Publications by authors named "Yuki Hatanaka"

78 Publications

Myasthenia gravis with inflammatory myopathy without elevation of creatine kinase.

Neuromuscul Disord 2021 Apr 1. Epub 2021 Apr 1.

Department of Neurology, Teikyo University School of Medicine, Kaga 2-11-1, Itabashi-ku 1738605, Tokyo, Japan. Electronic address:

Cases of myasthenia gravis with inflammatory myopathy usually show elevated creatine kinase (CK) levels. There are few case reports of myasthenia gravis with inflammatory myopathy without elevated CK levels, and clinical features and useful diagnostic methods for these patients are little known. We describe the case of a 79-year-old man with myasthenia gravis that was complicated with inflammatory myopathy without elevated CK levels and successfully treated with immunological treatment. Initially, he was diagnosed with ocular myasthenia gravis and treated with pyridostigmine, but dysphagia and weakness in the neck and bilateral upper limb without fatigability gradually developed. Needle electromyography revealed myopathic changes, and the results of muscle biopsy were consistent with inflammatory myopathy. Blood tests showed normal CK levels throughout the clinical course and elevated myoglobin levels alone. The possibility of developing inflammatory myopathy in patients with myasthenia gravis cannot be excluded, even if CK levels are normal.
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http://dx.doi.org/10.1016/j.nmd.2021.03.010DOI Listing
April 2021

Symmetrically dimethylated histone H3R2 promotes global transcription during minor zygotic genome activation in mouse pronuclei.

Sci Rep 2021 May 12;11(1):10146. Epub 2021 May 12.

Laboratory of Molecular Developmental Biology, Graduate School of Biology-Oriented Science and Technology, Kindai University, Wakayama, Japan.

Paternal genome reprogramming, such as protamine-histone exchange and global DNA demethylation, is crucial for the development of fertilised embryos. Previously, our study showed that one of histone arginine methylation, asymmetrically dimethylated histone H3R17 (H3R17me2a), is necessary for epigenetic reprogramming in the mouse paternal genome. However, roles of histone arginine methylation in reprogramming after fertilisation are still poorly understood. Here, we report that H3R2me2s promotes global transcription at the 1-cell stage, referred to as minor zygotic genome activation (ZGA). The inhibition of H3R2me2s by expressing a histone H3.3 mutant H3.3R2A prevented embryonic development from the 2-cell to 4-cell stages and significantly reduced global RNA synthesis and RNA polymerase II (Pol II) activity. Consistent with this result, the expression levels of MuERV-L as minor ZGA transcripts were decreased by forced expression of H3.3R2A. Furthermore, treatment with an inhibitor and co-injection of siRNA to PRMT5 and PRMT7 also resulted in the attenuation of transcriptional activities with reduction of H3R2me2s in the pronuclei of zygotes. Interestingly, impairment of H3K4 methylation by expression of H3.3K4M resulted in a decrease of H3R2me2s in male pronuclei. Our findings suggest that H3R2me2s together with H3K4 methylation is involved in global transcription during minor ZGA in mice.
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http://dx.doi.org/10.1038/s41598-021-89334-wDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8115239PMC
May 2021

Determining C5, C6 and C7 myotomes through comparative analyses of clinical, MRI and EMG findings in cervical radiculopathy.

Clin Neurophysiol Pract 2021 18;6:88-92. Epub 2021 Feb 18.

Department of Neurology, Teikyo University School of Medicine, Kaga 2-11-1, Itabashi-ku, Tokyo, Japan.

Objective: There are many myotome charts in the literature, but few studies have presented actual data to support their identification. We aimed to determine C5/C6/C7 myotomes based on clinical and EMG data of patients with cervical spondylotic radiculopathy (CSR) having a single-root lesion confirmed by MRI.

Methods: Medical Research Council (MRC) scores and EMG findings were retrospectively reviewed for patients enrolled from our EMG database.

Results: Enrolled were 25 patients (10 C5, 6 C6, and 9 C7 CSR). In C5 CSR, weakness or denervation potentials in EMG, or both, were observed in the deltoid (Del) and infraspinatus (Isp) muscles for all patients, and in the biceps brachii (BB) and brachioradialis (BR) muscles for 9/10 and 8/9 patients, respectively. In C6 CSR, weakness of the wrist extensor and/or denervation of the extensor carpi radialis longus (ECRL)/extensor carpi radialis brevis (ECRB), and those of the pronator teres (PT) were observed for all patients. Weakness was not observed for any other muscle in C6 CSR. Denervation potentials of ECRL were found in 5/8 and 3/5 patients with C5 and C6 CSR, respectively, whereas those of ECRB were found in 1/5, 6/6, and 2/5 patients with C5, C6 and C7 CSR, respectively. In C7 CSR, weakness/denervation of the triceps brachii (TB) and denervation potentials of the flexor carpi radialis (FCR) were observed for all patients. Denervation potentials in PT and weakness/denervation of the extensor digitorum (ED) were observed in 2/9 and 4/9 patients, respectively.

Conclusion: Suggested dominant myotomes are: C5 for the Del, Isp, BB, and BR, C5/6 for the ECRL, C6 > C7 for the ECRB and PT, and C7 for the TB and FCR.

Significance: The current study identified dominant myotomes that differ from the existing literature.
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http://dx.doi.org/10.1016/j.cnp.2021.02.002DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7966958PMC
February 2021

A Case Report of Nasogastric Tube Syndrome: The Size and Type of the Nasogastric Tube May Contribute to the Development of Nasogastric Tube Syndrome.

