A nationwide survey of bilirubin encephalopathy in preterm infants in Japan

ObjectivesTo examine the clinical characteristics of bilirubin encephalopathy in preterm infants (pBE) in Japan.MethodsWe performed a two-step nationwide questionnaire survey. The initial survey determined the number of children with pBE. Using a structured questionnaire, the second survey clarified the clinical manifestations and characteristics of children with pBE, including the perinatal history, neonatal complications, neurological features, verbal communication, diet, and magnetic resonance imaging (MRI) and auditory brainstem response (ABR) findings.ResultsThe initial survey included 190 pBE infants, indicating an incidence of approximately 10 per year. Clinical information was available for 142 of them. The median gestational age was 26 weeks and the median birthweight was 883 g. As to neonatal complications, 20% had none, 25% had one complication, 54% had two or more. Head control was observed in 45% and functional gait in 8%. Purposeful hand use was seen in 41% of patients and verbal communication in 40%. MRI showed T2 hyperintensities in the globi pallidi in 111 of 136 patients, especially between 7 and 18 months of corrected age. ABR abnormalities were present in 88 of 117 patients.ConclusionspBE was infrequent but constantly observed during the study period, especially in very preterm infants, even in those with no severe neonatal complications. Severely impaired gross motor function and relatively preserved manual function and verbal communication were characteristic. MRI and ABR abnormalities will facilitate diagnosis.     

Tourette syndrome in Japan: A nationwide questionnaire survey of psychiatrists and pediatricians

In order to shed light on the clinical picture of patients with Tourette syndrome (TS) treated at medical institutions in Japan, a nationwide survey covering both pediatric patients and psychiatric patients was conducted. We mailed 316 questionnaires on experience in treating TS cases and the patients' present conditions etc. to specialists such as psychiatrists and pediatricians. A total of 164 responded. The survey found 154 TS patients being treated at the time of survey, 45 (29.2%) had obsessive–compulsive symptoms (OCS), and 10 (6.5%) had family histories of TS. It was suggested that TS is often associated with OCS and that familial cases of TS are slightly less common in Japan than they are in the USA. Of the 116 respondents who described their experiences, 85 (73.2%) said that they had treated one or more patients displaying the symptoms of frequent coprolalia, and 42 (36.2%) said that they had treated one or more patients suffering from developmental disorders. Based on these findings, we speculated that the rate of coprolalia in Japan is a little higher than the previously reported 4% and that TS is often associated with developmental disorders.     

A nationwide survey of human metapneumovirus-associated encephalitis/encephalopathy in Japan

BackgroundAs there have been no comprehensive reports of human metapneumovirus-associated encephalopathy (hMPVE), this study examined the clinical features of hMPVE in children in Japan.MethodA nationwide survey of children with hMPVE was conducted using a structured research form. An initial survey asked pediatricians about children with hMPVE treated between 2014 and 2018. A second survey obtained patient information from hospitals that responded to the initial survey and those identified as having treated cases from a literature search. We collected demographic data, symptoms of hMPV infection, neurological symptoms, laboratory data, treatment, and outcomes. Outcomes were determined using the Pediatric Cerebral Performance Category Score.ResultClinical information was available for 16 children. Their median age was 37 months. Six had preexisting neurological disorders. The interval between the onsets of infection and hMPVE was 4 days. Outcomes were good in 11 patients and poor in 5. There were no significant differences in demographic data, neurological symptoms, or laboratory data between the patients with good and poor outcomes. The encephalopathy subtypes were acute encephalopathy with biphasic seizures and late reduced diffusion in 3, clinically mild encephalitis/encephalopathy with a reversible splenial lesion in 3, hemorrhagic shock and encephalopathy syndrome in 2, and others in 8.ConclusionThe outcomes of children with hMPVE were not very different from those of acute encephalopathy due to other viruses. We found no factors associated with poor outcomes.     

A clinical survey of hydrocephalus and current treatment for hydrocephalus in Japan: analysis by nationwide questionnaire

We analyzed current treatment for hydrocephalus in Japan by means of a nationwide questionnaire. Ventriculoperitoneal (VP) shunting was the most common form of cerebrospinal fluid (CSF) shunting, and in 66% of cases the ventricular catheter was inserted via the anterior horn. Various types of flow-control valves were used in 46% of CSF shunting procedures, and the proportion of cases in which such valves are used seems to be increasing. The Codman-Hakim valve was used most frequently, followed by the Sophy valve. The initial pressure setting of programmable pressure valves was selected in 82% of institutes on the basis of the CSF pressure obtained during the shunt operation. Most flow-control valves were implanted in the retroauricular region; however, Sophy valves were implanted in the chest wall in a high percentage of cases. The rate of shunt complications was significantly lower among VP shunt cases with flow-control valves than in those without flow-control valves. The most common cause of shunt complications was trouble with the abdominal catheter. These results will contribute to progress in the treatment for hydrocephalus. Received: 15 March 1999     

Result of nationwide survey of the management of severe head injury in Japan.

