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Concomitant achondroplasia and Chiari II malformation: A double-hit at the cervicomedullary junction

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World Journal of Clinical Cases 2014年第11期2卷 711-716页

作者： Al-Wala Awad Kyrieckos A Aleck Ratan D Bhardwaj College of Medicine-phoenix University of Arizona Phoenix AZ 85004 United States Division of Genetics and Metabolism Phoenix Children’s Hospital Phoenix AZ 85016 United States Division of neurological surgery Barrow Neurological Institute at Phoenix Children’s Hospital PhoenixAZ 85016 United States

We report the first case of a neonate with concurrent Chiari II malformation and achondroplasia. Although rare, both these conditions contribute to several deleterious anatomical changes at the cervicomedullary junction and thus predispose to acute hydrocephalus. Although our patient was initially asymptomatic, hydrocephalus ensued several weeks after birth and required cerebral spinal fluid diversion. We discuss the potential links between the two conditions, the pathophysiology, and the important clinical implications for the management of the increased risk of hydrocephalus.

关键词： Achondroplasia Chiari II malformation Hydrocephalus shunt failure Cervicomedullary junction Cerebral spinal fluid diversion

Female sex in experimental traumatic brain injury research:forging a path forward

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Neural Regeneration Research 2022年第3期17卷 550-552页

作者： Theresa Currier Thomas Caitlin E.Bromberg Gokul Krishna Department of Child Health University of Arizona College of Medicine-Phoenix barrow neurological institute at phoenix children’s hospital PhoenixAZUSA phoenix VA Health Care system PhoenixAZUSA Arizona state University TempeAZUSA

Traumatic brain injury(TBI) is a leading cause of death and disability worldwide,with estimates indicating that -50% of the world's population will acquire a head injury at some point in their lifetime(Maas et al.,2017).Mild TBIs account for -80% of all reported cases,with up to 43% of all TBI patients reporting symptoms beyond 2 weeks *** of 2016,only 7% of preclinical TBI experiments included both male and female sexes,and fewer studies analyzed the data using sex as a biological variable(spani et al.,2018).

关键词： injury death

Clinical significance of detection of antibodies to fetal and adult acetylcholine receptors in myasthenia gravis

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Neuroscience Bulletin 2012年第5期28卷 469-474页

作者： Qi-Guang shi Zhi-Hong Wang Xiao-Wei Ma Da-Qi Zhang Chun-sheng Yang Fu-Dong shi Li Yang Department of Neurology and Tianjin neurological institute Tianjin Medical University General Hospital Tianjin 300052 China Department of Neurology Barrow Neurological Institute St. Joseph's Hospital and Medical Center Phoenix Arizona 85013 USA

Objective To evaluate the frequency, distribution and clinical significance of the antibodies to the fetal and/or adult acetylcholine receptor （AChR） in patients with myasthenia gravis （MG）. Methods AChR antibodies were detected by cell-based assay in the serum of ocular MG （OMG）（n = 90） and generalized MG （GMG） patients （n = 110）. The fetal-type （2α： β： γ： δ） and adult-type （2α： β： ε： δ） AChR were used as antigens, and their relevance to disease presentation was assessed. Results The overall frequencies of anti-adult and anti-fetal AChR antibodies were similar in all 200 patients examined, with 14 having serum specific to the AChR-γ subunit, and 22 to the AChR-ε subunit. The overall sensitivity when using the fetal and adult AChR antibodies was higher than that when using the fetal AChR antibody only （P = 0.015）. Compared with OMG patients, the mean age at disease onset and the positive ratio of antibodies to both isoforms of the AChR were significantly higher in patients who subsequently progressed to GMG. Older patients and patients with both anti-fetal and anti-adult AChR antibodies had a greater risk for developing generalized disease [odds ratio （OR）, 1.03; 95% confidence interval （CI）, 1.01–1.06 and OR, 5.09; 95% CI, 2.23–11.62]. Conclusion Using both fetal-and adult-type AChRs as the antigens may be more sensitive than using either subtype. Patients with serum specific to both isoforms are at a greater risk of progressing to GMG. Patients with disease onset at an advanced age appear to have a higher frequency of GMG conversion.