Intern Med 2021 Feb 1. Epub 2021 Feb 1.

Department of Neurology, Teikyo University School of Medicine, Japan.

Nasogastric tube syndrome (NGTS) is a rare but life-threatening complication associated with nasogastric tube (NGT) placement. The effect of the NGT size and type on the development of NGTS has not yet been fully elucidated. We herein report the case of a 77-year-old man with cerebral infarction who was complicated with NGTS. The immediate removal of the NGT improved the symptoms of NGTS. Although the NGT was passed through the same route during reinsertion, the use of a softer and smaller-sized NGT did not cause any NGTS recurrence. To prevent the development of NGTS, using a NGT that is appropriate for the patient's condition is important.
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http://dx.doi.org/10.2169/internalmedicine.6258-20DOI Listing
February 2021

Aquaporin-4-antibody-positive Neuromyelitis Optica Spectrum Disorder in a Patient with Charcot-Marie-Tooth Disease Type 1A.

Intern Med 2021 May 22;60(10):1611-1614. Epub 2020 Dec 22.

Department of Neurology, Teikyo University School of Medicine, Japan.

Charcot-Marie-Tooth disease type 1A (CMT1A) is a hereditary peripheral neuropathy, and its involvement in the central nervous system (CNS) is very rare. We herein report a 51-year-old woman with CMT1A who suffered from recurrent optic neuritis and myelopathy. Under the diagnosis of anti-aquaporin-4 (anti-AQP4) antibody positive neuromyelitis optica spectrum disorder (NMOSD), we treated her successfully with corticosteroids. This is the first report of CMT1A complicated with anti-AQP4-positive NMOSD. Although the coexistence of the two disorders may simply be a coincidence, we speculated that immune cross-reaction between overexpressed peripheral myelin protein 22 and CNS myelin may have caused concomitant CMT1A and NMOSD.
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http://dx.doi.org/10.2169/internalmedicine.6153-20DOI Listing
May 2021

[Hemiplegia cruciata and severe facial pain due to infarction of the cervicomedullary junction: a case report].

Rinsho Shinkeigaku 2020 Oct 5;60(10):693-698. Epub 2020 Sep 5.

Department of Neurology, Teikyo University School of Medicine.

We report the case of a 66-year-old female with hemiplegia cruciata and severe facial pain due to infarction of the cervicomedullary junction. She presented to the hospital with complaints of acute-onset left facial pain and gait disturbance. Neurological examination revealed narrow left palpebral fissure, severe left facial pain and hypothermoesthesia, weakness predominantly in the left upper and right lower extremities, decreased pain and temperature sensation in the right lower extremity, decreased vibration sensation in the left lower extremity, hyperreflexia in the left upper extremity, and mild ataxia in the left upper and lower extremities. Brain MRI revealed a high-intensity lesion in the left cervicomedullary junction on diffusion-weighted and fluid-attenuated inversion recovery images. Hemiplegia cruciata due to the pyramidal tract injury at the cervicomedullary junction is an uncommon clinical manifestation. However, in patients with hemiplegia cruciata, identifying the lesion location may be difficult. Clinicians should consider the possibility of pyramidal decussation lesions. Anatomical differences, in the course of pyramidal tract fibers between the upper and lower limbs have been considered in the pyramidal decussation. Hemiplegia cruciata in this case was primarily caused by the impairment of the left upper limb pyramidal fibers after the pyramidal decussation and the right lower limb pyramidal fibers before the pyramidal decussation.
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http://dx.doi.org/10.5692/clinicalneurol.cn-001457DOI Listing
October 2020

[Severe sensory-motor axonal neuropathy following diabetic ketoacidosis].

Rinsho Shinkeigaku 2020 Sep 8;60(9):614-619. Epub 2020 Aug 8.

Department of Neurology, Teikyo University School of Medicine.

We report a case of severe sensory-motor axonal neuropathy on the lower extremities associated with diabetic ketoacidosis (DKA). A sixteen-year-old boy developed coma and admitted to our hospital. We diagnosed him with DKA based on remarkable hyperglycemia, severe acidosis with hyperketonemia. Intensive glycemic control with insulin was immediately started. He had complications of heart failure, rhabdomyolysis, and renal failure, which required intensive care including mechanical ventilation and hemodialysis. When recovered from the critical condition, he noticed severe weakness, numbness, and pain on the lower limbs, and urinary retention. On nerve conduction studies, both motor and sensory action potentials were absent. Serum anti-ganglioside antibodies were negative. Albuminocytologic dissociation was evident in the cerebrospinal fluid. MRI study revealed marked gadolinium enhancement of the cauda equina. After high-dose intravenous immunoglobulin treatment, he was relieved from leg pain, but the leg weakness and bladder bowel dysfunction did not show immediate improvement. It took approximately six months until he became able to stand and walk using ankle orthosis. Acute neuropathy is a rare complication of diabetes mellitus. Painful neuropathy is known to emerge in association with diabetic treatment, but it seldom causes severe motor disturbance. On the other hand, motor-dominant polyneuropathy has been reported to occur acutely along the treatment of DKA and hyperosmolar hyperglycemia syndrome (HHS). Present case and previous cases with DKA and HHS suggest that rapid correction of glucose level is one of the underlying factors of acute neuropathy related with diabetic treatment.
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http://dx.doi.org/10.5692/clinicalneurol.cn-001433DOI Listing
September 2020

Developmental competence of interspecies cloned embryos produced using cells from large Japanese field mice (Apodemus speciosus) and oocytes from laboratory mice (Mus musculus domesticus).

