<?xml version="1.0" encoding="ISO-8859-1"?><article xmlns:mml="http://www.w3.org/1998/Math/MathML" xmlns:xlink="http://www.w3.org/1999/xlink" xmlns:xsi="http://www.w3.org/2001/XMLSchema-instance">
<front>
<journal-meta>
<journal-id>0120-5633</journal-id>
<journal-title><![CDATA[Revista Colombiana de Cardiología]]></journal-title>
<abbrev-journal-title><![CDATA[Rev. Colomb. Cardiol.]]></abbrev-journal-title>
<issn>0120-5633</issn>
<publisher>
<publisher-name><![CDATA[Sociedad Colombiana de Cardiologia. Oficina de Publicaciones]]></publisher-name>
</publisher>
</journal-meta>
<article-meta>
<article-id>S0120-56332018000100067</article-id>
<article-id pub-id-type="doi">10.1016/j.rccar.2017.06.010</article-id>
<title-group>
<article-title xml:lang="es"><![CDATA[Marcapasos con sensor de asa cerrada en disautonomía secundaria a enfermedad de Sandhoff]]></article-title>
<article-title xml:lang="en"><![CDATA[Closed-loop pacemakers in dysautonomia secondary to Sandhoff disease]]></article-title>
</title-group>
<contrib-group>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Velásquez]]></surname>
<given-names><![CDATA[Jorge E.]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Gaviria]]></surname>
<given-names><![CDATA[María C.]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
<xref ref-type="aff" rid="Aaf"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Estupiñán]]></surname>
<given-names><![CDATA[Valentina L.]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Díaz-Martínez]]></surname>
<given-names><![CDATA[Juan C.]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Aristizábal]]></surname>
<given-names><![CDATA[Julián M.]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
<xref ref-type="aff" rid="Aaf"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Marín]]></surname>
<given-names><![CDATA[Jorge E.]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Uribe]]></surname>
<given-names><![CDATA[William]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Duque]]></surname>
<given-names><![CDATA[Mauricio]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
</contrib-group>
<aff id="Af1">
<institution><![CDATA[,Universidad CES Cardiología y Electrofisiología CES Cardiología]]></institution>
<addr-line><![CDATA[Medellín ]]></addr-line>
<country>Colombia</country>
</aff>
<aff id="Af2">
<institution><![CDATA[,Enlace Cardiología  ]]></institution>
<addr-line><![CDATA[Medellín ]]></addr-line>
<country>Colombia</country>
</aff>
<pub-date pub-type="pub">
<day>00</day>
<month>02</month>
<year>2018</year>
</pub-date>
<pub-date pub-type="epub">
<day>00</day>
<month>02</month>
<year>2018</year>
</pub-date>
<volume>25</volume>
<numero>1</numero>
<fpage>67</fpage>
<lpage>73</lpage>
<copyright-statement/>
<copyright-year/>
<self-uri xlink:href="http://www.scielo.org.co/scielo.php?script=sci_arttext&amp;pid=S0120-56332018000100067&amp;lng=en&amp;nrm=iso"></self-uri><self-uri xlink:href="http://www.scielo.org.co/scielo.php?script=sci_abstract&amp;pid=S0120-56332018000100067&amp;lng=en&amp;nrm=iso"></self-uri><self-uri xlink:href="http://www.scielo.org.co/scielo.php?script=sci_pdf&amp;pid=S0120-56332018000100067&amp;lng=en&amp;nrm=iso"></self-uri><abstract abstract-type="short" xml:lang="es"><p><![CDATA[Resumen  Introducción:  en la enfermedad de Sandhoff se ha descrito el compromiso del sistema nervioso autónomo en piel y tracto gastrointestinal; sin embargo, las alteraciones autonómicas del sistema cardiovascular, como la disautonomía, no se han documentado en la literatura revisada. De otro lado, el síncope neuralmente mediado de características cardioinhibitorias, refractario al manejo médico, puede ser tratado con marcapasos con sensor de asa cerrada.  