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We herein report a 70-year-old man with malaise and muscle weakness that had developed within a month. The patient also had abdominal fullness due to polycystic kidney disease. Severe proximal skeletal muscle weakness and mild elevation of creatinine kinase to 301 IU/L were noted. A muscle biopsy of the right bicep showed polymyositis. Computed tomography showed a right renal mass, and an analysis after right nephrectomy identified clear cell carcinoma. The muscle weakness subsided one month after nephrectomy and intravenous immunoglobulin therapy. Therefore, we suspect that the development of polymyositis in this patient was closely related to renal cell carcinoma.

Inclusive education is a process in which schools devise curricula and plan resource allocation to include the diverse group of children in society. Although not many schools promote inclusive education at a school-wide level, there are some schools making efforts toward inclusion in Japan. This study aimed to understand the process of inclusion in a Japanese elementary school by using Trajectory Equifinality Modeling. We conducted a participant observation and focused on Naomi, who was 7 years old and had Down's syndrome. Naomi spent more than 80% of her time in the general education classroom, where she began to refuse to do tasks different from other children. In regard to this, the opinions of the homeroom teacher and the coordinator of these classes were completely different, but their opposing opinions were transformed into complementary ones using time differentiation, and they were able to continue to collaborate toward inclusive education. We found "transductive resolution" as a key to understanding the processes of striving for inclusive education.

The effects of school refusal on one’s life depend on how the adolescent lives their life after refusing school. Previous studies have focused on meaning making for past refusal and have not adequately addressed the changes and transitions after school refusal. The current study elaborates on the richness of the transitions of adolescents who refused to go to school, based on the socio-cultural psychology of life course. By looking at school refusal from the perspective of the life course theory, we found that school refusal was a process of questioning normality. In addition, by using Yamada’s relational model of people and environments (Yamada, 1987, 2010), we found there are three patterns in the adolescents’ transitions: a) expanding their involvement in the here and now by encountering resources and then expanding reality, b) reforming relationships in the here and now by representing an other’s world and then reconstructing reality, and c) blocking their involvement in the here and now by touching resources and then escaping from reality. We discuss formation of resistance and richness of transition which would bring richness to our lives.

In collaboration between researchers and practitioners from Japan and Denmark, we applied a theoretical framework drawn from situated learning theory on the topic of dropout from education. We analysed cases of students at risk of dropout from high school, of a high school’s efforts to involve its students in the local community, and from social work with youths at risk of dropout. Throughout our analysis, we argue in favor of including a broader perspective where the whole life of the adolescent is considered in relation to dropout, since factors outside the educational context can be very influential. Prior research on dropout has pointed to the importance of seeing dropout as a gradual process involving the identity development of the adolescent. The analysis also underlines the importance of students being full members of their communities: Part of the dropout problem can be conceptualised as community vulnerability on the part of the student, in the sense that these adolescents are unusually vulnerable concerning inclusion in their communities. These analyses have led to novel understandings, and in this way, new methods to help students avoid dropping out. This chapter demonstrates the benefits of exchanging experience and knowledge among practitioners and researchers as well as across countries.

The diagnosis of neurological symptoms in patients with systemic lupus erythematosus (SLE) is often challenging, in part because they sometimes mimic features of multiple sclerosis (MS). Herein we report a case of a young female who presented with relapsing-remitting symptoms of unilateral visual loss and motor aphasia. Additionally, radiological findings revealed multiple white matter lesions on her brain that led to an initial diagnosis of MS based on the established diagnostic criteria. However, she was eventually diagnosed with neuropsychiatric SLE (NPSLE) presenting with extracranial internal carotid artery (ICA) occlusion. Her ICA occlusion had not been detected for 2 months until she underwent magnetic resonance angiography. Although exact underlying pathological mechanisms are unclear, both the ICA occlusion and MS-like brain white matter lesions could be attributed to SLE. This case demonstrated that both of these lesions can coexist in the same patient, suggesting that NPSLE with ICA occlusion can be a potential mimicker of MS, and vice versa. Care must be paid to avoid delay in the diagnosis.

The diagnosis of longus colli tendinitis (LCT) is sometimes challenging, especially when laboratory data show marked inflammation and neuroimaging studies do not indicate calcification within the tendon of the longus colli muscles. We herein report a case of LCT that presented with elevated inflammation parameters without calcification on imaging. Findings characteristic of LCT, such as prevertebral hyperintensity areas on T2-weighted images and no signs of purulent diseases, informed our diagnosis of LCT. Enhanced computed tomography and magnetic resonance imaging are useful procedures when diagnosing LCT after ruling out other critical purulent diseases.