J Reprod Dev 2020 Jun 26;66(3):255-263. Epub 2020 Mar 26.

Graduate School of Biology-Oriented Science and Technology, Kindai University, Wakayama 649-6493, Japan.

The large Japanese field mouse (Apodemus speciosus) is endemic to Japan and may be used as an animal model for studies related to environmental pollution, medical science, and basic biology. However, the large Japanese field mouse has low reproductive ability due to the small number of oocytes ovulated per female. To produce experimental models, we investigated the in vitro developmental potential of interspecies somatic cell nuclear transfer (iSCNT) embryos produced by fusing tail tip cells from the large Japanese field mouse with enucleated oocytes from laboratory mice (Mus musculus domesticus). Only a small number of iSCNT embryos developed to the 4-cell (0-4%) and blastocysts (0-1%) stages under sequential treatment using trichostatin A (TSA) and vitamin C (VC) supplemented with deionized bovine serum albumin (d-BSA). This sequential treatment led to the reduction in H3K9 trimethylation and did not affect H3K4 trimethylation in at least the 2-cell stage of the iSCNT embryos. Moreover, iSCNT embryos that received tail tip cells with exposure treatment to ooplasm from cell fusion to oocyte activation or VC treatment prior to cell fusion did not exhibit significant in vitro development improvement compared to that of each control group. This suggests that large Japanese field mice/laboratory mice iSCNT embryos that received sequential treatment using TSA and VC with d-BSA may have slightly better developmental potential beyond the 4-cell stage. Our results provide insights into the reprogramming barriers impeding the wider implementation of iSCNT technology.
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http://dx.doi.org/10.1262/jrd.2019-167DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7297636PMC
June 2020

Transient epileptic amnesia with amygdala enlargement.

Neurol Sci 2020 Jun 19;41(6):1591-1593. Epub 2019 Dec 19.

Department of Neurology, Teikyo University School of Medicine, Kaga 2-11-1, Itabashi-ku, Tokyo, 1738605, Japan.

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http://dx.doi.org/10.1007/s10072-019-04193-8DOI Listing
June 2020

Inflammatory myopathy with myasthenia gravis: Thymoma association and polymyositis pathology.

Neurol Neuroimmunol Neuroinflamm 2019 03 24;6(2):e535. Epub 2018 Dec 24.

Department of Neurology (N.U., K.T., C.I., A.U., A.K., T.T., J.S.), Graduate School of Medicine, University of Tokyo; Division of Neurology (M.K., K.K.), Department of Internal Medicine, National Defense Medical College, Saitama; Division of Neurology (Y. Shiio), Tokyo Teishin Hospital; Department of Neurology (Y. Sakurai), Mitsui Memorial Hospital; Department of Neurology (Y.H.), Teikyo University School of Medicine; and Department of Neurology (K.Y., S.N.-E.), Fukushima Medical University, Japan.

Objective: To provide evidence that idiopathic inflammatory myopathy (IM) with myasthenia gravis (MG) frequently shows thymoma association and polymyositis (PM) pathology and shares clinicopathologic characteristics with IM induced by immune checkpoint inhibitors (ICIs).

Methods: We analyzed the clinicopathologic features of 10 patients with idiopathic IM and MG identified in 970 consecutive patients with biopsy-proven IM.

Results: Seven patients (70%) had thymoma. IM and MG were diagnosed with more than 5-year time difference in 6 thymomatous patients and within 1 year in 1 thymomatous and 3 nonthymomatous patients. Seven thymomatous patients showed rhabdomyolysis-like features with respiratory failure (4/7), dropped head (3/7), cardiac involvement (2/7), and positive anti-acetylcholine receptor (anti-AChR) and anti-titin antibodies (7/7 and 4/6, respectively) but rarely showed ocular symptoms (2/7) or decremental repetitive nerve stimulation (RNS) responses (1/7) at IM diagnosis. Three nonthymomatous patients showed acute cardiorespiratory failure with rhabdomyolysis-like features (1/3), positive anti-AChR and anti-titin antibodies (3/2 and 2/2, respectively), and fluctuating weakness of the skeletal muscle without ocular symptoms (3/3). Muscle pathology showed a PM pathology with infiltration of CD8-positive CD45RA-negative T-lymphocytes (9/9), scattered endomysial programmed cell death 1 (PD-1)-positive cells (9/9), and overexpression of programmed cell death ligand 1 (PD-L1) on the sarcolemma of muscle fibers around the infiltrating PD-1-positive cells (7/9).

Conclusion: Rhabdomyolysis-like features, positive anti-AChR antibody without decremental RNS responses, and PD-L1 overexpression are possible characteristics shared by ICI-induced IM. Frequent thymoma association in patients with idiopathic IM and MG may suggest thymoma-related immunopathogenic mechanisms, including dysregulation of the immune checkpoint pathway.
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http://dx.doi.org/10.1212/NXI.0000000000000535DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6340335PMC
March 2019

Bosniak Category III Renal Cysts Caused by Crizotinib in an Anaplastic Lymphoma Kinase Gene-Rearranged Non-Small Cell Lung Cancer Patient.

Urology 2018 Nov 21;121:e3-e4. Epub 2018 Aug 21.

