Hemiconvulsion–hemiplegia syndrome in a patient with severe myoclonic epilepsy in infancy

We report a 2-year-old girl who had repeated febrile or afebrile seizures since infancy. Prolonged left/right hemiconvulsions and myoclonus of the eyelids/extremities with generalization to tonic–clonic seizures, were refractory to antiepileptic agents. At age 1 year and 4 months, she contracted rotavirus infection, and developed status epilepticus with persistent right hemiclonic seizures. Left unilateral brain edema with subsequent emergence of cortical laminar necrosis and white matter lesions, and progressive atrophy of the left cerebral hemisphere were noted during this period. She showed residual right hemiparesis and mild intellectual disability, and had generalized/eyelid myoclonia and hot water epilepsy after a 5-month seizure-free period. Analysis for SCN1A , the gene encoding the neuronal voltage-gated Na⁺ channel α1 subunit revealed a nonsense mutation, R1892X. These indicate the potential risk in patients with severe myoclonic epilepsy in infancy (SMEI) to develop hemiconvulsion–hemiplegia (HH) syndrome. SCN1A mutations may need to be further explored in patients with HH syndrome without features of SMEI.     

Permanent waving does not change mercury concentration in the proximal segment of hair close to scalp

Mercury in hair is a generally accepted biomarker of methylmercury exposure, and permanent waving has been reported to affect the mercury concentration in hair. We conducted an experimental-field study to examine the changes in the mercury concentration in hair induced by treatments such as permanent waving, straightening and coloring. Hair samples were collected from 19 female subjects enrolled before and after hair treatment by a beautician during each visit to a beauty saloon. A total of 38 pair samples were cut in 1-cm segments from the proximal end up to 10 cm, and then as 2-cm segments up to the distal end thereafter. Each segment was analyzed for total mercury concentration by cold-vapor atomic absorption spectrometry. Permanent waving decreased mercury concentration for most of the segments except for the proximal two segments and the 8-9 cm segment from the proximal end. Nevertheless the average mercury concentration of 3-cm segments from the proximal end showed no significant decrease by permanent waving. Since females usually have hair longer than 3 cm, hair samples subjected to permanent waving may give lower mercury exposure estimates when the full-length hair strands are analyzed. However, analyzing the proximal 3-cm segment of hair samples does not give lower mercury exposure estimates. Assuming that hair samples are collected from puerperal women around the time of delivery, the 3-cm segments represent fetal exposure to methylmercury during the third trimester when fetuses are most vulnerable to methylmercury exposure. Therefore, mercury concentrations in the proximal segment of maternal hair collected in the right time can be a good biomarker of fetal methylmercury exposure.     

Active c-jun N-terminal kinase induces caspase cleavage of tau and additional phosphorylation by GSK-3beta is required for tau aggregation

Neurofibrillary tangles (NFTs), comprising human intracellular microtubule-associated protein tau, are one of the hallmarks of tauopathies, including Alzheimer's disease. Recently, a report that caspase-cleaved tau is present in NFTs has led to the hypothesis that the mechanisms underlying NFT formation may involve the apoptosis cascade. Here, we show that adenoviral infection of tau into COS-7 cells induces activation of c-jun N-terminal kinase (JNK), followed by excessive phosphorylation of tau and its cleavage by caspase. However, JNK activation alone was insufficient to induce sodium dodecyl sulfate (SDS)-insoluble tau aggregation and additional phosphorylation by GSK-3β was required. In SH-SY5Y neuroblastoma cells, overexpression of active JNK and GSK-3β increased caspase-3 activation and cytotoxicity more than overexpression of tau alone. Taken together, these results indicate that, although JNK activation may be a primary inducing factor, further phosphorylation of tau is required for neuronal death and NFT formation in neurodegenerative diseases, including those characterized by tauopathy.     

