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BAFF controls neural cell survival through BAFF receptor
Abstracts / Journal of the Neurological Sciences 333 (2013) e679–e727
parasympathetic modulation as RRI-RMSSDs and RRI-high-frequencypowers (HF), and baroreflex sensitivity (BRS). We calculated Valsalva-ratios (VR) and – as indices of sympathetic withdrawal – intervals from the highest BP-value during phase-IVovershoot to the time when BP had decreased by 60% (60%-BPrecovery-time) and 90% (90%-BP-recovery-time) of the differences between phase-IV-BP-maxima and baseline-BP. We compared patientand control-parameters before and during VMs (ANOVA, post-hoc analysis; significance: p b 0.05). Results: At baseline, RRI-CVs, RRI-SDs, RRI-total-powers, RRI-LFpowers, RRI-RMSSDs, RRI-HF-powers and BRS were lower in patients than controls. During VMs, RRIs, BPs, and VRs were similar in both groups, while 60%- and 90%-BP-recovery-times were significantly longer in patients than controls. Conclusions: Baseline autonomic modulation was reduced in mTBIpatients. During VM-challenge, only prolonged 60%- and 90%-BPrecovery-times unveiled altered autonomic adjustment in mTBI-patients with prolonged sympathetic hyperactivity. Sympathetic hyperactivity upon challenge may contribute to increased cardiovascular risk years after mTBI. Acknowledgement: The study was partially funded by the IBRF, Flanders, NJ, USA. doi:10.1016/j.jns.2013.07.2378
Abstract — WCN 2013 No: 2673 Topic: 36 — Other Topic Genetic modifications present in choroid plexus neoplasias and associated disturbances of psychoneural development E. Castro-Sierraa, F. Chico-Ponce de Leónb, P. Dhellemmesc, F. ArenasHuerterod, P. Eguía-Aguilard, M. Perezpeña-Diazcontid, A. SánchezBoisoe, S. Martínez-Rodríguezf. aLaboratory of Psychoacoustics, Hospital Infantil de Mexico Federico Gomez, Mexico City, Mexico; bDepartment of Neurosugery, Hospital Infantil de Mexico Federico Gomez, Mexico City, Mexico; cCentre Hospitalier Universitaire, Lille, France; dDepartment of Pathology, Hospital Infantil de Mexico Federico Gomez, Mexico City, Mexico; eDepartment of Genetics, Hospital Infantil de Mexico Federico Gomez, Mexico City, Mexico; fDepartment of Social Work, Hospital Infantil de Mexico Federico Gomez, Mexico City, Mexico Choroid plexus papillomas or carcinomas are rare forms of brain neoplasias. Some years ago, Steichen-Gersdorf et al. found a choroid plexus papilloma associated to a case of hypomelanosis of Ito and a 17p: Xq translocation in this female patient (Hum Genet 1993; 90:611–613). Recently, a 10-month-old girl with a Brachmann–Cornelia de Lange syndrome (BCLS) and a choroid plexus papilloma of the third ventricle of the brain was operated upon and studied at Hospital Infantil de México Federico Gómez (HIMFG) in Mexico City. Karyotypes (GTG bands) of the proband and her parents undertaken at HIMFG were normal. Array CGH analyses of blood DNA of these 3 persons carried out at BlueGnome's Laboratory in Cambridge, U.K., set in evidence amplification of genes SPNS2, GGT6, SMTNL2, PELP1, MYBBP1A and ALOX15 in chromosome 17p of the proband. Since MYBBP1A is a proto-oncogene and ALOX15 participates in development of cancer and metastases of tumors, further FISH analyses of these 2 genes were implemented at HIMFG. Amplification of both genes was also found in the tumor of the case under study but not in an unrelated papilloma of the choroid plexus. The possible genetic counseling, diagnostic and prognostic importance of the findings, especially regarding genetic modifications of chromosome 17p in choroid plexus tumors and associated clinical disturbances of psychoneural development of the type of hypomelanosis of Ito or BCLS, is discussed. doi:10.1016/j.jns.2013.07.2379
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Abstract — WCN 2013 No: 1160 Topic: 36 — Other Topic BAFF controls neural cell survival through BAFF receptor S. Tadaa,b, T. Yasuib, T. Okunoa, Y. Nakatsujia, H. Mochizukia, S. Sakodac, H. Kikutanib. aDepartment of Neurology, Osaka University Graduate School of Medicine, Japan; bDepartment of Molecular Immunology, Research Institute for Microbial Disease, WPI Immunology Frontier Research Center (IFReC), Osaka University, Japan; cDepartment of Neurology, National Toneyama Hospital, Osaka, Japan Background: B cell activating factor (BAFF) is a fundamental survival factor for B lymphocytes and its effect on B cells has been extensively investigated. However, its effect on the central nervous system (CNS) has not been clarified. Objective: To explore the novel mechanism of neuronal survival by BAFF receptor (BAFF-R) signals