Department of Urology, National Defense Medical College, Tokorozawa, Saitama 359-8513, Japan.

Bosniak category III renal cystic masses are often treated with surgical resection because of high risk of malignancy. Crizotinib is an anaplastic lymphoma kinase (ALK) inhibitor used to treat ALK gene-rearranged non-small cell lung cancer and reported to be associated with complex renal cyst formation. We herein report a case of Bosniak category III renal cysts occurred in a crizotinib-treated ALK gene-rearranged non-small cell lung cancer patients. The cysts regressed spontaneously after cessation of crizotinib and we could thus avoid unnecessary surgical resection.
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http://dx.doi.org/10.1016/j.urology.2018.08.008DOI Listing
November 2018

[Primary central nervous system methotrexate associated lymphoproliferative disorders in a patient with rheumatoid arthritis].

Rinsho Shinkeigaku 2018 Aug 31;58(8):485-491. Epub 2018 Jul 31.

Department of Neurology, Teikyo University School of Medicine.

We report on a 52-year-old woman with rheumatoid arthritis (RA) who developed methotrexate associated lymphoproliferative disorders (MTX-LPD) in the central nervous system (CNS) in the course of immunosuppressive therapy for RA. The patient was admitted because of monoplegia in her left hand. She had been receiving methotrexate (MTX) for her RA for several years and etanercept had also been introduced because of a worsening of the arthritis six months before admission. Brain MRI revealed multiple lesions with enhancement scattered throughout both hemispheres. F-fluorodeoxyglucose-positron emission tomography/computed tomography showed abnormal accumulation suggesting malignancy in the right frontal lobe where abnormal enhancement was observed on the MRI. A brain biopsy was performed at the identified site and it confirmed diffuse large B-cell lymphoma (DLBCL). We therefore diagnosed her as MTX-LPD. According to previous reports, most MTX-LPD cases tend to show regression after the cessation of MTX. However, our case showed no regression and even needed chemotherapy. The patient had a poorer prognosis than previous cases and died 17 months after the onset. Although it is an uncommon complication, particularly in the CNS, MTX-LPD should be considered as a critical differential diagnosis if a patient receiving MTX develops central nervous system lesions. Immediate medical intervention including brain biopsy is required.
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http://dx.doi.org/10.5692/clinicalneurol.cn-001157DOI Listing
August 2018

Anti-ganglioside complex antibody profiles in a recurrent complicated case of GQ1b-seronegative miller fisher syndrome and Bickerstaff brainstem encephalitis: a case report.

BMC Neurol 2018 May 23;18(1):72. Epub 2018 May 23.

Department of Neurology, National Hospital Organization Nagoya Medical Center, 4-1-1 Sannomaru, Naka-ku, Nagoya, Japan.

Background: Guillain-Barré syndrome (GBS), Miller Fisher syndrome (MFS) and Bickerstaff brainstem encephalitis (BBE) are a group of autoimmune neurological disorders (GBS spectrum disorder) that rarely recur. Recently, anti-ganglioside complex antibodies (GSC-Abs) were identified in patients with GBS spectrum disorder. However, there has been no case report describing GSC-Abs profiles in a recurrent case showing different phenotypes.

Case Presentation: We report the case of a 33-year-old male patient with GQ1b-seronegative BBE-GBS after two prior episodes of MFS-GBS. Our patient showed ophthalmoplegia, ataxia, areflexia and a weakness of the extremities (MFS and GBS symptoms) in all episodes. In the episode reported here, our patient showed disturbed consciousness and an extensor response to cutaneous plantar stimulation was observed (BBE symptoms), with severe disability and requirement for artificial respiration management. GSC-Abs detected in previous episodes were also detected in the subsequent episodes, while new GSC-Abs emerged in each episode. Interestingly, whereas antibodies to GA1/GQ1b and GA1/GT1a, which are commonly identified in patients with GBS, MFS or BBE, appeared in all episodes, antibodies to GD1a/GD1b and GD1b/GT1b, which are predominantly associated with severe disability and the requirement for artificial respiration management in GBS, emerged for the first time in this episode.

Conclusion: This study reports novel phenomena about the GSC-Abs profiles and its relationship with clinical features in a case with recurrent GBS spectrum disorder, showing different phenotypes in different episodes. Further studies are required to reveal the significance of the GSC-Abs profiles in recurrent GBS spectrum disorder.
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http://dx.doi.org/10.1186/s12883-018-1077-5DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5966867PMC
May 2018

Repetitive Discharge in a Case of Isaacs Syndrome with Burning Sensation.

Intern Med 2018 09 27;57(17):2597. Epub 2018 Apr 27.

Department of Neurology, Mitsui Memorial Hospital, Japan.

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http://dx.doi.org/10.2169/internalmedicine.0392-17DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6172557PMC
September 2018

Peroxiredoxin as a functional endogenous antioxidant enzyme in pronuclei of mouse zygotes.

J Reprod Dev 2018 Apr 2;64(2):161-171. Epub 2018 Mar 2.

Laboratory of Molecular Developmental Biology, Graduate School of Biology-Oriented Science and Technology, Kindai University, Wakayama 649-6493, Japan.