Prevalence of and risk factors for suicide-related outcomes in the World Health Organization World Mental Health Surveys Japan

Aim:  Suicide is a major public health concern in Japan but little is known about the prevalence of and risk factors for suicidal ideation, plans, and attempts. The aim of the present study was to clarify the prevalence of and risk factors for important suicide-related outcomes.
Methods:  Important suicide-related outcomes and risk factors were assessed in face-to-face interviews with 2436 adult respondents in seven areas as part of the World Health Organization (WHO) World Mental Health Survey Initiative. Mental disorders were assessed with the WHO Composite International Diagnostic Interview (CIDI).
Results:   The lifetime prevalence estimates of suicidal ideation, plans, and attempts were 10.9%, 2.1%, and 1.9%, respectively. Risk of suicide plans and attempts was highest when suicidal ideation occurred at an early age and within the first year of ideation. In middle-aged individuals, the period after first employment and the presence of mental disorders were risk factors.
Conclusions:  Risk of suicide plans and attempts is highest when suicidal ideation occurred at an earlier age and within the first year of ideation. Mental disorders are as predictive of the suicide-related outcomes examined here, and comorbidity is an important predictor.     

Multidisciplinary treatment of lung cancer in 21st century

Lung cancer is the leading cause of cancer deaths in Japan. Recently, big progress in the treatment of lung cancer has been achieved, such as new anti-cancer drugs, molecular targeted therapy, stereotactic radiotherapy, etc. Multidisciplinary approach has been required to the therapy for lung cancer patients. In this paper, we introduce The 21st Century Multidisciplinary Center in Kanazawa Medical University, and the Hokuriku Training Program for Making Specialists in Cancer Treatment.     

Carotid atherosclerosis is associated with brain atrophy in Japanese elders

BACKGROUND: The relation between atherosclerosis and brain atrophy remains unclear in patients with risk factors for cardiovascular diseases. OBJECTIVE: This study was performed to clarify the relation between brain atrophy and carotid atherosclerosis. METHODS: A total of 142 patients (78 women and 64 men, mean age 74 years) with no neurologic disturbances were studied. Brain atrophy was evaluated on the basis of the brain atrophy index (BAI, BAI = brain parenchyma/intracranial space A 100%), calculated by means of digitized computed tomographic scans obtained at the level of the basal ganglia. Carotid atherosclerosis was evaluated on the basis of the plaque score (PS), defined as the sum of all plaque heights in both carotid arteries, intima-media thickness (IMT), and vessel diameter (VD) of the common carotid artery as assessed by ultrasonography. RESULTS: Age negatively correlated with BAI in both men (r = -0.587, p < 0.001) and women (r = -0.724, p < 0.001). PS of the carotid artery also negatively correlated with BAI in men (r = -0.502, p < 0.001) as well as women (r = -0.480, p < 0.001). VD and IMT of the right carotid artery negatively correlated with BAI in women (VD; -0.256, p < 0.05, IMT; -0.216, p < 0.05) but not in men. Other characteristics were unrelated to BAI. Multiple regression analysis showed that age and PS were independent predictors of brain atrophy in both sexes. The percentage of variance of BAI values explained by this model in women (51.9%) was much greater than that in men (35.5%). CONCLUSION: Carotid atherosclerosis may be a useful morphological index of brain atrophy.     

Much communication with family and appetite among elderly persons in Japan

The purpose of this study is to examine cross-sectionally the factors associated with appetite among the elderly persons living in the community in Japan. The subjects of this study were 754 non-institutionalized elderly men and women aged 65 years and older living in community in Japan. The data were collected by face-to-face interviews. The factors related to subjective health was examined using multiple logistic regression analyses. Four hundred and forty-nine (56.4%) participants evaluated appetite as very good; 239 (30.0%) as somewhat good; 92 (11.6%) as little/not at all. Subjects with good appetite were more physically active, had less depressive symptoms, subjective stress, much more communication with family and much more social support, as compared to those without appetite. Appetite was significantly associated with better subjective good health after controlling for other variables in the model. Only communication with family was significantly associated with good appetite after controlling for other variables (odds ratio, OR=2.43; 95% confidence interval, CI=1.30-4.74). The indirect effect of communication with family on appetite was much greater than the direct effect. These findings suggest that communication with family may be beneficial promoter of the appetite in the elderly.