The current paper focuses on the various types of laughter recorded during real social interactions in a virtual immersive environment. With this experiment, we investigate whether human beings are able to discriminate social from spontaneous laughter on the basis of auditory or audiovisual laughs presented outside any context. Towards this aim, we carried out two perceptual experiments proposing audio alone and audiovisual conditions, which were taken by French and Japanese native subjects. Each subject listened to (or looked at) 162 laughs and chose one response among three possibilities: social, spontaneous or unknown. The results of both experiments show that all participants are able to discriminate these two types of laughter with quite good confidence without contextual information: the correct identification rate for spontaneous laughter is about 70% with a similar amount for social laughter in audio alone and audiovisual conditions. We then extracted acoustic characteristics for each laugh in order to investigate potential differences between the two types of laughter. A multifactorial analysis showed that perceptual behaviors and some acoustic features (F0 and duration) are correlated. Especially, we observe a significant difference between social and spontaneous laughter through the features of total duration and voiced duration. Finally, we conducted a perceptual experiment on the subcategorization of social laughs based on three social factors: speaker’s physical state, speaker’s involvement and psychological distance. The results show that social laughter is characterized by similar contexts for both groups of listeners, except by Japanese subjects who regard the psychological distance between partners as distant. O presente artigo enfoca os diversos tipos de riso registrados durante interações sociais reais realizadas em um ambiente de imersão virtual. Neste experimento, investigamos se os seres humanos são capazes de discriminar risos sociais de risos espontâneos e gargalhadas audiovisuais, sem terem acesso a nenhum contexto de produção do riso. Para tal, realizamos dois experimentos perceptuais considerando apenas o estímulo auditivo, por um lado, e o estímulo audiovisual, por outro. Os sujeitos dos testes de percepção são falantes nativos franceses e japoneses. Cada sujeito ouviu e ouviu e viu 162 risadas e escolheu uma resposta entre três possibilidades: social, espontânea ou irreconhecível. Os resultados de ambos os experimentos mostram que todos os participantes são capazes de discriminar esses dois tipos de riso com um grau de confiança bastante elevado, mesmo sem as informações contextuais: a taxa de identificação correta para o riso espontâneo é de cerca de 70%, com uma quantidade semelhante de riso social considerando tanto os estímulos visuais quanto os audiovisuais. A seguir, foram extraídas medidas acústicas para cada risada, a fim de investigar possíveis diferenças entre os dois tipos de riso. A análise multifatorial demonstra que os comportamentos perceptuais e algumas características acústicas (F0 e duração) estão correlacionados. Observarmos, particularmente, uma diferença significativa entre o riso social e o riso espontâneo através dos traços da duração total e da duração do vozeamento. Por último, realizamos mais um experimento perceptual sobre a subcategorização dos risos sociais com base em três fatores sociais: estado físico do falante, envolvimento do falante e distância psicológica. Os resultados mostram que o riso social é evocado de forma semelhante no que diz respeito ao estado físico do falante e envolvimento do falante. Entretanto, no que diz respeito à distância social, há divergência de percepção entre franceses e japoneses sendo que nestes últimos predomina a percepção da distância psicológica.

A 70-year-old man with myotonic dystrophy (MD) showed repetitive vomiting and decreased food ingestion. These symptoms were caused by acute mass of steak impaction occluding the esophagus, known as “steakhouse syndrome,” which may have occurred in response to esophageal functional changes following gastrointestinal involvement due to MD pathology. The occluding food was successfully removed endoscopically, and his symptoms resolved without relapse. Our case suggests that MD patients can present with “steakhouse syndrome” due to bolus food impaction occluding the esophagus as one of their gastrointestinal manifestations, which underscores the need for its consideration in MD patients presenting with similar symptoms.