Materiales y métodos:  se describe el caso de una paciente de 42 años en quien se realizó el diagnóstico de enfermedad de Sandhof. Asociado a esta patología, presentó disautonomía con episodios de síncope diarios sin respuesta al tratamiento médico, que ameritaron el implante de un marcapasos con sensor de asa cerrada. Posteriormente se revisa la literatura existente sobre el tema.  Conclusiones:  hasta la fecha no se había reportado relación entre la enfermedad de Sandhoff y la disautonomía; en el caso de esta paciente el deterioro de la calidad de vida fue significativo. El uso del algoritmo CLS en síncope neuralmente mediado con características cardioinhibitorias, ha mostrado disminuir la cantidad de síncopes en este tipo de pacientes y mejorar la calidad de vida de los mismos.]]></p></abstract>
<abstract abstract-type="short" xml:lang="en"><p><![CDATA[Abstract  Introduction:  Although involvement of the autonomic nervous system in the skin and gastrointestinal tract has been described in Sandhoff disease, no reports of autonomic changes of the cardiovascular system, such as dysautonomia, have been documented in the literature reviewed. On the other hand, the neurally mediated cardioinhibitory syncope that is refractory to medical management can be treated with pacemakers with closed loop sensors.  Materials and methods:  The case is described of a 42 year-old patient who was diagnosed with Sandhoff disease. Associated to this condition, he presented with dysautonomia with daily episodes of syncope, with no response to medical treatment, which warranted the implant of a pacemaker with a closed loop sensor. The existing literature on this topic was subsequently reviewed.  Conclusions:  Up until now, there have been no reports on the relationship between Sandhoff disease and dysautonomia. In the case of this patient, the deterioration in the quality of life was significant. The use of the closed loop stimulation (CLS) algorithm in neurally mediated cardioinhibitory syncope has been shown to reduce the number of syncopes in this type of patient and improve their quality of life.]]></p></abstract>
<kwd-group>
<kwd lng="es"><![CDATA[Marcapasos]]></kwd>
<kwd lng="es"><![CDATA[Síncope]]></kwd>
<kwd lng="es"><![CDATA[Sistema nervioso autónomo]]></kwd>
<kwd lng="en"><![CDATA[Pacemakers]]></kwd>
<kwd lng="en"><![CDATA[Syncope]]></kwd>
<kwd lng="en"><![CDATA[Autonomic nervous system]]></kwd>
</kwd-group>
</article-meta>
</front><back>
<ref-list>
<ref id="B1">
<label>1</label><nlm-citation citation-type="book">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Douglas]]></surname>
<given-names><![CDATA[P]]></given-names>
</name>
<name>
<surname><![CDATA[Zipes]]></surname>
<given-names><![CDATA[HGC]]></given-names>
</name>
</person-group>
<source><![CDATA[Hipotensión y síncope. En: Braunwald Tratado de cardiología]]></source>
<year></year>
<edition>10</edition>
<page-range>861-71</page-range><publisher-loc><![CDATA[España ]]></publisher-loc>
<publisher-name><![CDATA[Elsevier]]></publisher-name>
</nlm-citation>
</ref>
<ref id="B2">
<label>2</label><nlm-citation citation-type="journal">
<collab>Developed in collaboration with.European Heart Rhythm Association.Heart Failure Association.and Heart Rhythm Society.Endorsed by the following societies.European Society of Emergency Medicine</collab>
<article-title xml:lang=""><![CDATA[Guidelines for the diagnosis and management of syncope (versión 2009) The Task Force for the Diagnosis and Management of Syncope of the European Society of Cardiology (ESC)]]></article-title>
<source><![CDATA[Eur Heart J]]></source>
<year>2009</year>
<volume>30</volume>
<numero>21</numero>
<issue>21</issue>
<page-range>2631-71</page-range></nlm-citation>
</ref>
<ref id="B3">