关键词： myasthenia gravis acetylcholine receptor antibodies acetylcholine receptor subunit cell-based assay adult fetal

Precision antiplatelet therapy for the prevention of ischaemic stroke

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stroke & Vascular Neurology 2022年第2期7卷 89-91页

作者： David Wang Neurovascular Division Department of NeurologyBarrow Neurological InstituteSt.Joseph's Hospital and Medical CenterPhoenixArizonaUSA

stroke happens to people of all ages and has become the leading cause of disability in the world.1 It is also the leading cause of death in China and fifth-leading cause of death in the UsA.23 Prevention of stroke is the best *** use of antiplatelet therapy is one of the main prevention *** is the only antiplatelet drug indicated for primary or secondary stroke *** the invention of aspirin nearly 120 years ago,more antiplatelet agents are *** can select one antiplatelet drug or the combination for a specific patient with a specific subtype of ischaemic *** kind of precision antiplatelet therapy can maximise the benefit and lower the risk of haemorrhagic complications.

关键词： prevention death Aspirin

Experimental models of neuromyelitis optica: current status, challenges and future directions

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Neuroscience Bulletin 2015年第6期31卷 735-744页

作者： Minshu Li Yaping Yan Department of Neurology Tianjin Neurological InstituteTianjin Medical University General Hospital Department of Neurology Barrow Neurological InstituteSt. Joseph's Hospital and Medical Center

Neuromyelitis optica （NMO） is a recurrent inflammatory disease that predominantly attacks the optic nerves and spinal cord. NMO-IgG, the specific autoantibody present in the vast majority of NMO patients, targets the astrocytic water channel protein aquaporin 4 （AQP4）, and differentiates NMO from multiple sclerosis. The growing clinical and research interest in NMO makes it urgent to produce an animal model of NMO. The pathogenic effect of anti-AQP4 antibodies derived from the serum of patients paves the way to generating an experimental model based on the anti-AQP4-mediated astrocyte damage. In this review, we discuss the contribution of experimental models to the understanding of the pathogenesis of the disease and drug development. Key questions raised by the existing models are also discussed.

关键词： neuromyelitis optica animal model NMO-IgG astrocyte

Advances in the imaging of cerebral aneurysm inflammation

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Neuroimmunology and Neuroinflammation 2015年第1期2卷 51-54页

作者： Michael R.Levitt M.Yashar s.Kalani Karam Moon Cameron G.McDougall Felipe C.Albuquerque Division of neurological surgery Barrow Neurological InstituteSt.Joseph’s Hospital and Medical CenterPhoenixAZ 85013USA

Cerebral aneurysm formation,growth and rupture are thought to be the result of a complex interaction between cerebrovascular hemodynamics and ***,new evidence has emerged regarding the role of inflammation in the walls of cerebral *** methods to characterize the degree of inflammation in aneurysms could enable clinicians to estimate the risk of future aneurysm growth and rupture,influencing *** review examines emerging techniques of imaging inflammatory biomarkers in cerebral aneurysms.

关键词： Ferrosoferric oxide inflammation intracranial aneurysm magnetic resonance angiography subarachnoid hemorrhage

Lipid mediators of inflammation in neurological injury： shifting the balance toward resolution

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Neural Regeneration Research 2016年第1期11卷 77-78页

作者： Jordan L.Harrison Rachel K.Rowe Jonathan Lifshitz barrow neurological institute at phoenix children's hospital Phoenix AZ USA Interdisciplinary Graduate Program in Neuroscience Arizona State University Tempe AZ USA Department of Child Health University of Arizona College of Medicine - Phoenix Phoenix AZ USA phoenix Veteran Affairs Healthcare system Phoenix AZ USA

Acquired neurological injuries initiate a pathological cascade of secondary injury processes,including inflammation,which continue for days to weeks following ***-induced neuroinflammation acts as a host defense mechanism contributing to the neutralization of the insult（removing offending factors）and restoring structure and function of the brain（establish homeostasis）.The timing of these protective functions of the immune response is vital,since chronic inflammation