Antioxidant mechanisms to adequately moderate levels of endogenous reactive oxygen species (ROS) are important for oocytes and embryos to obtain and maintain developmental competence, respectively. Immediately after fertilization, ROS levels in zygotes are elevated but the antioxidant mechanisms during the maternal-to-zygotic transition (MZT) are not well understood. First, we identified peroxiredoxin 1 (PRDX1) and PRDX2 by proteomics analysis as two of the most abundant endogenous antioxidant enzymes eliminating hydrogen peroxide (HO). We here report the cellular localization of hyperoxidized PRDX and its involvement in the antioxidant mechanisms of freshly fertilized oocytes. Treatment of zygotes at the pronuclear stage with HO enhanced pronuclear localization of hyperoxidized PRDX in zygotes and concurrently impaired the generation of 5-hydroxymethylcytosine (5hmC) on the male genome, which is an epigenetic reprogramming event that occurs at the pronuclear stage. Thus, our results suggest that endogenous PRDX is involved in antioxidant mechanisms and epigenetic reprogramming during MZT.
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http://dx.doi.org/10.1262/jrd.2018-005DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5902904PMC
April 2018

Fasciculation potentials and decremental responses in amyotrophic lateral sclerosis.

Clin Neurophysiol 2018 02 21;129(2):345-348. Epub 2017 Nov 21.

Department of Neurology, Teikyo University School of Medicine, 2-11-1 Kaga, Itabashi-ku, Tokyo 173-8605, Japan. Electronic address:

Objective: The positive correlation between fasciculation potentials (FPs) and decremental responses in repetitive nerve stimulation test (RNS) in amyotrophic lateral sclerosis (ALS) patients has been described based on only one past study. We revisited this issue.

Methods: Subjects consisted of 30 prospectively-enrolled ALS patients on whom both needle EMG and RNS were conducted in the same trapezius muscle. Fasciculation potentials (FPs) were identified off-line from the restored 3-min signal. Firing rate of FPs (FR-FP) per minute was calculated from the total count of FPs of different origins. Correlations between FR-FP, decremental percentage (Decr%) and the amplitude of the initial compound muscle action potential (CMAPamp) in RNS were investigated.

Results: There was no correlation between FR-FP and Decr% (r = 0.03) or between FR-FP and CMAPamp (r = 0.04). A significant negative correlation was observed between CMAPamp and Decr% (r = -0.56, P < .005).

Conclusion: FPs are not correlated with the decremental response in RNS.

Significance: The underlying mechanism for FPs and decremental responses in ALS must be different and unrelated to each other.
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http://dx.doi.org/10.1016/j.clinph.2017.11.007DOI Listing
February 2018

Quantitative muscle ultrasound is useful for evaluating secondary axonal degeneration in chronic inflammatory demyelinating polyneuropathy.

Brain Behav 2017 10 15;7(10):e00812. Epub 2017 Sep 15.

Department of Neurology Teikyo University School of Medicine Itabashi-ku Tokyo Japan.

Introduction: In chronic inflammatory demyelinating polyneuropathy (CIDP), exclusion of secondary axonal degeneration is challenging with conventional methods such as nerve conduction study (NCS), needle electromyography, and nerve biopsy. Increased echo intensity (EI) and decreased muscle thickness (MT) identified on muscle ultrasound (MUS) examination represent muscle denervation due to axonal degeneration in neurogenic disorders, suggesting MUS as a new tool to detect secondary axonal degeneration in patients with CIDP.

Methods: EI and MT of abductor pollicis brevis, abductor digiti minimi, and first dorsal interosseous muscles were measured in 16 CIDP patients. Raw values were converted into -scores using data from 60 normal controls (NCs).

Results: Six of 45 muscles showed abnormally high EI and low MT, suggesting denervation following secondary axonal degeneration. These six muscles belonged to two patients with long disease history, unresponsiveness to treatment, and long interval from onset to initial therapy. There were no significant differences in EI and MT ( = .23 and .67, respectively) between the CIDP and NC groups, although NCS results revealed obvious demyelinating abnormalities in all CIDP patients, suggesting the fact that muscle structures will be preserved, and EI and MT will not change unless secondary axonal degeneration occurs in CIDP.

Conclusion: MUS is a promising tool for evaluating secondary axonal degeneration in patients with CIDP.
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http://dx.doi.org/10.1002/brb3.812DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5651395PMC
October 2017

Histone H3 Methylated at Arginine 17 Is Essential for Reprogramming the Paternal Genome in Zygotes.

Cell Rep 2017 Sep;20(12):2756-2765

RIKEN BioResource Center, Ibaraki 305-0074, Japan; Graduate School of Life and Environmental Sciences, University of Tsukuba, Ibaraki 305-8572, Japan; Center for Disease Biology and Integrative Medicine, Faculty of Medicine, University of Tokyo, Tokyo 113-0033, Japan. Electronic address:

At fertilization, the paternal genome undergoes extensive reprogramming through protamine-histone exchange and active DNA demethylation, but only a few maternal factors have been defined in these processes. We identified maternal Mettl23 as a protein arginine methyltransferase (PRMT), which most likely catalyzes the asymmetric dimethylation of histone H3R17 (H3R17me2a), as indicated by in vitro assays and treatment with TBBD, an H3R17 PRMT inhibitor. Maternal histone H3.3, which is essential for paternal nucleosomal assembly, is unable to be incorporated into the male pronucleus when it lacks R17me2a. Mettl23 interacts with Tet3, a 5mC-oxidizing enzyme responsible for active DNA demethylation, by binding to another maternal factor, GSE (gonad-specific expression). Depletion of Mettl23 from oocytes resulted in impaired accumulation of GSE, Tet3, and 5hmC in the male pronucleus, suggesting that Mettl23 may recruit GSE-Tet3 to chromatin. Our findings establish H3R17me2a and its catalyzing enzyme Mettl23 as key regulators of paternal genome reprogramming.
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http://dx.doi.org/10.1016/j.celrep.2017.08.088DOI Listing
September 2017

An unusual case of sodium channel myotonia with transient weakness upon initiating movements which is characteristic in Becker disease.

