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It is difficult to distinguish manganism from idiopathic parkinsonism by clinical signs only. Case history and examination: A 48-year-old welder for over 10 years complained of masked face, right side (arm and leg) resting tremor, and bradykinesia for over one year. Magnetic resonance imaging (MRI) findings showed symmetrical high signal intensities in the(More)
RATIONALE AND OBJECTIVES High signal intensity in the basal ganglia on T1-weighted MR imaging has been reported in chronic manganese (Mn) poisoning. However, the exact meaning of the high signal intensity remains unclear: does it result from Mn itself, secondary pathologic changes of the brain tissue, or both? The goal of this study was to evaluate the(More)
BACKGROUND Autosomal dominant optic atrophy (ADOA) is the commonest form of inherited optic neuropathy. Mutations in the OPA1 gene encoding a dynamin-related mitochondrial protein underlie ADOA and may perturb the biogenesis and maintenance of mitochondria. OBJECTIVE To investigate the mutation spectrum of the OPA1 gene and assess alterations in(More)
We developed a method for the efficient generation of functional dopaminergic (DA) neurons from human embryonic stem cells (hESCs) on a large scale. The most unique feature of this method is the generation of homogeneous spherical neural masses (SNMs) from the hESC-derived neural precursors. These SNMs provide several advantages: (i) they can be passaged(More)
BACKGROUND In human and experimental models, diffusion-weighted magnetic resonance imaging (DWI) findings in status epilepticus (SE) have been reported to show that apparent diffusion coefficients are reduced during the initial phase and normalized or increased in the later phase of prolonged SE. This effect is caused by cytotoxic edema induced by(More)
Cocaine administration upregulates the levels of extracellular glutamate and dopamine in the striatum. Activation of the receptors alters calcium homeostasis in striatal neurons leading to the expression of the endoplasmic reticulum (ER) stress proteins. It was therefore hypothesized that cocaine upregulates the expression of the ER stress proteins,(More)
OBJECTIVE alpha-Synuclein gene (SNCA) multiplication was found in familial Parkinson disease (PD). We examined SNCA multiplication in patients with familial and sporadic PD and multiple system atrophy (MSA). METHODS We screened 1,106 patients with parkinsonism (PD = 906, MSA = 200) for SNCA multiplication by multiplex PCR. Fluorescent in situ(More)
Interferon (IFN) consensus sequence-binding protein/IFN regulatory factor 8 (IRF8) is a transcription factor that regulates the differentiation and function of macrophages, granulocytes, and dendritic cells through activation or repression of target genes. Although IRF8 is also expressed in lymphocytes, its roles in B cell and T cell maturation or function(More)
The K2K experiment observes indications of neutrino oscillation: a reduction of nu(mu) flux together with a distortion of the energy spectrum. Fifty-six beam neutrino events are observed in Super-Kamiokande (SK), 250 km from the neutrino production point, with an expectation of 80.1(+6.2)(-5.4). Twenty-nine one ring mu-like events are used to reconstruct(More)
OBJECTIVE To investigate the role of spinocerebellar ataxia type 17 (SCA17) in the development of parkinsonism. METHOD We screened 1,155 parkinsonian patients (931 with Parkinson disease and 224 with multiple system atrophy) and 400 normal subjects for SCA17. 99mTc-TRODAT-1 SPECT was used to evaluate the striatal dopamine transporter (DAT) status. (More)