A nationwide survey of the management of severe head injury was carried out in 1988 by sending the questionnaires to 1,088 main neurosurgical hospitals in Japan. The items of the survey included annual number of patients with closed severe head injury (GCS score of 8 or less), place of patients' care, type of neuromonitorings, medical and surgical treatments, severity and outcome measures. Out of 1,088 questionnaires, 457 (42% response rate) were collected and analyzed. Characteristic features of the management status were the scarcity of patients annually in each institution, limited use of specific neuromonitorings, and variety of the actual managements. Aggressive managements such as hyperventilation, barbiturate and/or hypothermia have been employed in many hospitals to control high ICP. External and internal decompression are also used widely for intradural hematomas. These results clarified not only present status of Japan but also the problems to be solved in the actual managements.     

Myelitis with atopic diathesis: a nationwide survey of 79 cases in Japan

We recently reported the occurrence of myelitis in patients with atopic disorders and its pathology to be eosinophilic inflammation. Because similar cases have been reported, we conducted a nationwide epidemiological survey (NES) of myelitis with atopy in Japan. We compared the clinicolaboratory features of the 30 NES cases with the 49 cases at Kyushu University Hospital (KU). Although the NES cases were distributed throughout Japan, the NES and KU cases shared common characteristics. We therefore combined all of the cases identified. The average onset age was 35.8+/-13.4 years, and the male/female ratio was 1:0.65. The onset mode was subacute/chronic in half the patients, and stepwise progression or symptom fluctuation was frequent (69.6%). The most common lesion site was determined clinically and by MRI to be the cervical cord, and paresthesia and/or dysesthesia were the most common symptoms initially (74.7%) and throughout the entire course (83.5%). Cerebrospinal fluid (CSF) abnormalities were infrequent and mild. These findings suggest that myelitis associated with atopy has mild but prolonged symptoms and occurs throughout Japan.     

Vision attention and discrimination in infants at risk and neurological outcome

Visual behavior has been studied in 129 high risk infants in order to appreciate the value of eye opening, visual fixation and pursuit and visual discrimination in predicting permanent brain damage. The assessment of visual discrimination according to Fantz and Miranda, has proven to be particularly helpful after corrected term. These tests allow the detection of severe permanent neurological sequelae even when visual attention and neurological examination appear to be normal. Certainly, during the 3 first months of life, visual behavior is of more value to predict future neurological outcome than the classical neurological examination.     

A nationwide survey of seasonal affective disorder at 53 outpatient university clinics in Japan

A nationwide survey of seasonal affective disorder (SAD) was performed from autumn 1990 to spring 1991 with the cooperation of 53 outpatient university psychiatric clinics in Japan. Forty-six SAD patients were identified among 5265 depressed outpatients. SAD was generally reported to occur in 1–3% of the depressed outpatients newly attending each facility. Hours of sunshine were found to be a more relevant variable influencing the prevalence of SAD than latitude or the mean temperature in December. The unexpectedly low percentage (20–30%) of SAD patients with atypical vegetative symptoms suggests that SAD patients who have no prior knowledge of SAD and those who are recruited via the media have different vegetative symptom profiles.     

Early assessment of visual information processing and neurological outcome in preterm infants

Psychological tests based on visual information processing have shown to be promising in predicting neurodevelopmental outcome in infants at risk. In the present study we prospectively investigated the early development in a group of 20 high-risk preterm infants by means of i) the Fagan Test of Infant Intelligence at 7, 9, and 12 months postterm and ii) a detailed battery for the early assessment of visual functions at 6 and 10 months postterm. The results were then correlated to the Griffiths development scales at two years. At around 7 months no correlation was found in our infants between the Fagan test and neurodevelopmental outcome, possibly as a consequence of the influence of abnormal oculomotor behaviour. At around 9 months most of the visual abnormalities were no more present and the Fagan test was significantly correlated with the outcome. At 12 months postterm a decline of the predictive value of the FTII was observed. In conclusion, nine months postterm age appears to be the best age for the early assessment of neurodevelopmental outcome in high-risk preterm infants, as the maturation of the attentional and visual systems allows a more reliable evaluation.     

Effects of G-CSF treatment on neutrophil mobilization and neurological outcome after transient focal ischemia

Several recent studies demonstrated beneficial effects of G-CSF treatment (granulocyte colony-stimulating factor) in various CNS disease. Possible mechanisms underlying this activity are neuroprotection, anti-apoptosis, angiogenesis and anti-inflammation. Hence, we investigated the efficacy of G-CSF administration in experimental stroke by determining infarct volume and neurological score in wildtype, G-CSF-deficient and G-CSF-treated G-CSF-deficient mice. Besides, cerebral ischemia is followed by an upregulation of endothelial adhesion molecules which promote leukocyte recruitment to the injured area. In combination with G-CSF-induced leukocytosis, increased peripheral neutrophils could aggregate within microvasculature and additionally impair blood perfusion of the ischemic tissue. Therefore, we analyzed the neutrophil counts in both vessel and tissue compartment 2 and 5 days post-stroke by immunohistochemistry.Here we show that G-CSF deficiency leads to increased infarct volumes, whereas G-CSF substitution revokes detrimental effects by reducing lesion size and enhancing neurological outcome compared to untreated animals. Administration of G-CSF is accompanied by significant increase of circulating neutrophils 2 days post-ischemia but leukocytosis is restricted to the vessel compartment and has no deleterious effect on lesion formation and functional recovery. These observations are likely to be important for therapeutic targeting of G-CSF-mediated neuroprotection in stroke.     