Rinsho Shinkeigaku 2017 06 26;57(6):287-292. Epub 2017 May 26.

Department of Neurology, Teikyo University School of Medicine.

We reported a 32-year-old man who was a sporadic case of myotonic syndrome with muscle stiffness or transient weakness of limbs upon initiating movements after rest. On examination, he showed painless myotonia with warm-up phenomenon, Hercules-like hypertrophic musculature and myotonic discharges in EMG. The clinical findings resembled to those of Becker disease rather than Thomsen disease. But electrodiagnosis suggested sodium channel myotonia instead of chloride channelopathy. Genetic testing detected a novel missense mutation (p.V1166A) in the SCN4A gene but not in the CLCN1 gene. Transient weakness upon initiating movements is usually observed in Becker disease but rare in Thomsen disease, which is not reported in sodium channel myotonia so far. He was probably the first case of sodium channel myotonia with transient weakness upon initiating movements, which was confirmed by 10 Hz repetitive nerve stimulation test as depolarization block.
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http://dx.doi.org/10.5692/clinicalneurol.cn-000980DOI Listing
June 2017

Amyotrophic lateral sclerosis with a sudden-onset history.

Clin Neurophysiol Pract 2017 6;2:103-104. Epub 2017 May 6.

Department of Neurology, Teikyo University School of Medicine, Kaga 2-11-1, Itabashi-ku, Tokyo 1738605, Japan.

Objective: We report on two patients with amyotrophic lateral sclerosis (ALS) complaining of sudden-onset difficulty in finger elevation.

Case Report: A 65-year-old man (the first patient) and a 66-year-old man (the second patient) suddenly became aware of difficulty in finger elevation of one hand. They were not aware of any other symptoms prior to the onset. In the first patient, cerebral infarction at the precentral gyrus was initially suspected. In the second patient, cervical spondylosis was initially suspected, and cervical spine surgery was planned. However, needle EMG revealed widespread neurogenic changes and abundant fasciculation potentials for both patients. Widespread weakness emerged in time and relentlessly progressed, and finally the diagnosis of ALS was made. In both cases, notable weakness in the extensor digitorum (ED) muscle with relatively mild weakness in the other muscles in the affected limb was a characteristic finding. Loss of one motor unit in ED that has already enlarged due to reinnervation must have caused sudden awareness of the weakness.

Significance: Clinicians should recognize the presence of ALS patients with a sudden-onset history because the risk of initial misdiagnosis is high for such patients.
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http://dx.doi.org/10.1016/j.cnp.2017.04.003DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6123847PMC
May 2017

The Rodent-Specific MicroRNA Cluster within the Sfmbt2 Gene Is Imprinted and Essential for Placental Development.

Cell Rep 2017 05;19(5):949-956

Bioresource Engineering Division, BioResource Center, RIKEN, Tsukuba, Ibaraki 305-0074, Japan; Graduate School of Life and Environmental Sciences, University of Tsukuba, Tsukuba, Ibaraki 305-8572, Japan. Electronic address:

MicroRNAs (miRNAs) represent small noncoding RNAs that are involved in physiological and developmental processes by posttranscriptionally inhibiting gene expression. One of the largest miRNA clusters in mice is located in intron 10 of the Sfmbt2 gene, containing 72 miRNA precursor sequences. In this study, we generated mice lacking the entire Sfmbt2 miRNA cluster to elucidate its functions during development. The Sfmbt2 miRNAs were expressed predominantly from the paternal allele in the placenta, as is the host Sfmbt2 gene. Loss of the paternal allele resulted in severely impaired development of the placenta, especially the spongiotrophoblast layer, and frequent lethality or defects of fetuses. The predicted target sequences of the miRNAs and gene expression analysis defined at least nine putative target genes, which function as tumor suppressors or apoptosis inducers. Our study has provided experimental evidence for the indispensable roles of placental miRNAs in trophoblast proliferation and thus fetal development.
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http://dx.doi.org/10.1016/j.celrep.2017.04.018DOI Listing
May 2017

Utility of repetitive nerve stimulation test for ALS diagnosis.

Clin Neurophysiol 2017 May 6;128(5):823-829. Epub 2017 Mar 6.

Department of Neurology, Teikyo University School of Medicine, Kaga 2-11-1, Itabashi-ku, Tokyo, Japan. Electronic address:

Objective: Decremental responses in the repetitive nerve stimulation (RNS) test in amyotrophic lateral sclerosis (ALS) patients have been reported, although their possible diagnostic role has received little investigation. We investigated their diagnostic role in differentiation between ALS and cervical spondylotic amyotrophy (CSA), an important ALS mimic especially in Japan.

Methods: Patients were prospectively enrolled and the diagnosis was confirmed by follow-up. RNS was performed on the abductor pollicis brevis (APB), upper trapezius (trapezius) and deltoid muscles.

Results: Enrolled subjects consisted of 53 ALS and 37 CSA patients. Abnormal decremental responses (>5%) were observed in 32%, 51% and 75% of ALS patients and 3%, 0% and 20% of CSA patients for the APB, trapezius and deltoid muscles, respectively. The sensitivity for 23 ALS patients with upper-limb onset was 78% for the trapezius and 100% for the deltoid muscles.

