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  1. Article ; Online: Sjogren's syndrome related autoimmune encephalopathy presenting as depression and involuntary agitation in a 15-year-old girl: a case report.

    Lee, Yu-Tung / Wu, Chao-Yi / Chou, I-Jun / Liu, Chun-Hao

    Frontiers in immunology

    2023  Volume 14, Page(s) 1145948

    Abstract: Introduction: Sjogren's syndrome is an autoimmune disease that commonly involves exocrinopathy. Although studies have reported psychiatric manifestations resulting from Sjogren's syndrome, few studies have focused on such manifestations in pediatric ... ...

    Abstract Introduction: Sjogren's syndrome is an autoimmune disease that commonly involves exocrinopathy. Although studies have reported psychiatric manifestations resulting from Sjogren's syndrome, few studies have focused on such manifestations in pediatric patients. Herein, we present a case of an adolescent girl with depression and involuntary self-harm behaviors related to Sjogren's syndrome with central nervous system involvement.
    Case presentation: A 15-year-old girl, with an underlying history of epilepsy, developed depressive symptoms of a year's duration, accompanied by three seizure episodes and involuntary self-harm behaviors. The self-harm behaviors, which included head banging and arm scratching, were sudden onset, involuntary, and unable to be recalled afterwards. After admission to our ward, the patient was positive for serum antinuclear antibodies and Schirmer's test. Moreover, 24-hour electroencephalography revealed epileptiform discharges during the mood swing episodes. Positive findings for antinuclear antibodies and anti-SSA antibodies in both serum and cerebrospinal fluid, suggested central nervous system involvement in Sjogren's syndrome. After rituximab treatment, her mood became euthymic, and her involuntary self-harm behaviors ceased.
    Conclusion: Central nervous system involvement leading to psychiatric presentations has rarely been reported in adolescents with Sjogren's syndrome. When treating adolescent patients with involuntary self-harm behaviors and neurological symptoms, it is crucial to consider autoimmune encephalitis related to Sjogren's syndrome in the differential diagnosis.
    MeSH term(s) Humans ; Female ; Child ; Adolescent ; Sjogren's Syndrome/complications ; Sjogren's Syndrome/diagnosis ; Sjogren's Syndrome/drug therapy ; Antibodies, Antinuclear ; Depression/etiology ; Autoimmune Diseases/diagnosis ; Autoimmune Diseases/drug therapy ; Autoimmune Diseases/complications ; Encephalitis/etiology ; Encephalitis/complications
    Chemical Substances Antibodies, Antinuclear
    Language English
    Publishing date 2023-06-29
    Publishing country Switzerland
    Document type Case Reports
    ZDB-ID 2606827-8
    ISSN 1664-3224 ; 1664-3224
    ISSN (online) 1664-3224
    ISSN 1664-3224
    DOI 10.3389/fimmu.2023.1145948
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  2. Article: Long-Term Outcome of Neonatal Seizure with

    Chou, I-Jun / Hou, Ju-Yin / Fan, Wen-Lang / Tsai, Meng-Han / Lin, Kuang-Lin

    Children (Basel, Switzerland)

    2023  Volume 10, Issue 4

    Abstract: Phosphofurin Acidic Cluster Sorting Protein 2 (PACS2) ...

    Abstract Phosphofurin Acidic Cluster Sorting Protein 2 (PACS2)
    Language English
    Publishing date 2023-03-26
    Publishing country Switzerland
    Document type Journal Article
    ZDB-ID 2732685-8
    ISSN 2227-9067
    ISSN 2227-9067
    DOI 10.3390/children10040621
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  3. Article: Natalizumab Treatment for Relapsing Multiple Sclerosis Stabilises Normal-Appearing White Matter Microstructure: A One-Year Prospective Ultra-High-Field Quantitative Imaging Study.

    Tanasescu, Radu / Mougin, Olivier / Chou, I-Jun / Al-Radaideh, Ali / Jerca, Oltita P / Lim, Su-Yin / Gowland, Penny / Constantinescu, Cris S

    Brain sciences

    2023  Volume 13, Issue 10

    Abstract: 1) Background: Natalizumab dramatically reduces relapses and MRI inflammatory activity (new lesions and enhancing lesions) in multiple sclerosis (MS). Chemical exchange saturation transfer (CEST) MRI can explore brain tissue in vivo with high resolution ...