<label>3</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Sun]]></surname>
<given-names><![CDATA[BC]]></given-names>
</name>
<name>
<surname><![CDATA[Emond]]></surname>
<given-names><![CDATA[JA]]></given-names>
</name>
<name>
<surname><![CDATA[Camargo]]></surname>
<given-names><![CDATA[CA]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Direct medical costs of syncope-related hospitalizations in the United States]]></article-title>
<source><![CDATA[Am J Cardiol]]></source>
<year>2005</year>
<volume>95</volume>
<page-range>668-71</page-range></nlm-citation>
</ref>
<ref id="B4">
<label>4</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Van Dijk]]></surname>
<given-names><![CDATA[N]]></given-names>
</name>
<name>
<surname><![CDATA[Sprangers]]></surname>
<given-names><![CDATA[MA]]></given-names>
</name>
<name>
<surname><![CDATA[Colman]]></surname>
<given-names><![CDATA[N]]></given-names>
</name>
<name>
<surname><![CDATA[Boer]]></surname>
<given-names><![CDATA[KR]]></given-names>
</name>
<name>
<surname><![CDATA[Wieling]]></surname>
<given-names><![CDATA[W]]></given-names>
</name>
<name>
<surname><![CDATA[Linzer]]></surname>
<given-names><![CDATA[M]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Clinical factors associated with quality of life in patients with transient loss of consciousness]]></article-title>
<source><![CDATA[J Cardiovasc Electrophysiol]]></source>
<year>2006</year>
<volume>17</volume>
<page-range>998-1003</page-range></nlm-citation>
</ref>
<ref id="B5">
<label>5</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Brignole]]></surname>
<given-names><![CDATA[M]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[A new management of syncope prospective systematic guideline-based evaluation of patients referred urgently to general hospitals]]></article-title>
<source><![CDATA[Eur Heart J]]></source>
<year>2005</year>
<volume>27</volume>
<page-range>76-82</page-range></nlm-citation>
</ref>
<ref id="B6">
<label>6</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Jhanjee]]></surname>
<given-names><![CDATA[R]]></given-names>
</name>
<name>
<surname><![CDATA[Can]]></surname>
<given-names><![CDATA[I]]></given-names>
</name>
<name>
<surname><![CDATA[Benditt]]></surname>
<given-names><![CDATA[DG]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Syncope]]></article-title>
<source><![CDATA[Dis Mon]]></source>
<year>2009</year>
<volume>55</volume>
<page-range>532-85</page-range></nlm-citation>
</ref>
<ref id="B7">
<label>7</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Duque]]></surname>
<given-names><![CDATA[M]]></given-names>
</name>
<name>
<surname><![CDATA[de la]]></surname>
<given-names><![CDATA[OL]]></given-names>
</name>
<name>
<surname><![CDATA[Duque]]></surname>
<given-names><![CDATA[M]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Tratamiento del síncope neuralmente mediado con marcapasos utilidad del sensor de asa cerrada]]></article-title>
<source><![CDATA[Rev Colomb Cardiol]]></source>
<year>2015</year>
<volume>22</volume>
<page-range>38-43</page-range></nlm-citation>
</ref>
<ref id="B8">
<label>8</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Brignole]]></surname>
<given-names><![CDATA[M]]></given-names>
</name>
<name>
<surname><![CDATA[Vardas]]></surname>
<given-names><![CDATA[P]]></given-names>
</name>
<name>
<surname><![CDATA[Hoffman]]></surname>
<given-names><![CDATA[E]]></given-names>
</name>
<name>
<surname><![CDATA[Huikuri]]></surname>
<given-names><![CDATA[H]]></given-names>
</name>
<name>
<surname><![CDATA[Moya]]></surname>
<given-names><![CDATA[A]]></given-names>
</name>
<name>
<surname><![CDATA[Ricci]]></surname>
<given-names><![CDATA[R]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Indications for the use of diagnostic implantable and external ECG loop recorders]]></article-title>