in CNS. Material and methods: We examined BAFF and BAFF-R expression in neurons and cultured embryonic neurons of either wild-type or A/ WySnJ mice (Baffrm/m), which carry a mutation in BAFF-R, to compare the survival of neurons of both groups in vitro. We also bred mice carrying the human Superoxide Dismutase-1 mutation G93A (mSOD1), animal models of familial amyotrophic lateral sclerosis (ALS) with Baffrm/m, and body weight and survival were assessed. Results: Immunohistochemical analysis and RT-PCR study revealed that both BAFF and BAFF-R were expressed in mouse neurons. Primary cultured neurons of Baffrm/m showed decreased survival compared with those of wild-type mice (Baffr+/+). Furthermore, mSOD1/Baffrm/m displayed reduced survival (141.56 days ± 2.25) vs control mice (mSOD1/Baffr+/+) (152.3 days ± 1.28); p b 0.001. mSOD1/Baffr+/+ at age 18 weeks displayed reduced body weight (90.80% ± 0.83 [n = 20]), compared with mSOD1/Baffr+/+ (97.18% ± 0.61 [n = 19]); p b 0.001. Comparable numbers of microglia/macrophages and astrocytes were observed at early- and middle-stage disease in spinal cord section of both groups. Conclusion: Our findings suggest a novel role of BAFF as a critical member of neuroprotective factors that directs neuronal survival independent of the action for glial cells. doi:10.1016/j.jns.2013.07.2380
Abstract — WCN 2013 No: 2656 Topic: 36 — Other Topic Severe cardiac dysautonomia in acute myelitis R. Rennaa, D. Plantonea, P. Proficea, F. Pilatoa, V. Di Lazzarob. a Dipartimento di Geriatria, Neuroscienze ed Ortopedia, Catholic University of Sacred Heart, Italy; bInstitute of Neurology, Campus Bio-Medico University, Rome, Italy Background: Traumatic spinal cord injury is frequently complicated by cardiocirculatory impairment (bradycardia and hypotension). It is unknown whether the same phenomenon can manifest in acute myelitis. Design and methods: Case report Results: A 35-year-old woman presented with left hemisoma weakness and hypoesthesia. Neurological examination revealed left arm weakness and dysmetria, left lateropulsion at Romberg test and hypoesthesia in the left hemisoma with a C2 upper level. Laboratory tests and CSF analysis were unremarkable. Brain MRI was negative. Cervical spine MRI showed a T2-hyperintense C2 intramedullary lesion. An acute myelitis was diagnosed and the patient started on a course of methylprednisolone, with initial benefit. Five days after
parasympathetic modulation as RRI-RMSSDs and RRI-high-frequencypowers (HF), and baroreflex sensitivity (BRS). We calculated Valsalva-ratios (VR) and – as indices of sympathetic withdrawal – intervals from the highest BP-value during phase-IVovershoot to the time when BP had decreased by 60% (60%-BPrecovery-time) and 90% (90%-BP-recovery-time) of the differences between phase-IV-BP-maxima and baseline-BP. We compared patientand control-parameters before and during VMs (ANOVA, post-hoc analysis; significance: p b 0.05). Results: At baseline, RRI-CVs, RRI-SDs, RRI-total-powers, RRI-LFpowers, RRI-RMSSDs, RRI-HF-powers and BRS were lower in patients than controls. During VMs, RRIs, BPs, and VRs were similar in both groups, while 60%- and 90%-BP-recovery-times were significantly longer in patients than controls. Conclusions: Baseline autonomic modulation was reduced in mTBIpatients. During VM-challenge, only prolonged 60%- and 90%-BPrecovery-times unveiled altered autonomic adjustment in mTBI-patients with prolonged sympathetic hyperactivity. Sympathetic hyperactivity upon challenge may contribute to increased cardiovascular risk years after mTBI. Acknowledgement: The study was partially funded by the IBRF, Flanders, NJ, USA. doi:10.1016/j.jns.2013.07.2378
Abstract — WCN 2013 No: 2673 Topic: 36 — Other Topic Genetic modifications present in choroid plexus neoplasias and associated disturbances of psychoneural development E. Castro-Sierraa, F. Chico-Ponce de Leónb, P. Dhellemmesc, F. ArenasHuerterod, P. Eguía-Aguilard, M. Perezpeña-Diazcontid, A. SánchezBoisoe, S. Martínez-Rodríguezf. aLaboratory of Psychoacoustics, Hospital Infantil de Mexico Federico Gomez, Mexico City, Mexico; bDepartment of Neurosugery, Hospital Infantil de Mexico Federico Gomez, Mexico City, Mexico; cCentre Hospitalier Universitaire, Lille, France; dDepartment of Pathology, Hospital Infantil de Mexico Federico Gomez, Mexico City, Mexico; eDepartment of Genetics, Hospital Infantil de Mexico Federico Gomez, Mexico City, Mexico; fDepartment of Social Work, Hospital Infantil de Mexico Federico Gomez, Mexico City, Mexico Choroid plexus papillomas or carcinomas are rare forms of brain neoplasias. Some years