[Neurological sequelae of acute encephalopathy with febrile convulsive status epilepticus].

OBJECTIVE: The clinical characteristics of neurological sequelae in patients with acute encephalopathy with febrile convulsive status epilepticus (AEFCSE) was elucidated. METHODS: We retrospectively reviewed 8 patients, which were admitted to our hospital from 2002 to 2011. RESULTS: In the subacute phase, transient neurological symptoms, such as dystonia (n = 3), choreoathetosis (n = 2), oral tendency (n = 5) and unilateral spatial neglect (n = 6), appeared from 3 weeks after onset. Then, severe intellectual disability (n = 7), attention deficit (n = 7), disturbance of communication skill (n = 7) and emotional disturbance (n = 2), persisted from one month after onset. Although seven patients resumed ambulatory abilities, six exhibited unstable gait without ataxia or muscular weakness. The neuroradiological findings on MRI corresponded to the clinical course. In the subacute phase, reversible bilateral signal changes were noted in the subcortical white matter (n = 8), caudate nuclei (n = 2), putamen (n = 1) and thalamus (n = 1). In the chronic phase, diffuse cortical atrophy, predominantly in the fronto-temporal lobes. Diffuse cortical atrophy suggested that the persistent neurological sequelae of AEFCSE represent cortical dysfunction. Therefore, we propose that the unstable gait in our patients was gait ataxia, being related to the frontal lobe dysfunction. CONCLUSIONS: These neurological findings of AEFCSE showed characteristic temporal changes, which should be considered in the development of rehabilitation programs.

Prevalence and clinical characteristics of children with medical complexity in Tottori Prefecture, Japan: A population-based longitudinal study.

OBJECTIVES: To investigate the prevalence and background of children with medical complexity (CMC) and its secular trend in Japan. METHODS: CMC were defined as patients under the age of 20 years requiring medical care and devices. The patients were enrolled using the national health insurance claims data of three hospitals and two rehabilitation centers in Tottori Prefecture. The study period was divided into three periods: Period 1, 2007-2010; Period 2, 2011-2014; and Period 3, 2015-2018. RESULTS: A total of 378 CMC were enrolled. The prevalence of CMC was 1.88 per 1000 population among subjects aged <20 years in 2018, and it increased by approximately 1.9 times during the study period. The number of CMC who presented with severe motor and intellectual disabilities did not change from Period 1 to Period 3. Meanwhile, the number of CMC who had relatively preserved motor and intellectual abilities increased from 58 to 98. The proportion of CMC who required respiratory management and oxygen therapy increased by 1.3 and 1.8 times, respectively. By contrast, the proportion of CMC who need tube feeding decreased significantly between periods 1 and 3 (P < 0.05). CONCLUSIONS: The prevalence of CMC increased almost twice during the 12-year study period; however, the increase in the number of patients with relatively preserved motor and intellectual abilities was pronounced. This study showed that the need for medical care and devices differed based on the underlying disorders and severity of CMC; therefore, individualized medical, welfare, and administrative services and education about the various types of CMC must be provided.

Japanese clinical guidelines for juvenile orthostatic dysregulation version 1.

This clinical practice guideline provides recommendations for the assessment, diagnosis and treatment of school-aged children and juveniles with orthostatic dysregulation (OD), usually named orthostatic intolerance in USA and Europe. This guideline is intended for use by primary care clinicians working in primary care settings. The guideline contains the following recommendations for diagnosis of OD: (i) initial evaluation composed of including and excluding criteria, the assessment of no evidence of other disease including cardiac disease and so on; (ii) a new orthostatic test to determine four different subsets: instantaneous orthostatic hypotension, postural tachycardia syndrome, neurally mediated syncope and delayed orthostatic hypotension; (iii) evaluation of severity; and (iv) judgment of psychosocial background with the use of rating scales. The guideline also contains the following recommendations for treatment of OD on the basis of the result of an orthostatic test in addition to psychosocial assessment: (i) guidance and education for parents and children; (ii) non-pharmacological treatments; (iii) contact with school personnel; (iv) use of adrenoceptor stimulants and other medications; (v) strategies of psychosocial intervention; and (vi) psychotherapy. This clinical practice guideline is not intended as a sole source of guidance in the evaluation of children with OD. Rather, it is designed to assist primary care clinicians by providing a framework for decision making of diagnosis and treatments.