Conclusions: An abnormal decremental response in the trapezius muscle was 100% specific to ALS in comparison with CSA: abnormal decrement in this muscle would strongly suggest ALS. No decrement in the deltoid muscle might exclude ALS in patients having symptoms with upper-limb onset.

Significance: RNS is useful in differentiation between ALS and CSA.
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http://dx.doi.org/10.1016/j.clinph.2017.02.021DOI Listing
May 2017

Repeated 3-minute stair climbing-descending exercise after a meal over 2 weeks increases serum 1,5-anhydroglucitol levels in people with type 2 diabetes.

J Phys Ther Sci 2017 Jan 30;29(1):75-78. Epub 2017 Jan 30.

Laboratory of Sports and Exercise Medicine, Graduate School of Human and Environmental Studies, Kyoto University, Japan.

[Purpose] The purpose of this study was to examine the hypoglycemic effect of a postprandial exercise program using brief stair climbing-descending exercise in people with type 2 diabetes. [Subjects and Methods] Seven males with uncomplicated type 2 diabetes (age 68.0 ± 3.7 years) performed two sets of stair climbing-descending exercise 60 and 120 min after each meal for the first 2 weeks but not for the following 2 weeks. Each set of exercise comprised 3-min of continuous repetition of climbing briskly to the second floor followed by slow waking down to the first floor in their home. A rest period of 1-2 min was allowed between each set. [Results] Serum 1,5-anhydroglucitol level was significantly higher by 11.5% at the end of the 2-week exercise period than at the baseline. By contrast, the 1,5-anhydroglucitol level at the end of the following 2-week period did not differ from the baseline value. Fasting blood glucose level and insulin resistance index at the end of the exercise period did not differ from the baseline value. [Conclusion] Repeated 3-min bouts of stair climbing-descending exercise after a meal may be a promising method for improving postprandial glycemic control in people with type 2 diabetes.
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http://dx.doi.org/10.1589/jpts.29.75DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5300809PMC
January 2017

CRISPR/Cas9-mediated genome editing in wild-derived mice: generation of tamed wild-derived strains by mutation of the a (nonagouti) gene.

Sci Rep 2017 02 14;7:42476. Epub 2017 Feb 14.

RIKEN BioResource Center, Tsukuba, Ibaraki 305-0074, Japan.

Wild-derived mice have contributed to experimental mouse genetics by virtue of their genetic diversity, which may help increase the chance of identifying novel modifier genes responsible for specific phenotypes and diseases. However, gene targeting using wild-derived mice has been unsuccessful because of the unavailability of stable embryonic stem cells. Here, we report that CRISPR/Cas9-mediated gene targeting can be applied to the Japanese wild-derived MSM/Ms strain (Mus musculus molossinus). We targeted the nonagouti (a) gene encoding the agouti protein that is localized in hair and the brain. We obtained three homozygous knockout mice as founders, all showing black coat colour. While homozygous knockout offspring were physiologically indistinguishable from wild-type litter-mates, they showed specific domesticated behaviours: hypoactivity in the dark phase and a decline in the avoidance of a human hand. These phenotypes were consistent over subsequent generations. Our findings support the empirical hypothesis that nonagouti is a domestication-linked gene, the loss of which might repress aggressive behaviour.
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http://dx.doi.org/10.1038/srep42476DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5307340PMC
February 2017

High PR3-ANCA positivity in a patient with chronic inflammatory demyelinating polyneuropathy.

eNeurologicalSci 2017 Mar 5;6:4-5. Epub 2016 Oct 5.

Department of Neurology, Mitsui Memorial Hospital, Tokyo, Japan.

Proteinase 3 anti-neutrophil cytoplasmic antibody (PR3-ANCA) is reported to be highly specific to vasculitis compared to myeloperoxidase (MPO)-ANCA. We report a case of a 19-year-old woman with chronic inflammatory demyelinating polyneuropathy (CIDP) with high PR3-ANCA positivity. The patient responded well to intravenous immunoglobulin plus oral steroid, and showed no signs of systemic vasculitis during the subsequent 10 months of follow-up. Our present case suggests that CIDP may accompany high PR3-ANCA levels, which should be differentiated from axonal neuropathy due to vasculitis.
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http://dx.doi.org/10.1016/j.ensci.2016.10.001DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5721574PMC
March 2017

Stair climbing/descending exercise for a short time decreases blood glucose levels after a meal in people with type 2 diabetes.

BMJ Open Diabetes Res Care 2016 25;4(1):e000232. Epub 2016 Jul 25.

Laboratory of Sports and Exercise Medicine , Graduate School of Human and Environmental Studies, Kyoto University , Kyoto , Japan.

Objective: We examined whether stair climbing-descending exercise (ST-EX), a convenient method to increase physical activity in daily life, for a short period would acutely improve the postprandial blood glucose (BG) response in people with type 2 diabetes (T2D).

Methods: 16 people with T2D (age 65.4±1.1 years) participated in 2 separate sessions. After an overnight fast, each participant consumed a test meal and then kept resting for 180 min, except when performing each 3 min bout of ST-EX at 60 and 120 min after the meal (ST-EX session), or kept resting for 180 min (REST session). ST-EX comprised 6 continuous repetitions of climbing to the second floor (21 steps) at a rate of 80-110 steps/min followed by walking down slowly to the first floor at a free step rate.