    Abstract (1) Background: Natalizumab dramatically reduces relapses and MRI inflammatory activity (new lesions and enhancing lesions) in multiple sclerosis (MS). Chemical exchange saturation transfer (CEST) MRI can explore brain tissue in vivo with high resolution and sensitivity. We investigated if natalizumab can prevent microstructural tissue damage progression measured with MRI at ultra-high field (7 Tesla) over the first year of treatment. (2) Methods: In this one-year prospective longitudinal study, patients with active relapsing-remitting MS were assessed clinically and scanned at ultra-high-field MRI at the time of their first natalizumab infusion, at 6 and 12 months, with quantitative imaging aimed to detect microstructural changes in the normal-appearing white matter (NAWM), including sequences sensitive to magnetisation transfer (MT) effects from amide proton transfer (MTRAPT) and the nuclear Overhauser effect (MTRNOE). (3) Results: 12 patients were recruited, and 10 patients completed the study. The difference in the T1 relaxation times at month 6 and month 12 of natalizumab treatment was not significant, suggesting the lack of accumulation of tissue damage, while improvements were seen in MTR (MTRAPT and MTRNOE measures) at month 12, suggesting a tissue repair effect. This paralleled the expected lack of clinical and radiological worsening of conventional MRI measures of disease activity (new lesions or gadolinium-enhancing lesions). (4) Conclusion: Natalizumab prevents microstructural brain damage and has effects suggesting an improved white matter microstructure measured at ultra-high field during the first year of treatment.
    Language English
    Publishing date 2023-10-17
    Publishing country Switzerland
    Document type Journal Article
    ZDB-ID 2651993-8
    ISSN 2076-3425
    ISSN 2076-3425
    DOI 10.3390/brainsci13101464
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  4. Article ; Online: Anterior chest pain as an unusual initial presentation in pediatric spinal arteriovenous malformations.

    Hung, Po-Cheng / Chou, I-Jun / Wu, Chieh-Tsai / Wong, Ho-Fai

    Pediatrics and neonatology

    2020  Volume 61, Issue 5, Page(s) 551–553

    Language English
    Publishing date 2020-05-25
    Publishing country Singapore
    Document type Journal Article
    ZDB-ID 2441816-X
    ISSN 2212-1692 ; 1875-9572
    ISSN (online) 2212-1692
    ISSN 1875-9572
    DOI 10.1016/j.pedneo.2020.05.002
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  5. Article: Unusual Presentations of Birth Related Cervical Spinal Cord Injury.

    Lee, Chien-Chung / Chou, I-Jun / Chang, Yi-Jung / Chiang, Ming-Chou

    Frontiers in pediatrics

    2020  Volume 8, Page(s) 514

    Abstract: Neonatal spinal cord injury is a rare complication of birth trauma by difficult delivery. The typical manifestations are often catastrophic, include decreased or absent movement, loss of reflexes, apnea or periodic breathing, and a lack of response to ... ...

    Abstract Neonatal spinal cord injury is a rare complication of birth trauma by difficult delivery. The typical manifestations are often catastrophic, include decreased or absent movement, loss of reflexes, apnea or periodic breathing, and a lack of response to painful stimulation. The outcome is usually fatal or severe, with long-term sequelae of respiratory insufficiency, limb weakness, or even paralysis of the limbs. We described a male neonate with a C2 spinal cord injury who was born smoothly by vaginal delivery and was unnoticed initially due to unusual subtle symptoms. He presented with a hoarse voice, swallowing dysfunction, decreased movement of upper limbs, and hypercapnia. After receiving corticosteroid therapy and rehabilitation, he recovered much except that he still needed ventilator support at night.
    Language English
    Publishing date 2020-09-29
    Publishing country Switzerland
    Document type Case Reports
    ZDB-ID 2711999-3
    ISSN 2296-2360
    ISSN 2296-2360
    DOI 10.3389/fped.2020.00514
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  6. Article: The clinical relevance of anti-glutamic acid decarboxylase antibodies in children with encephalitis/encephalopathy.

    Hou, Ju-Yin / Liu, Hsin-Uei / Kuo, Cheng-Yen / Liu, Yi-Hsuan / Lin, Jainn-Jim / Hsieh, Meng-Ying / Hung, Po-Cheng / Cheng, Yi-Ting / Su, I-Chen / Wang, Huei-Shyong / Chou, I-Jun / Lin, Kuang-Lin

    Frontiers in neuroscience

    2023  Volume 16, Page(s) 1081580

    Abstract: Anti-glutamic acid decarboxylase (anti-GAD) antibodies are associated with different types of syndromes. However, few studies have investigated the correlation between anti-GAD antibody titers with clinical severity and outcomes in children with ... ...

