recurrence of epileptic attacks. ¹²³I-IMP SPECT showed hypoperfusion in the left frontal lobe, where the accumulation count ratio (left/right ratio) declined from 0.86 (at 3 years) to 0.64 (at 11 years).
These findings suggest that epileptic attacks are related to cognitive dysfunction in FCD patients. This cognitive dysfunction appears to correlate with the appearance of hypoperfusion areas, as detected by ¹²³IIMP SPECT.

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We report a 4-year-old girl with multiple sclerosis (MS) and increased serum anti-arrestin antibody. She was admitted to our hospital because of sustained fever, headache and anorexia. Serial magnetic resonance imaging (MRI) revealed diffuse high intensity of cerebral white matter and basal ganglia on T₂-weighted imaging. We treated her with high- dose intravenous methylprednisolone under a diagnosis of ADEM. She recovered without any neurological sequelae in three months and the MRI lesion almost disappeared. However, she developed optic neuritis and two episodes of neurologic involvement associated with multiple white matter lesions in the next two years. She was then diagnosed as having clinically definite MS.
Anti-arrestin antibody is reportedly present specifically in adult patients with MS and uveitis. We performed an immunoblot assay using bovine retina, and our case revealed the presence of anti- arrestin antibody. Serial examinations showed that the serum anti-arrestin antibody titer correlated well with the disease activity. These results indicate that anti-arrestin antibody may be a good parameter of the disease activity of MS even in childhood.

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Two cases of auditory disturbance caused by carbamazepine are reported. Patient 1, a 9-year-old boy with benign Rolandic epilepsy, developed impairment of pitch perception one day after administration of carbamazepine. Patient 2, a 33-year-old female with glossopharyngeal neuralgia, developed impairment of pitch perception several hours after administration of carbamazepine, complaining that the perceived sounds became lower than previously by a semitone. Her pitch perception recovered soon after the cessation of carbamazepine. The carbamazepine-induced disturbance in pitch perception may be more frequent than we guess.

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Shuddering attacks (SA), an uncommon benign disorder occurring during infancy or early childhood, should be distinguished from epileptic seizures. The attacks are shivering movements occurring daily for several seconds without impairment of consciousness. SA are regarded as an early premature manifestation of essential tremor (ET), and the provoking mechanism is the same between them. There are case reports of SA, but none from Japan.
We studied the pathophysiology of SA in four children aged between 8 and 14 months using a video-EEG monitoring system. In one patient, the shuddering movements, as indicated by contaminating electromyogram during electroencephalography, was almost as frequent as that of ET. SA decreased in frequency or disappeared in all our patients. One of them showed immature brain development on MRI and had relatives with epilepsy. Another showed flattened sella turcica. Although previous reports suggest that SAs are benign and require no investigation, children with SA could have borderline problems related to the development of the nervous system.

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We reported here three infants aged 11 to 12 months who, according to their parents, suddenly became unwilling to stand up. All the patients had barely achieved the ability to stand or to walk at the time of the illness. The symptom developed relatively acutely in the winter months from 1997 to 1998 with a probable preceding infection. On examination, no paralysis of the lower extremities was observed, and muscle weakness was minimal. There were neither violent tremors, nystagmus, nor sensory disturbance. Orthopaedic disorders were considered unlikely. Biochemical examinations of the serum were not diagnostic, and imagings of the central nervous system were normal. Spinal tap revealed mild cerebrospinal fluid pleocytosis in two of the patients. All the children showed almost complete recovery without any medication during the following preriod of 2 days to 2 months. Although no definite signs of ataxia were recognized in the three patients, a diagnosis of acute cerebellar ataxia was considered to be appropriate.
Mild, infantile acute cerebellar ataxia may be overlooked because of the obscure complaints of the parents and the difficulty in performing a thorough neurological examination of infants.

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A 6-year-old girl with attention-deficit hyperactivity disorder (ADHD) who had been abused by her mother in infancy developed supplementary motor area (SMA) epilepsy. The seizure was characterized by bilateral tonic seizure of the upper and lower extremities, speech arrest, preserved consciousness and a lack of postictal confusion. The duration of the seizure was usually 10-60 seconds. The seizures sometimes clustered. She was diagnosed as having SMA epilepsy based on the characteristic clinical symptoms, interictal EEG, ictal video-EEG and ictal SPECT. Though her seizure was initially improved by anti-epileptic drugs, the symptoms appeared again after discharge. Since her clinical course indicated that her seizure was aggravated by her mental state, treatment included both medication with anti- epileptic drugs and the adjustment of her living environment in cooperation with a child guidance clinic. Thereafter both her epileptic seizure and ADHD symptoms improved. These changes may be related to each other, because both conditions are associated with frontal lobe dysfunction. It was interesting that the adjustment of the environment improved frontal lobe epilepsy, which inturn ameliorated ADHD symptoms.

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Although brain death of children has recently been hotly discussed in Japan, there still remain uncertainty and confusion. A pediatrician's diagnosis that a child is brain-dead entails delicate and emotional issues. Circumstances of the patients, families and institutions are so variable that many questions cannot be answered. It is most important to be aware of these problems and to seek consensus in the community. Pediatricians should provide their best care to both the patients and their families.

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In Japan, “brain death” has been discussed exclusively in connection with organ transplantation. However, the concept of brain death, which emerged with the progress in intensive care medicine, should be discussed in the context of palliative care in the ICU. It should be recognized that intensive care medicine includes not only life-saving high-tech therapeutic modalities, but also ethical and psycho-social aspects of modern medicine. In order to find out a decent solution to pediatric brain death issues, it is essential to develop pediatric intensive care in Japan.

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To define the roles of donor coordinators (certified procurement transplant coordinators; CPTCs), we visited University of Florida Colleges of Nursing and Medicine and certified procurement transplant interviewed (CPTCs) and clinical specialists in pediatric critical care. Recipients were dealt with certified clinical transplant coordinators (CCTCs). These coordinators are certified by taking examinations to see if they are equipped with specialized knowledge and communication skills. The small number of organs available for transplant poses a serious problem.
In many cases, medical personnels are not proficient enough to recommend organ donations to the family members of potential donors. Thus, the role of CPTC includes education of health professionals. In one study, 95% of the donor family members felt that hospital staff members explained about transplantation well, while 67% of the nondonor family members felt the same. The concept of “brain death” was not fully understood by some of the latter. The timing and place of discussion as well as the skill levels of medical staffs (preferably CPTCs) were important in receiving the consent. It is necessary for the donor family members to understand the concept of “death”. Communications between the hospital staff and family members are critical.
In Japan, there is no solid foundation for pediatric organ transplantation. Specialized nurses in pediatrics are limited. Further, there is meager certification programs for CPTCs or CCTCs. Under the present state, nurses inICU and pediatric wards may act as potential coordinators. Education of these nurses will be critical to increase the number of pediatric organ transplantation.

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