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Background Takayasu’s arteritis is extremely rare in children aged below 6 years. At the onset of Takayasu’s arteritis in children, symptoms are varied but differ from those in adults. Corticosteroids are the mainstay of treatment for preventing irreversible vascular damage but there is no standard treatment for progressive vascular stenosis. Case presentation A Japanese 11-month-old baby boy presented with Takayasu’s arteritis and heart failure, possibly due to afterload mismatch caused by high blood pressure. Computed tomography was performed and revealed thoracic and abdominal aortic aneurysms. It also revealed severe celiac artery stenosis and bilateral renal artery stenosis. Prednisolone was initiated as first-line therapy. The fever resolved, and C-reactive protein levels returned to normal. Although his general condition improved, deterioration of vascular lesions was evident. Celiac artery occlusion, severe right renal artery stenosis, and new superior mesenteric artery stenosis were observed. We decided to use a continuous infusion of lipo-prostaglandin E1 for prevention of branch stenosis of his abdominal aorta. The progression of vascular stenosis was stopped and our patient’s cardiac function gradually improved. Conclusions A differential diagnosis of heart failure with high blood pressure should be considered in babies. The progression of vascular stenosis may be suppressed by lipo-prostaglandin E1.

Background There is a paucity of studies on self-assessed generic health-related quality of life (HRQOL) in children with epilepsy. The purpose of this study was to investigate generic HRQOL and associated factors among Japanese children with epilepsy. Methods In this clinic-based study, 277 children (aged 8–18 years) with epilepsy and 429 children without any chronic illnesses were recruited. HRQOL was evaluated using the Japanese version of the KIDSCREEN-52 self-reported questionnaire, which consisted of 52 items categorized into 10 dimensions related to the environment surrounding children. Multiple regression analysis was applied to explore related factors with low HRQOL in each dimension. Results We obtained the questionnaire from 171 (61.7%) and 306 (71.3%) children in the epilepsy and control groups, respectively. Short treatment period ( 30 min, and post-ictal symptoms were associated with a low HRQOL for School Environment (OR: 3.81; 95% CI: 1.34–10.86), Moods & Emotions (OR: 3.82; 95% CI: 1.67–8.78), and Parent Relations & Home Life (OR: 3.53; 95% CI: 1.29–9.72) dimensions, respectively. Complex neurodevelopmental disorders were associated with a low HRQOL for Social Support & Peers (OR: 3.59; 95% CI: 1.33–9.66), School Environment (OR: 2.49; 95% CI: 1.07–5.77), and Psychological Well-being (OR: 3.47; 95% CI: 1.20–10.00) dimensions. Conclusions Our results suggest that early psychosocial support and better management of epilepsy may improve HRQOL. More support in school environments may be required for children with epilepsy and neurodevelopmental disorders.

Field potentials were recorded on self-paced hand and mouth movements with implanted electrodes on the surface and at 2.0-3.0 mm depth in respective cortical areas in seven monkeys. Surface-negative (s-N), depth-positive (d-P) premovement slow potentials were recorded in the prefrontal cortex only when the movements were performed with an intense motivation for a reward. Such a motivation-dependent activity was mainly obtained in the rostral bank of the arcuate sulcus on both sides (except for the inferior limb of the arcuate sulcus in the left hemisphere). Three of the seven monkeys were also tasked with visuo-initiated hand movements. As motivation for reward decreased, s-N, d-P potentials at a latency of about 80 ms after stimulus onset became gradually smaller in the rostral bank of the arcuate sulcus in the right hemisphere. These facts suggest that motivation-dependent activity is represented in the dorsolateral part of the prefrontal cortex in monkeys, and that the cortical part is involved in motivational functions as well as in cognitive functions.

Background: Myelin oligodendrocyte glycoprotein (MOG) localizes on the outermost surface of the myelin sheath and oligodendrocytes in the central nervous system (CNS). Autoantibodies against MOG are reportedly found in patients with spectrum of inflammatory demyelinating diseases of the CNS, including acute disseminated encephalomyelitis, multiple sclerosis, and neuromyelitis optica. In addition, recent studies have emphasized an association between anti-MOG antibodies and optic neuritis. Patient: We present the first case report of a 7-year-old Japanese boy who was positive for anti-MOG antibodies. He experienced four episodes of unilateral optic neuritis and one seizure event. Magnetic resonance imaging revealed T2-hyperintense lesions in the subcortical white matter and midbrain. Although he fulfilled the diagnostic criteria for multiple sclerosis, recombinant interferon beta did not prevent recurrence. Established cell-based immunoassays revealed that he was positive for anti-MOG antibodies and negative for anti-aquaporin 4 antibodies. Conclusions: Our case report supports the relationship between anti-MOG antibodies and recurrent optic neuritis. Additional studies are needed to establish the clinical significance of anti-MOG antibodies for diagnosis, treatment, and prognosis.