<collab>Task Force members</collab>
<source><![CDATA[Europace]]></source>
<year>2009</year>
<volume>11</volume>
<page-range>671-87</page-range></nlm-citation>
</ref>
<ref id="B9">
<label>9</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Izcovich]]></surname>
<given-names><![CDATA[A]]></given-names>
</name>
<name>
<surname><![CDATA[Gonzalez Malla]]></surname>
<given-names><![CDATA[C]]></given-names>
</name>
<name>
<surname><![CDATA[Manzotti]]></surname>
<given-names><![CDATA[M]]></given-names>
</name>
<name>
<surname><![CDATA[Catalano]]></surname>
<given-names><![CDATA[HN]]></given-names>
</name>
<name>
<surname><![CDATA[Guyatt]]></surname>
<given-names><![CDATA[G]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Midodrine for orthostatic hypotension and recurrent reflex syncope A systematic review]]></article-title>
<source><![CDATA[Neurology]]></source>
<year>2014</year>
<volume>83</volume>
<page-range>1170-7</page-range></nlm-citation>
</ref>
<ref id="B10">
<label>10</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Raviele]]></surname>
<given-names><![CDATA[A]]></given-names>
</name>
<name>
<surname><![CDATA[Giada]]></surname>
<given-names><![CDATA[F]]></given-names>
</name>
<name>
<surname><![CDATA[Menozzi]]></surname>
<given-names><![CDATA[C]]></given-names>
</name>
<name>
<surname><![CDATA[Speca]]></surname>
<given-names><![CDATA[G]]></given-names>
</name>
<name>
<surname><![CDATA[Orazi]]></surname>
<given-names><![CDATA[S]]></given-names>
</name>
<name>
<surname><![CDATA[Gasparini]]></surname>
<given-names><![CDATA[G]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[A randomized, double-blind, placebo-controlled study of permanent cardiac pacing for the treatment of recurrent tiltinduced vasovagal syncope The vasovagal syncope and pacing trial (SYNPACE)]]></article-title>
<source><![CDATA[Eur Heart J]]></source>
<year>2004</year>
<volume>25</volume>
<page-range>1741-8</page-range></nlm-citation>
</ref>
<ref id="B11">
<label>11</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Sutton]]></surname>
<given-names><![CDATA[R]]></given-names>
</name>
<name>
<surname><![CDATA[Ungar]]></surname>
<given-names><![CDATA[A]]></given-names>
</name>
<name>
<surname><![CDATA[Sgobino]]></surname>
<given-names><![CDATA[P]]></given-names>
</name>
<name>
<surname><![CDATA[Russo]]></surname>
<given-names><![CDATA[V]]></given-names>
</name>
<name>
<surname><![CDATA[Massa]]></surname>
<given-names><![CDATA[R]]></given-names>
</name>
<name>
<surname><![CDATA[Melissano]]></surname>
<given-names><![CDATA[D]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Cardiac pacing in patients with neurally mediated syncope and documented asystole effectiveness analysis from the Third International Study on Syncope of Uncertain Etiology (ISSUE-3) Registry]]></article-title>
<source><![CDATA[Europace]]></source>
<year>2014</year>
<volume>16</volume>
<page-range>595-9</page-range></nlm-citation>
</ref>
<ref id="B12">
<label>12</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Connolly]]></surname>
<given-names><![CDATA[SJ]]></given-names>
</name>
<name>
<surname><![CDATA[Sheldon]]></surname>
<given-names><![CDATA[R]]></given-names>
</name>
<name>
<surname><![CDATA[Thorpe]]></surname>
<given-names><![CDATA[KE]]></given-names>
</name>
<name>
<surname><![CDATA[Roberts]]></surname>
<given-names><![CDATA[RS]]></given-names>
</name>
<name>
<surname><![CDATA[Ellenbogen]]></surname>
<given-names><![CDATA[KA]]></given-names>
</name>
<name>
<surname><![CDATA[Wilkoff]]></surname>
<given-names><![CDATA[BL]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Pacemaker Therapy for Prevention of Syncope in Patients With Recurrent Severe Vasovagal Syncope Second Vasovagal Pacemaker Study (VPS II): A Randomized Trial]]></article-title>