关键词： inflammation neurological homeostasis initiate protective removing vital toward contributing cascade

A liquid chromatography with tandem mass spectrometry method for quantitating total and unbound ceritinib in patient plasma and brain tumor

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Journal of Pharmaceutical Analysis 2018年第1期8卷 20-26页

作者： Xun Bao Jianmei Wu Nader sanai Jing Li Karmanos Cancer institute Wayne State University barrow neurological institute St. Joseph's Hospital& Medical Center

A rapid, sensitive, and robust reversed-phase liquid chromatography with tandem mass spectrometry method was developed and validated for the determination of total and unbound ceritinib, a secondgeneration ALK inhibitor, in patient plasma and brain tumor tissue samples. sample preparation involved simple protein precipitation with acetonitrile. Chromatographic separation was achieved on a Waters ACQUITY UPLC BEH C_(18) column using a 4-min gradient elution consisting of mobile phase A(0.1% formic acid in water) and mobile phase B(0.1% formic acid in acetonitrile), at a flow rate of 0.4 m L/min. Ceritinib and the internal standard([^(13)C_6]ceritinib) were monitored using multiple reaction monitoring mode under positive electrospray ionization. The lower limit of quantitation(LLOQ) was 1 n M of ceritinib in plasma. The calibration curve was linear over ceritinib concentration range of 1–2000 n M in plasma. The intra-and interday precision and accuracy were within the generally accepted criteria for bioanalytical method( o15%).The method was successfully applied to assess ceritinib brain tumor penetration, as assessed by the unbound drug brain concentration to unbound drug plasma concentration ratio, in patients with brain tumors.

关键词： Ceritinib Reversed-phase liquid chromatography with tandem mass spectrometry (LC–Ms/Ms) Fraction unbound in plasma Fraction unbound in brain tissue Brain tumor penetration Unbound brain-to-plasma partition coefficient

sodium and water perturbations in patients who had an acute stroke:clinical relevance and management strategies for the neurologist

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stroke & Vascular Neurology 2022年第3期7卷 258-266页

作者： Kevin C J Yuen Valerie sharf Elizabeth smith Minhwan Kim Alissya s M Yuen Paul R MacDonald Departments of Neuroendocrinology and Neurosurgery Barrow Neurological InstitutePhoenixArizonaUSA University of Arizona College of Medicine and Creighton school of Medicine PhoenixArizonaUSA Department of Neurology Barrow Neurological InstitutePhoenixArizonaUSA Midwestern University GlendaleIllinoisUSA Creighton University school of Medicine PhoenixArizonaUSA University of California Davis DavisCaliforniaUSA

sodium and water perturbations,manifesting as hyponatraemia and hypernatraemia,are common in patients who had an acute stroke,and are associated with worse outcomes and increased *** non-stroke related causes of sodium and water perturbations in these patients include underlying comorbidities and concomitant ***,hospitalised patients who had an acute stroke may receive excessive intravenous hypotonic solutions,have poor fluid intake due to impaired neurocognition and consciousness,may develop sepsis or are administered drugs(eg,mannitol);factors that can further alter serum sodium *** and water perturbations can also be exacerbated by the development of endocrine consequences after an acute stroke,including secondary adrenal insufficiency,syndrome of inappropriate antidiuretic hormone secretion and diabetes ***,COVID-19 infection has been reported to increase the risk of development of sodium and water perturbations that may further worsen the outcomes of patients who had an acute *** there are currently no accepted consensus guidelines on the management of sodium and water perturbations in patients who had an acute stroke,we conducted a systematic review of the literature published in English and in peer-reviewed journals between January 2000 and December 2020,according to PRIsMA guidelines,to assess on the current knowledge and clinical practices of this *** this review,we discuss the signs and symptoms of hyponatraemia and hypernatraemia,the pathogenesis of hyponatraemia and hypernatraemia,their clinical relevance,and we provide our recommendations for effective treatment strategies for the neurologist in the management of sodium and water perturbations in commonly encountered aetiologies of patients who had an acute stroke.

关键词： patients impaired consequences