Objectives Impaired renal function is a risk factor for cefepime (CFPM)-induced encephalopathy (CFPMIE) in patients treated with CFPM; dose-titration to renal function is recommended to prevent CFPMIE. However, available evidence on the incidence of CFPMIE or preventive efficacy of dose adjustment against CFPMIE in end-stage renal disease (ESRD) patients is limited. Methods Single-centre, retrospective observational study. We reviewed consecutive in-hospital adult patients treated with adjusted-dose of CFPM in the period between September 2012 and September 2016, and assessed the CFPMIE in ESRD patients treated with adjusted-dose of CFPM. Results Out of 422 eligible patients, 6 patients (1.4%) were diagnosed with CFPMIE. The incidence of CFPMIE in ESRD patients was 7.5% (5/67). Among ESRD patients, pre-existing central nervous system (CNS) morbidity was significantly associated with the risk of CFPMIE. CFPMIE occurred in ESRD patients regardless of daily dose, and even with 0.5 g/day of CFPM. Conclusions Pre-existing CNS morbidity may be associated with an increased risk of CFPMIE in ESRD patients. No significant association was observed between CFPM dose and incidence of CFPMIE in ESRD patients, and future investigation on the safer dose-adjustment strategy in ESRD patients is required for achieving balance between successful infectious treatment and reducing CFPMIE.

Serum antibodies to GQ1b are associated with Miller Fisher syndrome (MFS) and Guillain-Barré syndrome (GBS) with ophthalmoplegia. Antibodies to ganglioside complexes (GSCs) have not yet been examined in a large population of patients with MFS or GBS. This study aimed to determine the clinical significance of antibodies to GSCs in MFS and GBS.The study investigated serum anti-GSC antibodies and the clinical features in 64 MFS patients, 53 GBS patients with ophthalmoplegia (GBS-OP(+)) and 53 GBS patients without ophthalmoplegia (GBS-OP(-)).Thirty patients with MFS (47%), 25 with GBS-OP(+) (47%) and none with GBS-OP(-) had antibodies to GSCs containing GQ1b or GT1a. Patients with MFS and GBS-OP(+) were subdivided according to the antibody reactivities; patients with antibodies specific to GQ1b and/or GT1a (without anti-GSCs antibodies) were placed in Group 1, those with antibodies against GSCs with a total of two sialic acids in the terminal residues, such as GQ1b/GM1, were placed in Group 2, and those with antibodies against GSCs with a total of three sialic acids in the terminal residue, such as GQ1b/GD1a, were placed in Group 3. In MFS, sensory disturbances were infrequent in Group 2 compared with the other groups (p0.0001). Antibodies specific to GQ1b were observed more often in MFS than in GBS-OP(+) (p = 0.0002).IgG antibodies to GSCs containing GQ1b or GT1a were closely associated with the development of ophthalmoplegia in GBS, as well as MFS. Both GQ1b and clustered epitopes of GSCs containing GQ1b or GT1a may be prime target antigens for MFS and GBS-OP(+).

Ganglioside complexes (GSCs) are known as target antigens in Guillain-Barré syndrome (GBS). To elucidate the clinical importance of the anti-GSC antibodies in GBS, we investigated serum antibodies to GSCs containing two of the gangliosides, GM1, GD1a, GD1b and GT1b, and analyzed clinical features of anti-GSC-positive GBS patients. Thirty-nine (17%) of 234 GBS patients had IgG anti-GSC antibodies. Anti-GSC-positive GBS had antecedent gastrointestinal infection and lower cranial nerve deficits more frequently than control GBS. The presence of antibody specificity to GD1a/GD1b and/or GD1b/GT1b was significantly associated with severe disability and a requirement for mechanical ventilation.

Background: Some ganglioside complexes (GSCs) are target antigens for serum antibodies in patients with Guillain–Barre syndrome (GBS). Anti-GSC antibodies may be associated with particular clinical features of GBS. Objective: To investigate antibodies to GSCs in the sera of patients with Miller Fisher syndrome (MFS) characterised by elevation of the IgG anti-GQ1b antibody. Results: In all, 7 of 12 (58%) consecutive patients with MFS were found to have IgG antibodies to GSCs containing GQ1b, of whom 5 had IgG antibodies to GQ1b-GM1 complex (GQ1b/GM1) and 2 had antibodies to GQ1b/GD1a; 4 of 5 patients without sensory symptoms had anti-GQ1b/GM1 antibodies. Conclusions: At least three different specificities in MFS-associated antibodies, GQ1b-specific, anti-GQ1b/GM1-positive and anti-GQ1b/GD1a-positive, were observed. In patients with MFS not only GQ1b itself but also clustered epitopes of GSCs, including GQ1b, may be considered to be prime target antigens for serum antibodies. A tendency to escape sensory disturbances is shown by anti-GQ1b/GM1-positive MFS.