A Study on the Incidence and Comorbidities of Autism Spectrum Disorders Accompanied by Intellectual Disabilities in Yonago City, Japan.

BACKGROUND: Autism spectrum disorders (ASD) with intellectual disabilities may be associated with many factors. This study focused on patients with ASD with intellectual disabilities, defined by a threshold intelligence quotient (IQ) or development quotient (DQ) of 70. We also discuss comorbidities and other factors related to ASD. METHODS: We extracted case records of patients born between April 1995 and March 2001 who lived in Yonago City, as of January 2011, and had visited the two specialist institutions for consultation regarding developmental issues. The list was further narrowed down to patients identified, as having ASD by pediatric neurologists based on Diagnostic and Statistical Manual of Mental Disorders (Fifth Edition) (DSM-5). We selected patients with < IQ/DQ 70 using the most recent intelligence/development test to determine comorbidities and other factors related to ASD. RESULTS: The data of 81 patients (59 males and 22 females) were extracted, corresponding to an incidence of 76.2 patients out of every 10,000 births. The male-to-female ratio was 2.7:1. Comorbidities and related factors of ASD were observed in 25 cases (30.9%). Eleven cases had perinatal abnormalities. Other abnormalities were observed in 17 cases, including epilepsies in 7, chromosomal abnormalities in 4, familial mental retardation in 1, and acquired brain injury in 1. CONCLUSION: It is important to treat and support individuals with ASD and intellectual disabilities taking into account the characteristics and prognosis of the comorbidities and related factors.

[Study of situational recognition of attention deficit/hyperactivity disorders, Asperger's disorder and high functioning autism with the Metaphor and Sarcasm Scenario Test (MSST)].

The purpose of this study is to clear up the difference between Asperger's disorder (AS) and high functioning autism (HFA) with a newly developed test, the metaphor and sarcasm scenario test (MSST) can be used to distinguish pervasive developmental disorder (PDD) from attention deficit/hyperactivity disorders (AD/HD). So 66 AS children, 20 HFA children and 37 AD/HD children were selected, compared the profiles of score on the MSST. The results showed that the inability to understand sarcastic situation was specific to group with AS, both group with HFA and AD/HD were differ little to understand between metaphor and sarcasm. In this study suggest differences in situational recognition among the PDD subtypes, the clinical symptoms of PDD reflects characteristics of AS.

The metaphor and sarcasm scenario test: a new instrument to help differentiate high functioning pervasive developmental disorder from attention deficit/hyperactivity disorder.

It is sometimes difficult to discriminate high functioning pervasive developmental disorders (HFPDD) from attention deficit/hyperactivity disorders (AD/HD) in young children because of the behavioral similarities between the two. For adequate diagnosis, understanding fundamental differences in their social cognitive abilities might become significant. In order to detect the differences in social cognitive abilities between AD/HD and HFPDD, a new test, the Metaphor and Sarcasm Scenario Test (MSST) was developed. One hundred and ninety-nine normal school children (the control group), 29 AD/HD children and 54 HFPDD children were involved. The results showed that the inability to understand a sarcastic situation was specific to children with HFPDD, both children with AD/HD and HFPDD could not equally understand metaphor. The correlation between the comprehension of sarcasm and success in the theory of mind task was remarkably high but not for comprehension of metaphor. In conclusion, the MSST has the potential to discriminate HFPDD from AD/HD in young children.

[Epilepsy in patients with cerebral palsy--analysis of frequency and clinical prognosis].

This study was designed to investigate the incidence and prognosis of epilepsy in 109 patients with cerebral palsy and to attempt to correlate these clinical data with the type of palsy. The incidence of epilepsy, the onset of age and the type of first seizure were associated with the regions affected by palsy. A good association exists between tetraplegia and age-dependent epileptic encephalopathy. In patients with cerebral cortical lesions demonstrated by radiological examination, the incidence of epilepsy was significantly increased. The prognosis of epilepsy is not related to the type of palsy. In spastic palsy, the patients with epilepsy showed more severe intellectual disabilities.