Results: The BG at 60 min after the meal during the ST-EX session (immediately before the first ST-EX) did not differ from that during the REST session, but analysis of variance revealed a significant interaction between time and treatment (p<0.01). The BG at 150 min after the meal (30 min after the second ST-EX) was significantly lower than that during the REST session (p<0.01). The area under the curve was also 18% lower during the ST-EX session than during the REST session (p<0.05). The heart rate and blood lactate levels indicated that the actual intensity of ST-EX was 'hard'. In contrast, the rating of perceived exertion (RPE) indicated that the overall intensity of ST-EX was 'moderate' because of decreased RPE scores during descent.

Conclusions: The present findings suggest that performing 3 min ST-EX 60 and 120 min after a meal may be a useful strategy to accelerate the decrease in postprandial BG levels in people with T2D.
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http://dx.doi.org/10.1136/bmjdrc-2016-000232DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4964213PMC
August 2016

Mouse D1Pas1, a DEAD-box RNA helicase, is required for the completion of first meiotic prophase in male germ cells.

Biochem Biophys Res Commun 2016 09 27;478(2):592-8. Epub 2016 Jul 27.

RIKEN BioResource Center, Tsukuba, Ibaraki, 305-0074, Japan; Graduate School of Life and Environmental Science, University of Tsukuba, Ibaraki, 305-8572, Japan; The Center for Disease Biology and Integrative Medicine, Faculty of Medicine, University of Tokyo, Tokyo, 113-0033, Japan. Electronic address:

D1Pas1 is a mouse autosomal DEAD-box RNA helicase expressed predominantly in the testis. To assess its possible function, we generated D1Pas1-deficient mice using embryonic stem cells with a targeted D1Pas1 allele. Deletion of D1Pas1 did not cause noticeable embryonic defects or death, indicating that D1Pas1 is not essential for embryogenesis. Whereas homozygous knockout female mice showed normal reproductive performance, homozygous knockout male mice were completely sterile. The seminiferous epithelium of D1Pas1-deficient males contained no spermatids or spermatozoa because of spermatogenic arrest at the late pachytene stage. Upregulation of retrotransposons such as LINE-1 was not found in D1Pas1-deficient males, unlike males lacking Mvh, another testicular DEAD-box RNA helicase. Meiotic chromosome behavior in developing spermatocytes of D1Pas1-deficient males was indistinguishable from that in wild-type males, at least until synaptonemal complex formation. Thus, mouse D1Pas1 is the first-identified DEAD-box RNA helicase that plays critical roles in the final step of the first meiotic prophase in male germ cells.
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http://dx.doi.org/10.1016/j.bbrc.2016.07.109DOI Listing
September 2016

Anti-TIF1-γ antibody and cancer-associated myositis: A clinicohistopathologic study.

Neurology 2016 07 24;87(3):299-308. Epub 2016 Jun 24.

Objective: We aimed to analyze the clinical and histopathologic features of cancer-associated myositis (CAM) in relation to anti-transcriptional intermediary factor 1 γ antibody (anti-TIF1-γ-Ab), a marker of cancer association.

Methods: We retrospectively studied 349 patients with idiopathic inflammatory myopathies (IIMs), including 284 patients with pretreatment biopsy samples available. For the classification of IIMs, the European Neuromuscular Center criteria were applied. Patients with CAM with (anti-TIF1-γ-Ab[+] CAM) and without anti-TIF1-γ-Ab (anti-TIF1-γ-Ab[-] CAM) were compared with patients with IIM without cancers within and beyond 3 years of myositis diagnosis.

Results: Cancer was detected in 75 patients, of whom 36 (48%) were positive for anti-TIF1-γ-Ab. In anti-TIF1-γ-Ab(+) patients with CAM, cancers were detected within 1 year of myositis diagnosis in 35 (97%) and before 1 year of myositis diagnosis in 1. All the anti-TIF1-γ-Ab(+) patients with CAM satisfied the dermatomyositis (DM) criteria, including 2 possible DM sine dermatitis cases, and were characterized histologically by the presence of perifascicular atrophy, vacuolated fibers (VFs), and dense C5b-9 deposits on capillaries (dC5b-9). In contrast, 39 anti-TIF1-γ-Ab(-) patients with CAM were classified into various subgroups, and characterized by a higher frequency of necrotizing autoimmune myopathy (NAM). Notably, all 7 patients with CAM classified into the NAM subgroup were anti-TIF1-γ-Ab(-) and exhibited no dC5b-9 or VFs.

Conclusions: CAM includes clinicohistopathologically heterogeneous disease entities. Among CAM entities, anti-TIF1-γ-Ab(+) CAM has characteristically shown a close temporal association with cancer detection and the histopathologic findings of dC5b-9 and VFs, and CAM with NAM is a subset of anti-TIF1-γ-Ab(-) CAM.
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http://dx.doi.org/10.1212/WNL.0000000000002863DOI Listing
July 2016

Cerebral air embolism likely due to pulmonary Mycobacterium avium complex disease.

J Neurol Sci 2016 Jun 20;365:188-9. Epub 2016 Apr 20.

Department of Neurology, Teikyo University School of Medicine, Kaga 2-11-1, Itabashi-ku, Tokyo 1738605, Japan. Electronic address:

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http://dx.doi.org/10.1016/j.jns.2016.04.036DOI Listing
June 2016