Antibodies specific for a complex of gangliosides GD1a and GD1b (GD1a/GD1b) were found in sera from eight of 100 patients with Guillain-Barre syndrome (GBS) by the use of enzyme-linked immunosorbent assay and thin-layer chromatogram immunostaining. Those sera also had antibody activities to such ganglioside complexes as GD1a/GM1, GD1b/GT1b, and GM1/GT1b but had little or no reactivity to the each isolated antigen. Clustered epitopes of the ganglioside complex in the plasma membrane may be targeted by such an antibody, and interaction between the antibody and ganglioside complex may induce the neuropathy.

Selegiline protects nigral dopaminergic neurons and is recommended for the treatment of patients in the early stage of Parkinson's disease (PD). We treated 112 PD patients and noted that those given selegiline had a high incidence of hallucination. Our objective was to determine which clinical therapeutic factors cause such hallucinations. The Kruskal-Wallis and chi-square test showed that in 94 patients, the severity of the hallucinations was significantly related to the duration of illness, Hoehn and Yahr stage, doses of levodopa and cabergoline, whether or not selegiline was used, and whether or not medication for constipation was required. In addition, patients who were treated with a low dose of levodopa (< or =300 mg/day), who had a low Hoehn and Yahr stage, and a short duration of illness (< or =8 years) together with a high dose of selegiline or cabergoline also tended to have hallucinations. MRI findings were not related to the incidence of hallucination. When selegiline is given to patients who have PD of long duration and a high Hoehn and Yahr stage, and who already are receiving levodopa and a dopamine agonist, the doses of levodopa and the dopamine agonists given, as well as the presence of constipation, may be related to the incidence of hallucination.

Objectives Contact with Urtica ferox (ongaonga) causes an intense pain with subsequent sustained numbness. While all factors responsible for the initial painful response are known, the compound(s) responsible for the sustained numbness remain unidentified. There is a need for new therapeutics to treat chronic pain and the yet-to-be identified compounds in Urtica ferox may provide a novel topical treatment for chronic pain. Methods A collection expedition in Kahurangi National Park, New Zealand, was conducted. Urtica ferox trichomes were collected and a water extraction performed. Primary hippocampal neurons and NT2 cells were treated with extract to characterise toxicity. A small animal model was used to assess functional response to extract exposure. Results The extract of Urtica ferox induces a dose-dependent selective toxicity to neurons with morphologic characteristics of apoptosis. Evaluation of extract exposure in the small animal model suggest a direct and specific, effect on neurons. Preliminary characterisation of the active compound indicates a relatively small entity with phenolic ring structure. Conclusions Initial evaluations of the Urtica ferox extract indicate the presence of a compound(s) with promising characteristics. Specifically, a compound that selectively effects neurons that is small and water soluble. This unidentified compound will be characterised using metabolomics.

Since the development of effective antibiotic therapy, the occurrence of neurosyphilis has become less frequent. The number of syphilitic patients is gradually increasing as a complication in acquired immunodeficiency syndrome, but the diagnosis of neurosyphilis sometimes is difficult. We describe six patients with neurosyphilis and an analysis of their tibial nerve somatosensory evoked potentials. Four of them, including two with no tabes dorsalis symptoms, had delayed P15-N21 or the absence of N21. These abnormalities were ameliorated by treatment for syphilis. Analysis of tibial nerve SEPs provides a useful tool for the diagnosis of neurosyphilis and the evaluation of the extent to which neurosyphilis has progressed.

We report two cases of idiopathic carotid-cavernous fistula (CCF) with primary symptoms of headache and diplopia. A 47-year-old woman presented with throbbing headache in her right frontal region followed by right trochlear nerve palsy. Brain magnetic resonance imaging (MRI) was normal but magnetic resonance angiography (MRA) and computed tomographic angiography (CTA) revealed abnormal signals around the right cavernous sinus. CCF was diagnosed by conventional angiography. The symptoms improved naturally but after about 1 year she suddenly exhibited conjunctival congestion. A 41-year-old man complained of fluctuating headache with sudden left abducens nerve palsy. MRI and MRA were normal but CTA showed abnormal signals around the left cavernous sinus. A final diagnosis of CCF was made by conventional angiography and he was transferred to another hospital for stereotactic radiosurgery. Diagnosis of CCF tends to be delayed in cases presenting with only headache and external ophthalmoplegia. However, CCF with cortical vein drainage can lead to cerebral hemorrhage and early correct diagnosis is needed. Our cases showed a dilated superior ophthalmic vein in enhanced CT and an abnormal signal around the cavernous sinus in CTA. Therefore, CTA may be useful as a relatively non-invasive method that can provide diagnostic clues for CCF.