Computer interpreted fetal electroencephalogram: sharp wave detection and classification of infants for one year neurological outcome.

The presence of visually discernible sharp waves (SWs) in the fetal electroencephalogram (FEEG) has been found to be associated with abnormal neurological infant outcome, but no method of programmed SW detection for FEEG was available. In order to develop an algorithm for SW detection, the first and second derivatives for visually identified SWs and non-SWs were examined and five random variables chosen for discriminant function analysis (DFA). The resulting equation, incorporated into program logic along with logic for artifact rejection, produced classifications from 85% to 89% consistent with visual identifications, suggesting that the number of SWs/epoch (NSW) corresponds with visually identified SWs. In addition, in 61 cases using a threshold for NSW derived by DFA, computer recognized SWs were found to be significantly related to the overall visual interpretation of the tracings (P less than 0.005). Finally, NSW alone produced correct classification of 65.5% of infants for 1 year neurological outcome. The overall consistency was increased to as high as 80% using additional FEEG and neonatal data. These findings imply that some forms of brain damage are present before birth and can be detected during labor using FEEG.     

Psychogeriatric specialization in state mental hospitals: A nationwide survey

Despite deinstitutionalization and transinstitutionalization, psychogeriatric patients continue to be served in state mental hospitals. This paper reports data from a national survey of state hospitals describing the current status of geriatric services and specialists in these facilities. The data indicate that nearly three-quarters of hospitals serving elderly patients have geriatric units, but many hospitals, including those maintaining such units, have few or no psychogeriatric specialists. It is proposed that clinical training and education programs need to be expanded in order to meet the demand for specialists in this area.     

Headache with neurological deficits and CSF lymphocytosis: A transient ischemic attack mimic.

Headache with neurological deficits and cerebrospinal fluid (CSF) lymphocytosis (HaNDL) is a benign condition with a transient ischemic attack (TIA)-like presentation. It is a disease of young adults that is characterized by headache, transient focal neurological symptoms, and lymphocytic pleocytosis. The onset of neurological symptoms after cerebral angiography in patients with this disease has occasionally been reported. The authors present the case of a 28-year-old man with episodes of left-sided numbness and weakness associated with headache. He underwent cerebral angiography as part of his evaluation, after which he experienced an episode of right hemiplegia and aphasia. A subsequent magnetic resonance image (MRI) revealed two small new infarcts in the left parietal cortex. A diagnosis of HaNDL was made based mainly on clinical symptoms and CSF analysis. The symptoms resolved with conservative therapy. HaNDL is a benign condition that can present with symptoms similar to a TIA. Although HaNDL remains a diagnosis of exclusion, caution is required when considering cerebral angiography in the evaluation of patients with a HaNDL-like syndrome, because these patients seem prone to developing neurological symptoms after angiography.     

A nationwide survey of monocarboxylate transporter 8 deficiency in Japan: Its incidence,clinical course,MRI and laboratory findings

BackgroundMonocarboxylate transporter 8 (MCT8) deficiency is an X-linked recessive developmental disorder characterized by initially marked truncal hypotonia, later athetotic posturing, and severe intellectual disability caused by mutations in SLC16A2, which is responsible for the transport of triiodothyronine (T3) into neurons. We conducted a nationwide survey of patients with MCT8 deficiency to clarify their current status.MethodsPrimary survey: In 2016–2017, we assessed the number of patients diagnosed with MCT8 deficiency from 1027 hospitals. Secondary survey: in 2017–2018, we sent case surveys to 31 hospitals (45 cases of genetic diagnosis), who responded in the primary survey. We asked for: 1) perinatal history, 2) developmental history, 3) head MRI findings, 4) neurophysiological findings, 5) thyroid function tests, and 5) genetic test findings.ResultsWe estimated the prevalence of MCT8 deficiency to be 1 in 1,890,000 and the incidence of MCT8 deficiency per million births to be 2.12 (95 % CI: 0.99–3.25). All patients showed severe psychomotor retardation, and none were able to walk or speak. The significantly higher value of the free T3/free T4 (fT3/fT4) ratio found in our study can be a simple and useful diagnostic biomarker (Our value 11.60 ± 4.14 vs control 3.03 ± 0.38). Initial white matter signal abnormalities on head MRI showed recovery, but somatosensory evoked potentials (SEP) showed no improvement, suggesting that the patient remained dysfunctional.ConclusionFor early diagnosis, including in mild cases, it might be important to consider the clinical course, early head MRI, SEP, and fT3/fT4 ratio.