<source><![CDATA[JAMA]]></source>
<year>2003</year>
<volume>289</volume>
<page-range>2224</page-range></nlm-citation>
</ref>
<ref id="B13">
<label>13</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Griesbach]]></surname>
<given-names><![CDATA[L]]></given-names>
</name>
<name>
<surname><![CDATA[Schweika]]></surname>
<given-names><![CDATA[O]]></given-names>
</name>
<name>
<surname><![CDATA[Huber]]></surname>
<given-names><![CDATA[T]]></given-names>
</name>
<name>
<surname><![CDATA[Knote]]></surname>
<given-names><![CDATA[B]]></given-names>
</name>
<name>
<surname><![CDATA[Härtel]]></surname>
<given-names><![CDATA[J]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Closed loop stimulation therapy for malignant neurocardiogenic syncope]]></article-title>
<source><![CDATA[Prog Biomed Res]]></source>
<year>2002</year>
<volume>7</volume>
<page-range>242-7</page-range></nlm-citation>
</ref>
<ref id="B14">
<label>14</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Occhetta]]></surname>
<given-names><![CDATA[E]]></given-names>
</name>
<name>
<surname><![CDATA[Bortnik]]></surname>
<given-names><![CDATA[M]]></given-names>
</name>
<name>
<surname><![CDATA[Audoglio]]></surname>
<given-names><![CDATA[R]]></given-names>
</name>
<name>
<surname><![CDATA[Vassanelli]]></surname>
<given-names><![CDATA[C]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Closed loop stimulation in prevention of vasovagal syncope Inotropy controlled pacing in vasovagal syncope (INVASY): a multicentre randomized, single blind, controlled study]]></article-title>
<source><![CDATA[Europace]]></source>
<year>2004</year>
<volume>6</volume>
<page-range>538-47</page-range></nlm-citation>
</ref>
<ref id="B15">
<label>15</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Russo]]></surname>
<given-names><![CDATA[V]]></given-names>
</name>
<name>
<surname><![CDATA[Rago]]></surname>
<given-names><![CDATA[A]]></given-names>
</name>
<name>
<surname><![CDATA[Papa]]></surname>
<given-names><![CDATA[AA]]></given-names>
</name>
<name>
<surname><![CDATA[Golino]]></surname>
<given-names><![CDATA[P]]></given-names>
</name>
<name>
<surname><![CDATA[Calabrò]]></surname>
<given-names><![CDATA[R]]></given-names>
</name>
<name>
<surname><![CDATA[Russo]]></surname>
<given-names><![CDATA[MG]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[The effect of dual- chamber closed-loop stimulation on syncope recurrence in healthy patients with tilt- induced vasovagal cardioinhibitory syncope a prospective, randomised, single blind, crossover study]]></article-title>
<source><![CDATA[Heart]]></source>
<year>2013</year>
<volume>99</volume>
<page-range>1609-13</page-range></nlm-citation>
</ref>
<ref id="B16">
<label>16</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Kanjwal]]></surname>
<given-names><![CDATA[K]]></given-names>
</name>
<name>
<surname><![CDATA[Karabin]]></surname>
<given-names><![CDATA[B]]></given-names>
</name>
<name>
<surname><![CDATA[Kanjwal]]></surname>
<given-names><![CDATA[Y]]></given-names>
</name>
<name>
<surname><![CDATA[Grubb]]></surname>
<given-names><![CDATA[BP]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Preliminary observationson the use of closed-loop cardiac pacing in patients with refractory neurocardiogenic syncope]]></article-title>
<source><![CDATA[J Interv Card Electrophysiol]]></source>
<year>2010</year>
<volume>27</volume>
<page-range>69-73</page-range></nlm-citation>
</ref>
<ref id="B17">
<label>17</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Palmisano]]></surname>
<given-names><![CDATA[P]]></given-names>
</name>
<name>
<surname><![CDATA[Zaccaria]]></surname>
<given-names><![CDATA[M]]></given-names>