Hypoxia plays a key role in the pathophysiology of many disease states, and expression of the retinoic acid receptor-related orphan receptor alpha (RORalpha) gene increases under hypoxia. We investigated the mechanism for this transient hypoxia-induced increase in RORalpha expression. Reverse transcription-coupled PCR analysis revealed that the steady-state level of mRNA for the RORalpha4 isoform, but not the RORalpha1 isoform, increased in HepG2 cells after 3 h of hypoxia. Transient transfection studies showed that the hypoxia-induced increase in RORalpha4 mRNA occurs at the transcriptional level and is dependent on a hypoxia-responsive element (HRE) located downstream of the promoter. A dominant-negative mutant of hypoxia-inducible factor-1alpha (HIF-1alpha) abrogates the transcription activated by hypoxia as well as the transcription activated by exogenously expressed HIF-1alpha, demonstrating the direct involvement of HIF-1alpha in the transcriptional activation. However, HIF-1 alone was not sufficient to activate transcription in hypoxic conditions but, rather, required Sp1/Sp3, which binds to a cluster of GC-rich sequences adjacent to the HRE. Deletion of one or more of these GC boxes reduced or eliminated the HIF-1-dependent transcription. Together, these results suggest that the hypoxia-responsive region of the RORalpha4 promoter is composed of the HRE and GC-rich sequences and that the transcriptional activation under hypoxia is conferred through the cooperation of HIF-1 with Sp1/Sp3.

Field potentials preceding vocalization were recorded with electrodes chronically implanted on the surface and at 2.0-3.0 mm depth in various cortical areas in three monkeys. Surface-negative, depth-positive (s-N, d-P) slow potentials prior to vocalization were recorded in the premotor (including the Broca's homology), motor and somatosensory cortices in both hemispheres, and the supplementary motor area in the cerebral hemisphere. Such premovement potentials were recorded also in the cingulate and prefrontal cortices when a monkey uttered with intense motivation for reward. Cerebellar hemispherectomy on the right side eliminated the s-N, d-P premovement potentials in the motor cortex and in the posterior bank of inferior limb of the arcuate sulcus (homolog of Broca's area) in the left hemisphere. Vocal tone contained less components in different frequencies, and its duration became more variable than before the operation. These facts suggest that the neocortical area homologous to the human speech area takes part in generation and control of monkey vocalization together with the cerebellum, possibly through cerebro-cerebellar interactions, against ideas so far offered on nonhuman primate vocalization.

Field potentials were recorded with electrodes implanted in various cortical areas while a naive monkey was learning reaction time hand movements with complex tone. When cortical surface-negative, depth-positive potential (at a latency of about 80 ms after a stimulus onset) appeared in the rostral bank of the inferior limb of the arcuate sulcus of the left cerebral hemisphere, and became gradually larger, the monkey began to respond to the stimulus with the movement. As the potential in the prefrontal (prearcuate) cortex and the cerebellar-mediated potential in the motor cortex gradually increased with further training, the movement became quicker and more skillful. Three naive monkeys achieved the movement with complex tone in shorter training days than the movement with pure tone; the movement with pure tone was not accompanied by any significant potential in the prefrontal cortex. It is deduced from the present study, and previous studies on visuo-initiated movements, that the prefrontal area, especially in the left hemisphere, plays a significant role for a monkey to associate a stimulus with appropriate motor execution.

Field potentials before vocalization were studied with electrodes chronically implanted on the surface and at a 2.0-3.0 mm depth in various cortical areas in three monkeys. Surface-negative, depth-positive (s-N, d-P) slow potentials prior to vocalization were recorded in the premotor (including the Broca's homolog), motor and somatosensory cortices, and the supplementary motor area (SMA). Such premovement potentials were recorded also in the cingulate and prefrontal cortices when a monkey uttered a sound with intense motivation for reward. Cerebellar hemispherectomy on the right side eliminated the s-N, d-P premovement potentials in the motor cortex and in the posterior bank of inferior limb of the arcuate sulcus (homolog of Broca's area) in the left hemisphere. After the operation, the tone came to have less components of different frequencies, and its duration varied much more than before. These facts suggest that the neocortical area homologous to the human speech area takes part in the generation and control of monkey vocalization together with the cerebellum possibly through cerebro-cerebellar interactions. This is against ideas so far proposed on nonhuman primate vocalization, i.e., it is generally considered that animal vocalization differs fundamentally from human speech.