A 57-year-old woman who had severe opticospinal multiple sclerosis (OSMS) was admitted to our hospital. She had presented with visual loss and gait disturbance at the age of 48, and had since experienced more than 10 relapses and been hospitalized 9 times. Interferon beta-lb treatment (8,000,000 units on alternate days) had been started at her last admission. Prior to the present admission, she had developed left visual loss and gait difficulty after headache lasting a few days. Cerebrospinal fluid showed elevation of cell count (322/mm3), protein (130 mg/dl), and myelin basic protein (462 pg/ml; normal,102 pg/ml). On examination, she exhibited decreased left visual acuity, paraplegia, ataxia of the right upper extremity, and sensory disturbance, particularly in the lower extremities. Bowel and bladder disturbances were also evident Laboratory testing showed lymphocytopenia (420/microl), compared to a white cell count of 1700/microl just before initiation of interferon beta-lb1therapy. MRI revealed a new lesion in the cerebellum in addition to small T2-hyperintense lesions in the white matter of the brain;these had been noted previously. Interferon beta-1b therapy was ceased and she was treated using methylprednisolone pulse therapy. After the abnormal findings resolved, however, interferon beta-lb1therapy was restarted. Three months after, she exhibited right hemiparesis without facial palsy concurrent with lymphocytopenia. MRI showed T2-hyperintense lesions in the periventricular white matter, left cerebral peduncle, bilateral middle cerebellar peduncles, and right cerebellar hemisphere. We reduced the doses of interferon beta-lb1immediately. Thereafter, she did not have relapse for 29 months, but her EDSS (expanded disability status scale) has not recovered. Although interferon beta-lb1has been recognized as an effective drug for decreasing the relapse rate and severity of both secondary progressive MS and relapsing-remitting MS, the present case showed the possibility of interferon beta-1b being associated with phenotype change from OSMS to conventional MS (CMS).

Antibodies specific for a complex of gangliosides GD1a and GD1b (GD1a/GD1b) were found in sera from eight of 100 patients with Guillain-Barre syndrome (GBS) by the use of enzyme-linked immunosorbent assay and thin-layer chromatogram immunostaining. Those sera also had antibody activities to such ganglioside complexes as GD1a/GM1, GD1b/GT1b, and GM1/GT1b but had little or no reactivity to the each isolated antigen. Clustered epitopes of the ganglioside complex in the plasma membrane may be targeted by such an antibody, and interaction between the antibody and ganglioside complex may induce the neuropathy.

Compared with 87 unventilated patients with Guillain-Barre syndrome (GBS), 44 ventilated patients with GBS more frequently had multiple cranial nerve involvement (91 vs 50%; p < 0.001) and IgG anti-GQ1b antibody (27 vs 8%; p = 0.006). In GBS patients without ophthalmoparesis, the presence of IgG anti-GQ1b antibody was associated with respiratory failure (12 [3/25] vs 0% [0/67]; p = 0.04). The presence of the antibody may be a factor predictive of respiratory failure in GBS.

We presented the case of a 58-year-old woman with spinal sarcoidosis at the C4-C6 level. The lesion itself as visualized with magnetic resonance imaging, and its neurological findings responded favorably to treatment with prednisolone. Comparison of pre- and post-treatment values for somatosensory evoked potential (SEP) and central motor conduction time (CMCT) also showed improvement. Analysis of SEP and CMCT can be a useful tool for following up spinal sarcoidosis.

Several reports have described the seasonal variation of multiple sclerosis (MS) attacks in the European countries and in the US. Some have insisted that attacks occurred more frequently in winter or spring. We investigated the possibility of a seasonal variation in the frequency of MS attacks among patients in Japan. A total of 172 MS exacerbations in 34 MS patients were analyzed retrospectively. Attacks were divided into two groups: opticospinal type and brain type. The 12 months of the year were assigned to 6 groups based on average monthly temperature. Of the 172 MS exacerbations, 123 were opticospinal type and 49 were brain type of attacks. The total number of attacks was significantly more frequent in the warmest (July and August) and coldest (January and February) months. The heat of summer in warmer, low latitude areas may be a risk factor for MS attacks.