</name>
<name>
<surname><![CDATA[Luzzi]]></surname>
<given-names><![CDATA[G]]></given-names>
</name>
<name>
<surname><![CDATA[Nacci]]></surname>
<given-names><![CDATA[F]]></given-names>
</name>
<name>
<surname><![CDATA[Anaclerio]]></surname>
<given-names><![CDATA[M]]></given-names>
</name>
<name>
<surname><![CDATA[Favale]]></surname>
<given-names><![CDATA[S]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Closed-loop cardiac pacing vs conventional dual-chamber pacing with specialized sensing and pacing algorithms for syncope prevention in patients with refractory vasovagal syncope: results of a long-term follow-up]]></article-title>
<source><![CDATA[Europace]]></source>
<year>2012</year>
<volume>14</volume>
<page-range>1038-43</page-range></nlm-citation>
</ref>
<ref id="B18">
<label>18</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Bortnik]]></surname>
<given-names><![CDATA[M]]></given-names>
</name>
<name>
<surname><![CDATA[Occhetta]]></surname>
<given-names><![CDATA[E]]></given-names>
</name>
<name>
<surname><![CDATA[Dell'Era]]></surname>
<given-names><![CDATA[G]]></given-names>
</name>
<name>
<surname><![CDATA[Secco]]></surname>
<given-names><![CDATA[GG]]></given-names>
</name>
<name>
<surname><![CDATA[Degiovanni]]></surname>
<given-names><![CDATA[A]]></given-names>
</name>
<name>
<surname><![CDATA[Plebani]]></surname>
<given-names><![CDATA[L]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Long- term follow-up of DDDR closed-loop cardiac pacing for the prevention of recurrent vasovagal syncope]]></article-title>
<source><![CDATA[J Cardiovasc Med]]></source>
<year>2012</year>
<volume>13</volume>
<page-range>242-5</page-range></nlm-citation>
</ref>
<ref id="B19">
<label>19</label><nlm-citation citation-type="">
<source><![CDATA[Benefit of Dual-chamber Pacing With Closed Loop Stimulation (CLS) in Tilt-induced Cardioinhibitory Reflex Syncope - Full Text View - ClinicalTrials.gov]]></source>
<year></year>
</nlm-citation>
</ref>
<ref id="B20">
<label>20</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Saouab]]></surname>
<given-names><![CDATA[R]]></given-names>
</name>
<name>
<surname><![CDATA[Mahi]]></surname>
<given-names><![CDATA[M]]></given-names>
</name>
<name>
<surname><![CDATA[Abilkacem]]></surname>
<given-names><![CDATA[R]]></given-names>
</name>
<name>
<surname><![CDATA[Boumdin]]></surname>
<given-names><![CDATA[H]]></given-names>
</name>
<name>
<surname><![CDATA[Chaouir]]></surname>
<given-names><![CDATA[S]]></given-names>
</name>
<name>
<surname><![CDATA[Agader]]></surname>
<given-names><![CDATA[O]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[A Case Report of Sandhoff Disease]]></article-title>
<source><![CDATA[Clin Neuroradiol]]></source>
<year>2011</year>
<volume>21</volume>
<page-range>83-5</page-range></nlm-citation>
</ref>
<ref id="B21">
<label>21</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Denny]]></surname>
<given-names><![CDATA[CA]]></given-names>
</name>
<name>
<surname><![CDATA[Heinecke]]></surname>
<given-names><![CDATA[KA]]></given-names>
</name>
<name>
<surname><![CDATA[Kim]]></surname>
<given-names><![CDATA[YP]]></given-names>
</name>
<name>
<surname><![CDATA[Baek]]></surname>
<given-names><![CDATA[RC]]></given-names>
</name>
<name>
<surname><![CDATA[Loh]]></surname>
<given-names><![CDATA[KS]]></given-names>
</name>
<name>
<surname><![CDATA[Butters]]></surname>
<given-names><![CDATA[TD]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Restricted ketogenic diet enhances the therapeutic action of N-butyldeoxynojirimycin towards brain GM2 accumulation in adult Sandhoff disease mice: Ketogenic diet, N B-DNJ, and Sandhoff disease]]></article-title>
<source><![CDATA[J Neurochem]]></source>
<year>2010</year>