ago, Steichen-Gersdorf et al. found a choroid plexus papilloma associated to a case of hypomelanosis of Ito and a 17p: Xq translocation in this female patient (Hum Genet 1993; 90:611–613). Recently, a 10-month-old girl with a Brachmann–Cornelia de Lange syndrome (BCLS) and a choroid plexus papilloma of the third ventricle of the brain was operated upon and studied at Hospital Infantil de México Federico Gómez (HIMFG) in Mexico City. Karyotypes (GTG bands) of the proband and her parents undertaken at HIMFG were normal. Array CGH analyses of blood DNA of these 3 persons carried out at BlueGnome's Laboratory in Cambridge, U.K., set in evidence amplification of genes SPNS2, GGT6, SMTNL2, PELP1, MYBBP1A and ALOX15 in chromosome 17p of the proband. Since MYBBP1A is a proto-oncogene and ALOX15 participates in development of cancer and metastases of tumors, further FISH analyses of these 2 genes were implemented at HIMFG. Amplification of both genes was also found in the tumor of the case under study but not in an unrelated papilloma of the choroid plexus. The possible genetic counseling, diagnostic and prognostic importance of the findings, especially regarding genetic modifications of chromosome 17p in choroid plexus tumors and associated clinical disturbances of psychoneural development of the type of hypomelanosis of Ito or BCLS, is discussed. doi:10.1016/j.jns.2013.07.2379
e689
Abstract — WCN 2013 No: 1160 Topic: 36 — Other Topic BAFF controls neural cell survival through BAFF receptor S. Tadaa,b, T. Yasuib, T. Okunoa, Y. Nakatsujia, H. Mochizukia, S. Sakodac, H. Kikutanib. aDepartment of Neurology, Osaka University Graduate School of Medicine, Japan; bDepartment of Molecular Immunology, Research Institute for Microbial Disease, WPI Immunology Frontier Research Center (IFReC), Osaka University, Japan; cDepartment of Neurology, National Toneyama Hospital, Osaka, Japan Background: B cell activating factor (BAFF) is a fundamental survival factor for B lymphocytes and its effect on B cells has been extensively investigated. However, its effect on the central nervous system (CNS) has not been clarified. Objective: To explore the novel mechanism of neuronal survival by BAFF receptor (BAFF-R) signals in CNS. Material and methods: We examined BAFF and BAFF-R expression in neurons and cultured embryonic neurons of either wild-type or A/ WySnJ mice (Baffrm/m), which carry a mutation in BAFF-R, to compare the survival of neurons of both groups in vitro. We also bred mice carrying the human Superoxide Dismutase-1 mutation G93A (mSOD1), animal models of familial amyotrophic lateral sclerosis (ALS) with Baffrm/m, and body weight and survival were assessed. Results: Immunohistochemical analysis and RT-PCR study revealed that both BAFF and BAFF-R were expressed in mouse neurons. Primary cultured neurons of Baffrm/m showed decreased survival compared with those of wild-type mice (Baffr+/+). Furthermore, mSOD1/Baffrm/m displayed reduced survival (141.56 days ± 2.25) vs control mice (mSOD1/Baffr+/+) (152.3 days ± 1.28); p b 0.001. mSOD1/Baffr+/+ at age 18 weeks displayed reduced body weight (90.80% ± 0.83 [n = 20]), compared with mSOD1/Baffr+/+ (97.18% ± 0.61 [n = 19]); p b 0.001. Comparable numbers of microglia/macrophages and astrocytes were observed at early- and middle-stage disease in spinal cord section of both groups. Conclusion: Our findings suggest a novel role of BAFF as a critical member of neuroprotective factors that directs neuronal survival independent of the action for glial cells. doi:10.1016/j.jns.2013.07.2380
Abstract — WCN 2013 No: 2656 Topic: 36 — Other Topic Severe cardiac dysautonomia in acute myelitis R. Rennaa, D. Plantonea, P. Proficea, F. Pilatoa, V. Di Lazzarob. a Dipartimento di Geriatria, Neuroscienze ed Ortopedia, Catholic University of Sacred Heart, Italy; bInstitute of Neurology, Campus Bio-Medico University, Rome, Italy Background: Traumatic spinal cord injury is frequently complicated by cardiocirculatory impairment (bradycardia and hypotension). It is unknown whether the same phenomenon can manifest in acute myelitis. Design and methods: Case report Results: A 35-year-old woman presented with left hemisoma weakness and hypoesthesia. Neurological examination revealed left arm weakness and dysmetria, left lateropulsion at Romberg test and hypoesthesia in the left hemisoma with a C2 upper level. Laboratory tests and CSF analysis were unremarkable. Brain MRI was negative. Cervical spine MRI showed a T2-hyperintense C2 intramedullary lesion. An acute myelitis was diagnosed and the patient started on a course of methylprednisolone, with initial benefit. Five days after