    Abstract Anti-glutamic acid decarboxylase (anti-GAD) antibodies are associated with different types of syndromes. However, few studies have investigated the correlation between anti-GAD antibody titers with clinical severity and outcomes in children with encephalitis/encephalopathy. In this single-center retrospective cohort study, we consecutively enrolled hospitalized children who had encephalitis and/or encephalopathy with positive anti-GAD antibodies in serum and/or cerebrospinal fluid (CSF) from February 2010 to October 2021. Thirty-seven patients were included and divided into high-titer and low-titer groups. The patients with high anti-GAD antibody titers were associated with initial symptoms of language difficulty and ataxia. The level of titers was not associated with severity or outcomes. Anti-GAD antibody titers decreased after immunotherapy, however, the clinical response to immunotherapy was variable. A transient elevation in anti-GAD antibody titers during immunotherapy was noted. Further studies are warranted to investigate the role of anti-GAD antibodies in the pathogenesis and immune mechanisms of encephalitis/encephalopathy.
    Language English
    Publishing date 2023-02-02
    Publishing country Switzerland
    Document type Journal Article
    ZDB-ID 2411902-7
    ISSN 1662-453X ; 1662-4548
    ISSN (online) 1662-453X
    ISSN 1662-4548
    DOI 10.3389/fnins.2022.1081580
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  7. Article: Natalizumab Treatment of Relapsing Remitting Multiple Sclerosis Has No Long-Term Effects on the Proportion of Circulating Regulatory T Cells.

    Tanasescu, Radu / Frakich, Nanci / Chou, I-Jun / Filippini, Perla / Podda, Giulio / Xin, Gao / Muraleedharan, Ranjithmenon / Jerca, Oltita / Onion, David / Constantinescu, Cris S

    Neurology and therapy

    2023  Volume 12, Issue 6, Page(s) 2041–2052

    Abstract: Introduction: Natalizumab (NTZ), a monoclonal antibody against the integrin α4β1 (VLA-4) found on activated T cells and B cells, blocks the interaction of this integrin with adhesion molecules of central nervous system (CNS) endothelial cells and ... ...

    Abstract Introduction: Natalizumab (NTZ), a monoclonal antibody against the integrin α4β1 (VLA-4) found on activated T cells and B cells, blocks the interaction of this integrin with adhesion molecules of central nervous system (CNS) endothelial cells and lymphocyte migration through the blood-brain barrier, effectively preventing new lesion formation and relapses in multiple sclerosis (MS). Whether NTZ treatment has additional effects on the peripheral immune system cells, and how its actions compare with other MS disease-modifying treatments, have not been extensively investigated. In particular, its effect on the proportions of circulating regulatory T cells (Treg) is unclear.
    Methods: In this study, we investigated the effect of NTZ treatment in 12 patients with relapsing MS, at 6 and 12 months after the start of treatment. We evaluated the proportions of regulatory T cells (Treg), defined by flow cytometry as CD4+ CD25++ FoxP3+ cells and CD4+ CD25++ CD127- cells at these intervals. As an exploratory study, we also investigated the NTZ effects on the proportions of bulk T and B lymphocyte populations, and of those expressing novel the markers CD195 (CCR5), CD196 (CCR6), or CD161 (KLRB1), which are involved in MS pathogenesis but have been studied less in the context of MS treatment. The effects of NTZ were compared to those obtained with 11 patients under interferon-beta-1a (IFN-β1a) treatment, and against 9 healthy volunteers.
    Results: We observed a transient increment in the proportion of Treg cells at 6 months, which was not sustained at 12 months. We observed a reduction in the proportion of T cells expressing CD195 (CCR5) and CD161 (KLRB1) subsets of T cells.
    Conclusion: We conclude that NTZ does not have an effect on the proportion of Treg cells over 1 year, but it may affect the expression of molecules important for some aspects MS pathogenesis, in a manner that is not shared with IFN-β1a.
    Language English
    Publishing date 2023-09-16
    Publishing country New Zealand
    Document type Journal Article
    ISSN 2193-8253
    ISSN 2193-8253
    DOI 10.1007/s40120-023-00539-9
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  8. Article ; Online: The impact of epilepsy and antiseizure medications on pregnancy and neonatal outcomes: A nationwide cohort study.