</nlm-citation>
</ref>
<ref id="B22">
<label>22</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Krivit]]></surname>
<given-names><![CDATA[W]]></given-names>
</name>
<name>
<surname><![CDATA[Desnick]]></surname>
<given-names><![CDATA[RJ]]></given-names>
</name>
<name>
<surname><![CDATA[Lee]]></surname>
<given-names><![CDATA[J]]></given-names>
</name>
<name>
<surname><![CDATA[Moller]]></surname>
<given-names><![CDATA[J]]></given-names>
</name>
<name>
<surname><![CDATA[Wright]]></surname>
<given-names><![CDATA[F]]></given-names>
</name>
<name>
<surname><![CDATA[Sweeley]]></surname>
<given-names><![CDATA[CC]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Generalized accumulation of neutral glycosphingolipids with GM2 ganglioside accumulation in the brain Sandhoff's disease (variant of Tay-Sachs disease)]]></article-title>
<source><![CDATA[Am J Med]]></source>
<year>1972</year>
<volume>52</volume>
<page-range>763-70</page-range></nlm-citation>
</ref>
<ref id="B23">
<label>23</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Guertl]]></surname>
<given-names><![CDATA[B]]></given-names>
</name>
<name>
<surname><![CDATA[Noehammer]]></surname>
<given-names><![CDATA[C]]></given-names>
</name>
<name>
<surname><![CDATA[Hoefler]]></surname>
<given-names><![CDATA[G]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Metabolic cardiomyopathies]]></article-title>
<source><![CDATA[Int J Exp Pathol]]></source>
<year>2000</year>
<volume>81</volume>
<page-range>349-72</page-range></nlm-citation>
</ref>
<ref id="B24">
<label>24</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Modigliani]]></surname>
<given-names><![CDATA[R]]></given-names>
</name>
<name>
<surname><![CDATA[Lemann]]></surname>
<given-names><![CDATA[M]]></given-names>
</name>
<name>
<surname><![CDATA[Melancon]]></surname>
<given-names><![CDATA[SB]]></given-names>
</name>
<name>
<surname><![CDATA[Mikol]]></surname>
<given-names><![CDATA[J]]></given-names>
</name>
<name>
<surname><![CDATA[Potier]]></surname>
<given-names><![CDATA[M]]></given-names>
</name>
<name>
<surname><![CDATA[Salmeron]]></surname>
<given-names><![CDATA[M]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Diarrhea and autonomic dysfunction in a patient with hexosaminidase B deficiency (Sandhoff disease)]]></article-title>
<source><![CDATA[Gastroenterology]]></source>
<year>1994</year>
<volume>106</volume>
<page-range>775-81</page-range></nlm-citation>
</ref>
<ref id="B25">
<label>25</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Pellegrini]]></surname>
<given-names><![CDATA[M]]></given-names>
</name>
<name>
<surname><![CDATA[Zicari]]></surname>
<given-names><![CDATA[E]]></given-names>
</name>
<name>
<surname><![CDATA[Teresa Dotti]]></surname>
<given-names><![CDATA[M]]></given-names>
</name>
<name>
<surname><![CDATA[Federico]]></surname>
<given-names><![CDATA[A]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Dysautonomic achalasia in two siblings with Sandhoff disease]]></article-title>
<source><![CDATA[J Neurol Sci]]></source>
<year>2006</year>
<volume>241</volume>
<numero>1-2</numero>
<issue>1-2</issue>
<page-range>107-9</page-range></nlm-citation>
</ref>
<ref id="B26">
<label>26</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Salman]]></surname>
<given-names><![CDATA[MS]]></given-names>
</name>
<name>
<surname><![CDATA[Clarke]]></surname>
<given-names><![CDATA[JT]]></given-names>
</name>
<name>
<surname><![CDATA[Midroni]]></surname>
<given-names><![CDATA[G]]></given-names>
</name>
<name>
<surname><![CDATA[Waxman]]></surname>
<given-names><![CDATA[MB]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Peripheral and autonomic nervous system involvement in chronic GM2- gangliosidosis]]></article-title>
<source><![CDATA[J Inherit Metab Dis]]></source>
