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A research committee supported by the Japanese government conducted a nationwide survey on the epidemiology of acute encephalopathy in Japan using a questionnaire. A total of 983 cases reportedly had acute encephalopathy during the past 3 years, 2007-2010. Among the pathogens of the preceding infection, influenza virus was the most common, followed by human(More)
We elucidated efferent projections of second-order relay neurons (P-cells) activated by afferents originating from slowly adapting pulmonary receptors (SARs) to determine the central pathway of the SAR-evoked reflexes. Special attention was paid to visualizing the P-cell projections within the nucleus tractus solitarii (NTS), which may correspond to the(More)
Niemann-Pick disease type C (NPC) is a neurovisceral lipid storage disorder characterized by progressive and widespread neurodegeneration. Although some characteristic symptoms of NPC result from brainstem dysfunction, little information is available about which brainstem structures are affected. In this study, the brainstems of mutant BALB/c NPC1-/- mice(More)
We report a 1-year 6-month-old girl with ganglioglioma in the right medial temporal lobe who showed epileptic spasms in clusters. Spasms occasionally followed a dazed and fearful gaze. Interictal electroencephalography (EEG) showed diffuse bursts of slightly irregular high-voltage spikes and slow waves without hypsarrhythmia. The findings on ictal EEG,(More)
To delineate the evolution of non-epileptic and epileptic paroxysmal events in alternating hemiplegia of childhood (AHC), we reviewed clinical information of nine patients (4-40 years) with AHC. Paroxysmal abnormal ocular movements, head turning, and tonic, clonic, or myoclonic limb movements were the initial symptoms (birth-8m) in each patient. Ictal(More)
Alternating hemiplegia of childhood (AHC) is a rare disorder characterized by repeated plegic attacks, movement disorders, autonomic phenomena, and developmental delay. To obtain insights into the pathophysiology of AHC, we determined the concentrations of matrix metalloproteinase-9 (MMP-9), tissue inhibitor of MMP-1 (TIMP-1), calcitonin gene-related(More)
There are two types of expiratory neurons with augmenting firing patterns (E-AUG neurons), those in the Bötzinger complex (BOT) and those in the caudal ventral respiratory group (cVRG). We studied their axonal projections morphologically using intracellular labeling of single E-AUG neurons with Neurobiotin, in anesthetized, paralyzed and(More)
The epileptogenesis of the striatum is unknown. We describe the case of a 12-year-old girl with intractable epilepsy who was treated by surgical interventions. Magnetic resonance imaging (MRI) showed ambiguous corticomedullary boundary in the left frontal lobe, and magnetoencephalography (MEG) revealed spike dipoles in the vicinity of the left ventral(More)
No association between mitochondrial disease and pancreatitis has yet been established, although diabetes mellitus and diseases caused by exocrine insufficiency, such as Pearson syndrome, are the commonest pancreatic complications of mitochondrial diseases. Here, we report 2 cases of mitochondrial disease complicated by pancreatitis as an unusual pancreatic(More)
We report on a 14-year-old male suffering from acute encephalitis, whose clinical course met the criteria for acute encephalopathy with refractory, repetitive partial seizures (AERRPS). He presented with extremely refractory partial and secondary generalized seizures, and required high-dose barbiturate infusion therapy for 57 days under mechanical(More)