    Kuo, Cheng-Yen / Kuo, Chang-Fu / See, Lai-Chu / Chiou, Meng-Jiung / Hung, Po-Cheng / Lin, Jainn-Jim / Lin, Kuang-Lin / Wang, Huei-Shyong / Chou, I-Jun

    Brain and behavior

    2023  Volume 13, Issue 12, Page(s) e3287

    Abstract: Purpose: Our objective was to assess the adverse outcomes during pregnancy, as well as for the fetus and neonates, in women with epilepsy, both with and without the use of antiseizure medications (ASMs).: Methods: A cohort of singleton pregnancies ... ...

    Abstract Purpose: Our objective was to assess the adverse outcomes during pregnancy, as well as for the fetus and neonates, in women with epilepsy, both with and without the use of antiseizure medications (ASMs).
    Methods: A cohort of singleton pregnancies between January 1, 2004 and December 31, 2014 was identified using the Taiwan National Health Database. The pregnancies were categorized into ASM exposure, ASM nonexposure, and control (consisting of women without an epilepsy diagnosis) groups. We recorded adverse outcomes in neonates and documented pregnancy complications. The generalized estimating equation with logit link was used to estimate adjusted odds ratios.
    Results: There were 629 singleton pregnancies in the group exposed to ASMs, 771 in the epilepsy group without ASM exposure, and 2,004,479 in the control group. Women with epilepsy had a significantly higher risk of puerperal cerebrovascular diseases (adjusted odds ratios in the exposure and nonexposure groups = 54.46 and 20.37, respectively), respiratory distress syndrome (5.1 and 2.99), mortality (3.15 and 3.22), sepsis (2.67 and 2.54), pregnancy-related hypertension (1.71 and 1.8), preeclampsia (1.87 and 1.79), cesarean delivery (1.72 and 2.15), and preterm labor (1.38 and 1.56). The use of ASMs may increase the risk of eclampsia (adjusted odds ratio = 12.27). Compared to controls, fetuses/neonates born to women with epilepsy had a higher risk of unexplained stillbirth (adjusted odds ratios in the exposure and nonexposure groups = 2.51 and 2.37, respectively), congenital anomaly (1.37 and 1.33), central nervous system malformation (3.57 and 2.25), low birth weight (1.90 and 1.97), and a low Apgar score at 5 min (2.63 and 1.3). The use of ASMs may introduce an additional risk of small for gestational age; the adjusted odds ratio was 1.51.
    Conclusion: Women with epilepsy, irrespective of their exposure to ASMs, had a slightly elevated risk of pregnancy and perinatal complications. Puerperal cerebrovascular diseases may be a hidden risk for women with epilepsy.
    MeSH term(s) Pregnancy ; Infant, Newborn ; Humans ; Female ; Cohort Studies ; Epilepsy/drug therapy ; Epilepsy/epidemiology ; Pregnancy Complications/drug therapy ; Pregnancy Complications/epidemiology ; Infant, Small for Gestational Age ; Cerebrovascular Disorders
    Language English
    Publishing date 2023-10-14
    Publishing country United States
    Document type Journal Article ; Research Support, Non-U.S. Gov't
    ZDB-ID 2623587-0
    ISSN 2162-3279 ; 2162-3279
    ISSN (online) 2162-3279
    ISSN 2162-3279
    DOI 10.1002/brb3.3287
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  9. Article ; Online: Rhombencephalitis in Children: Diffusion Magnetic Resonance Imaging (MRI) Correlation With Clinical Outcomes.

    Wong, Alex Mun-Ching / Yeh, Chih-Hua / Lin, Jainn-Jim / Chou, I-Jun / Lin, Kuang-Lin

    Journal of child neurology

    2020  Volume 35, Issue 6, Page(s) 404–409

    Abstract: In children with rhombencephalitis, neuroimaging abnormalities have been infrequently correlated with clinical outcome. We investigated whether magnetic resonance (MR) neuroimaging studies could predict clinical outcomes and disturbance of consciousness ... ...

