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Hakone, Japan

Sato S.,Kitasato University | Takiyama Y.,Kitasato University | Ogino Y.,National Hakone Hospital | Watanabe K.,Tokyo Metropolitan Matsuzawa Hospital | And 7 more authors.
Clinical Neurology | Year: 2013

It is controversial whether the STN or the GPi, the main targets of DBS therapy in patients with Parkinson's disease, is the appropriate target. We select GPi-DBS in patients judged by our cognitive function test battery to be at high-risk for cognitive decline after STN-DBS. While DBS surgery is usually performed under local anesthesia for the precise placement of DBS electrodes, general anesthesia might be useful in patients intolerant of long-lasting surgical stress. Our monkey experiments revealed that the most medial part of the STN receives direct input from the limbic cortex, suggesting that the spread of stimulation to these limbic territories: may elicit adverse emotional effects. Other monkey experiments on the physiological mechanism of DBS suggest that high-frequency GPi stimulation disrupts information flow through the GPi. Source

Yagi T.,Keio University | Ito D.,Keio University | Nihei Y.,Keio University | Ishihara T.,National Hakone Hospital | Suzuki N.,Keio University
Human Molecular Genetics | Year: 2011

Heterozygosity for mutations (N88S and P90L) in the N-glycosylation site of seipin/BSCL2 is associated with the autosomal dominant motor neuron diseases, spastic paraplegia 17 and distal hereditary motor neuropathy type V, referred to as 'seipinopathies'. Previous in vitro studies have shown that seipinopathy-linked mutations result in accumulation of unfolded proteins in the endoplasmic reticulum (ER), leading to the unfolded protein response and cell death, suggesting that seipinopathies is closely associated with ER stress. To further understand the molecular pathogenesis of seipinopathies, we generated a transgenic (tg) mouse line expressing the human N88S seipin mutant with the murine Thy-1 promoter to permit analyses of in vivo phenotypic changes. The N88S seipin tg mice develop a progressive spastic motor deficit, reactive gliosis in the spinal cord and neurogenic muscular atrophy, recapitulating the symptomatic and pathological phenotype in patients of seipinopathy. We also found that expression of mutant seipin in mice upregulated the ER stress marker, immunoglobulin-heavy-chain-binding protein, protein disulfide isomerase and X-box binding protein 1, but was not linked to significant neuronal loss in affected tissue, thereby indicating that ER stress is sufficient, while neuronal death is not necessary, for the development of motor phenotypes of seipinopathies. Our findings in the mutant seipin tg mouse provide clues to understand the relationship with ER stress and neurodegeneration, and the seipin tg mouse is a valid tool for the development of novel therapeutic strategies against ER stress-related diseases. © The Author 2011. Published by Oxford University Press. All rights reserved. Source

Isose S.,Chiba University | Misawa S.,Chiba University | Sonoo M.,Teikyo University | Shimuzu T.,Tokyo Metropolitan Neurological Hospital | And 12 more authors.
Journal of Clinical Neurophysiology | Year: 2014

Purpose: In current electrodiagnostic criteria for chronic inflammatory demyelinating polyneuropathy, the cutoff values of distal compound muscle action potential (DCMAP) duration are defined using electromyogram low-cut filter setting of 20 Hz. We aimed to assess effects of low-cut filter on DCMAP duration (10 vs. 20 Hz). Methods: We prospectively measured DCMAP duration in 130 normal controls and 42 patients, fulfilling diagnostic criteria for typical chronic inflammatory demyelinating polyneuropathy by European Federation of Neurological Societies/Peripheral Nerve Society. Results: Distal compound muscle action potential duration was significantly shortened with 20-Hz than 10-Hz filtering. When the cutoff values were defined as the upper limit of normal (ULN, mean 1 2.5SD), the sensitivity/specificity was 67%/95% in 10-Hz recordings, and 69%/95% in 20-Hz recordings. This diagnostic accuracy was similar to that defined by receiver operating characteristic analyses. Conclusions: Distal compound muscle action potential duration significantly affected by the low-cut electromyogram filter setting, but with at least 10 and 20 Hz, the diagnostic accuracy is similar. Copyright © 2014 by the American Clinical Neurophysiology Society. Source

Komori T.,National Hakone Hospital
Clinical Neurology | Year: 2011

Life quality is various among patients. In this article, it is shown that several progresses of both physical and psychological care for those patients during resent 15 years which we have had Japanese National Project for the quality of life in intractable diseases. Of course total cure of the disease is the best for the patients, but the second best is continuous better patient care. Total respiratory care of amyotrophic lateral sclerosis including non-invasive positive pressure ventilation and systematic approach of respiratory rehabilitation are good examples of better physical care. The approach with SEIQoL-DW is another good trial to evaluate and maintain their psychological condition. These should be also useful to another intractable diseases such as multi-system atrophy, spinocerebellar atrophy or Parkinson disease. Long term contribution of multi-disciplinary care team of individual patient has to be needed to make the better effort for the patient support now. We should have more options of treatment, rehabilitation or social life support those with which the patients could decide to have their favorable life with intractable disease. Source

Mitsuma S.,Chiba University | Van den Bergh P.,Catholic University of Leuven | Rajabally Y.A.,University of Leicester | Van Parijs V.,Catholic University of Leuven | And 22 more authors.
Clinical Neurophysiology | Year: 2015

Objective: The duration of the distal compound muscle action potential (DCMAP) is a useful index to detect demyelination in the distal nerve segments. However in published electrodiagnostic criteria for chronic inflammatory demyelinating polyneuropathy (CIDP), the cut-off values of DCMAP duration are defined using an EMG low frequency filter of only 20. Hz. We aimed to provide widely-available reference data using several low cut filters. Methods: In 13 Japanese and European tertiary centers, DCMAP duration data using 2, 5, 10, and 20. Hz low frequency filters were prospectively collected from 147 normal controls, 59 patients with typical CIDP, and 100 with diabetic polyneuropathy. Optimal cut-off values were calculated with receiver-operating characteristic curves, offering 100% specificity versus normal controls. Results: The higher low frequency filter was associated with significantly shorter DCMAP duration in all groups. For CIDP diagnosis, the calculated cut-off values had a sensitivity ranging from 51% to 66%, and a specificity versus diabetic neuropathy from 96% to 98%. Conclusions: Our results show that DCMAP duration is largely dependent on low frequency filter settings, but is a useful index for CIDP diagnosis when the cut-off values are properly determined at each filter setting. Significance: Our data provide the systematic reference values of DCMAP duration for CIDP diagnosis available for most EMG laboratories. © 2014 International Federation of Clinical Neurophysiology. Source

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