<year>2001</year>
<volume>24</volume>
<page-range>65-71</page-range></nlm-citation>
</ref>
<ref id="B27">
<label>27</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Hendriksz]]></surname>
<given-names><![CDATA[CJ]]></given-names>
</name>
<name>
<surname><![CDATA[Corry]]></surname>
<given-names><![CDATA[PC]]></given-names>
</name>
<name>
<surname><![CDATA[Wraith]]></surname>
<given-names><![CDATA[JE]]></given-names>
</name>
<name>
<surname><![CDATA[Besley]]></surname>
<given-names><![CDATA[GTN]]></given-names>
</name>
<name>
<surname><![CDATA[Cooper]]></surname>
<given-names><![CDATA[A]]></given-names>
</name>
<name>
<surname><![CDATA[Ferrie]]></surname>
<given-names><![CDATA[CD]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Juvenile Sandhoff disease-Nine new cases and a review of the literature]]></article-title>
<source><![CDATA[J Inherit Metab Dis]]></source>
<year>2004</year>
<volume>27</volume>
<page-range>241-9</page-range></nlm-citation>
</ref>
<ref id="B28">
<label>28</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Villamizar-Schiller]]></surname>
<given-names><![CDATA[IT]]></given-names>
</name>
<name>
<surname><![CDATA[Pabón]]></surname>
<given-names><![CDATA[LA]]></given-names>
</name>
<name>
<surname><![CDATA[Hufnagel]]></surname>
<given-names><![CDATA[SB]]></given-names>
</name>
<name>
<surname><![CDATA[Serrano]]></surname>
<given-names><![CDATA[NC]]></given-names>
</name>
<name>
<surname><![CDATA[Karl]]></surname>
<given-names><![CDATA[G]]></given-names>
</name>
<name>
<surname><![CDATA[Jefferies]]></surname>
<given-names><![CDATA[JL]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Neurological and cardiac responses after treatment with miglustat and a ketogenic diet in a patient with Sandhoff disease]]></article-title>
<source><![CDATA[Eur J Med Genet]]></source>
<year>2015</year>
<volume>58</volume>
<page-range>180-3</page-range></nlm-citation>
</ref>
<ref id="B29">
<label>29</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Zhang]]></surname>
<given-names><![CDATA[W]]></given-names>
</name>
<name>
<surname><![CDATA[Zeng]]></surname>
<given-names><![CDATA[H]]></given-names>
</name>
<name>
<surname><![CDATA[Huang]]></surname>
<given-names><![CDATA[Y]]></given-names>
</name>
<name>
<surname><![CDATA[Xie]]></surname>
<given-names><![CDATA[T]]></given-names>
</name>
<name>
<surname><![CDATA[Zheng]]></surname>
<given-names><![CDATA[J]]></given-names>
</name>
<name>
<surname><![CDATA[Zhao]]></surname>
<given-names><![CDATA[X]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Clinical, biochemical and molecular analysis of five Chinese patients with Sandhoff disease]]></article-title>
<source><![CDATA[Metab Brain Dis]]></source>
<year>2016</year>
<volume>31</volume>
<page-range>861-7</page-range></nlm-citation>
</ref>
<ref id="B30">
<label>30</label><nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Bradbury]]></surname>
<given-names><![CDATA[AM]]></given-names>
</name>
<name>
<surname><![CDATA[Peterson]]></surname>
<given-names><![CDATA[TA]]></given-names>
</name>
<name>
<surname><![CDATA[Gross]]></surname>
<given-names><![CDATA[AL]]></given-names>
</name>
<name>
<surname><![CDATA[Wells]]></surname>
<given-names><![CDATA[SZ]]></given-names>
</name>
<name>
<surname><![CDATA[McCurdy]]></surname>
<given-names><![CDATA[VJ]]></given-names>
</name>
<name>
<surname><![CDATA[Wolfe]]></surname>
<given-names><![CDATA[KG]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[AAV-mediated gene delivery attenuates neuroinflammation in feline Sandhoff disease]]></article-title>
<source><![CDATA[Neuroscience]]></source>
<year>2017</year>
<volume>340</volume>
<page-range>117-25</page-range></nlm-citation>
</ref>
</ref-list>
</back>
</article>