    Abstract In children with rhombencephalitis, neuroimaging abnormalities have been infrequently correlated with clinical outcome. We investigated whether magnetic resonance (MR) neuroimaging studies could predict clinical outcomes and disturbance of consciousness in patients with rhombencephalitis. We retrospectively analyzed the MR studies of 19 pediatric patients with rhombencephalitis (median age: 4.2 years, range 0.5-17; sex: 32% male). Fluid-attenuated inversion recovery imaging and diffusion-weighted imaging findings were graded to create imaging scores according to the extent of imaging abnormality. Clinical outcomes in the first week and 12th month were graded by using Glasgow Outcome Scale scores (1-5) and dichotomized to unfavorable or favorable outcome. Correlations of the imaging scores with the clinical outcomes and with disturbance of consciousness were assessed by using multivariate logistic regression analysis. No significant correlation was found between fluid-attenuated inversion recovery score or diffusion-weighted imaging score (
    MeSH term(s) Adolescent ; Brain/diagnostic imaging ; Child ; Child, Preschool ; Diffusion Magnetic Resonance Imaging ; Female ; Humans ; Infant ; Male ; Retrospective Studies ; Rhombencephalon/diagnostic imaging
    Language English
    Publishing date 2020-03-03
    Publishing country United States
    Document type Journal Article ; Research Support, Non-U.S. Gov't
    ZDB-ID 639288-x
    ISSN 1708-8283 ; 0883-0738
    ISSN (online) 1708-8283
    ISSN 0883-0738
    DOI 10.1177/0883073820904480
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  10. Article ; Online: Electroencephalogram pattern predicting neurological outcomes of children with seizures secondary to abusive head trauma.

    Chou, Cheng-Che / Hou, Ju-Yin / Chou, I-Jun / Lan, Shih-Yun / Kong, Shu-Sing / Huang, Man-Hsu / Weng, Yu-Chieh / Lin, Yi-Yu / Kuo, Cheng-Yen / Hsieh, Meng-Ying / Chou, Min-Liang / Hung, Po-Cheng / Wang, Huei-Shyong / Lin, Kuang-Lin / Wang, Yi-Shan / Lin, Jainn-Jim

    Pediatrics and neonatology

    2023  

    Abstract: Background: The clinical presentations of abusive head trauma can abruptly worsen, so the occurrence of seizures and changes of EEG can be variable according to patients' conditions. Since the changes of EEG background waves reflect the cortical ... ...

    Abstract Background: The clinical presentations of abusive head trauma can abruptly worsen, so the occurrence of seizures and changes of EEG can be variable according to patients' conditions. Since the changes of EEG background waves reflect the cortical function of children, we aimed to find out whether the timing of EEG background, epileptiform discharges and seizure patterns were associated with the outcomes of patients with AHT.
    Material and methods: Using seizure type and acute stage electroencephalographic (EEG) characteristics to assess adverse neurological outcomes in children with seizures secondary to abusive head trauma (AHT). Children who were hospitalized with AHT at a tertiary referral hospital from October 2000 to April 2010 were evaluated retrospectively. A total of 50 children below 6 years of age admitted due to AHT were included. KOSCHI outcome scale was used to evaluate the primary outcome and neurological impairment was used as secondary outcome after 6 months discharge.
    Results: Children with apnea, cardiac arrest, reverse blood flow and skull fracture in clinic had a higher mortality rate even in the no-seizure group (3/5 [60%] vs. 3/45 [6.7%], odds ratio [OR] = 11; 95% CI = 2.3-52; p = 0.025). Seizure occurrence reduced mostly at the second day after admission in seizure groups; but children with persistent seizures for 1 week showed poor neurological outcomes. The occurrence of initial seizure was frequency associated with younger age; focal seizure, diffuse cortical dysfunction in acute-stage EEG, and low Glasgow Coma Scale (GCS) score were significantly related to poor outcomes after 6 months. Diffuse cortical dysfunction was also associated with motor, speech, and cognitive dysfunction.
    Conclusions: Diffuse cortical dysfunction in acute-stage EEG combined with low GCS score and focal seizure may related to poor outcomes and neurological dysfunctions in children with AHT.
    Language English
    Publishing date 2023-10-21
    Publishing country Singapore
    Document type Journal Article
    ZDB-ID 2441816-X
    ISSN 2212-1692 ; 1875-9572
    ISSN (online) 2212-1692
    ISSN 1875-9572
    DOI 10.1016/j.pedneo.2023.05.012
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