<?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>2413-4341</journal-id>
<journal-title><![CDATA[Revista Paraguaya de Reumatología]]></journal-title>
<abbrev-journal-title><![CDATA[Rev. parag. reumatol.]]></abbrev-journal-title>
<issn>2413-4341</issn>
<publisher>
<publisher-name><![CDATA[Sociedad Paraguaya de Reumatología]]></publisher-name>
</publisher>
</journal-meta>
<article-meta>
<article-id>S2413-43412025000200083</article-id>
<article-id pub-id-type="doi">10.18004/rpr/2025.11.02.83</article-id>
<title-group>
<article-title xml:lang="es"><![CDATA[Síndrome de Susac. Desafíos diagnósticos y terapéuticos]]></article-title>
<article-title xml:lang="en"><![CDATA[Susac Syndrome. Diagnostic and therapeutic challenges]]></article-title>
</title-group>
<contrib-group>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Ferreira-Maidana]]></surname>
<given-names><![CDATA[Santiago]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Báez]]></surname>
<given-names><![CDATA[Luis]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Delgado-Herrera]]></surname>
<given-names><![CDATA[Leticia]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
<contrib contrib-type="author">
<name>
<surname><![CDATA[Colman]]></surname>
<given-names><![CDATA[Nelly]]></given-names>
</name>
<xref ref-type="aff" rid="Aff"/>
</contrib>
</contrib-group>
<aff id="Af1">
<institution><![CDATA[,Universidad Nacional de Asunción Facultad de Ciencias Médicas Hospital de Clínicas, Departamento de Reumatología]]></institution>
<addr-line><![CDATA[San Lorenzo ]]></addr-line>
<country>Paraguay</country>
</aff>
<aff id="Af2">
<institution><![CDATA[,Universidad Nacional de Asunción Facultad de Ciencias Médicas Hospital de Clínicas, Departamento de Neurología]]></institution>
<addr-line><![CDATA[San Lorenzo ]]></addr-line>
<country>Paraguay</country>
</aff>
<aff id="Af3">
<institution><![CDATA[,Fundación Visión  ]]></institution>
<addr-line><![CDATA[Fernando de la Mora ]]></addr-line>
<country>Paraguay</country>
</aff>
<pub-date pub-type="pub">
<day>00</day>
<month>12</month>
<year>2025</year>
</pub-date>
<pub-date pub-type="epub">
<day>00</day>
<month>12</month>
<year>2025</year>
</pub-date>
<volume>11</volume>
<numero>2</numero>
<fpage>83</fpage>
<lpage>86</lpage>
<copyright-statement/>
<copyright-year/>
<self-uri xlink:href="http://scielo.iics.una.py/scielo.php?script=sci_arttext&amp;pid=S2413-43412025000200083&amp;lng=en&amp;nrm=iso"></self-uri><self-uri xlink:href="http://scielo.iics.una.py/scielo.php?script=sci_abstract&amp;pid=S2413-43412025000200083&amp;lng=en&amp;nrm=iso"></self-uri><self-uri xlink:href="http://scielo.iics.una.py/scielo.php?script=sci_pdf&amp;pid=S2413-43412025000200083&amp;lng=en&amp;nrm=iso"></self-uri><abstract abstract-type="short" xml:lang="es"><p><![CDATA[RESUMEN  Introducción: El Síndrome de Susac (SS) es una rara microangiopatía autoinmune que se manifiesta con encefalopatía, vasculitis ocular y afectación auditiva. Su detección temprana es clave para evitar secuelas irreversibles.  Caso clínico:  Mujer de 28 años, sin antecedentes patológicos, acude por cuadro clínico de 3 semanas de evolución, de acúfenos asociados a hipoacusia bilateral, cefalea pulsátil, náuseas, vómitos y fotofobia, al que se agrega 10 días antes, disminución de la agudeza visual (AV). La evaluación oftalmológica reveló vasculitis oclusiva 360°, edema macular, hemorragias peripapilares y papiledema. Audiometría normal con acúfenos grado V. El líquido cefalorraquídeo y los estudios inmunológicos/serológicos fueron normales. La resonancia magnética encefálica y medular contrastada no mostró alteraciones. Se inició tratamiento con metilprednisolona intravenosa y ciclofosfamida IV. Posteriormente, mantenimiento con azatioprina, presenta recaída del cuadro ocular y auditivo, por lo que recibe nuevamente corticoides, rituximab y micofenolato, con buena respuesta.  Conclusión: El síndrome de Susac debe incluirse dentro del diagnóstico diferencial en mujeres jóvenes que presenten síntomas neurológicos, visuales y auditivos inusuales. La identificación temprana y el inicio oportuno de un tratamiento inmunosupresor intensivo son fundamentales para optimizar el pronóstico y prevenir secuelas permanentes.]]></p></abstract>
<abstract abstract-type="short" xml:lang="en"><p><![CDATA[ABSTRACT  Introduction: Susac syndrome (SS) is a rare autoimmune microangiopathy that manifests with encephalopathy, ocular vasculitis, and hearing impairment. Early detection is key to preventing irreversible sequelae.  Case report: A 28-year-old woman with no prior medical history presented with a 3-week history of tinnitus associated with bilateral hearing loss, throbbing headache, nausea, vomiting, and photophobia. Ten days prior, she also experienced decreased visual acuity (VA). Ophthalmological examination revealed 360° occlusive vasculitis, macular edema, peripapillary hemorrhages, and papilledema. Audiometry was normal, with tinnitus classified as grade V. Cerebrospinal fluid analysis and immunological/serological studies were normal. Brain and spinal cord MRI with contrast showed no abnormalities. She received intravenous methylprednisolone and intravenous cyclophosphamide. Subsequently, while on maintenance therapy with azathioprine, the patient experienced a relapse of the ocular and auditory symptoms. She showed improvement with corticosteroids, rituximab, and mycophenolate.  Conclusion: Susac syndrome should be included in the differential diagnosis of young women presenting with unusual neurological, visual, and auditory symptoms. Early identification and timely initiation of intensive immunosuppressive therapy are essential to optimize the prognosis and prevent permanent sequelae.]]></p></abstract>
<kwd-group>
<kwd lng="es"><![CDATA[síndrome de Susac]]></kwd>
<kwd lng="es"><![CDATA[vasculitis retiniana]]></kwd>
<kwd lng="es"><![CDATA[hipoacusia]]></kwd>
<kwd lng="es"><![CDATA[encefalopatía autoinmune]]></kwd>
<kwd lng="en"><![CDATA[Susac syndrome]]></kwd>
<kwd lng="en"><![CDATA[retinal vasculitis]]></kwd>
<kwd lng="en"><![CDATA[hearing loss]]></kwd>
<kwd lng="en"><![CDATA[autoimmune encephalopathy]]></kwd>
</kwd-group>
</article-meta>
</front><back>
<ref-list>
<ref id="B1">
<nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Rennebohm]]></surname>
<given-names><![CDATA[RM]]></given-names>
</name>
<name>
<surname><![CDATA[Asdaghi]]></surname>
<given-names><![CDATA[N]]></given-names>
</name>
<name>
<surname><![CDATA[Srivastava]]></surname>
<given-names><![CDATA[S]]></given-names>
</name>
<name>
<surname><![CDATA[Gertner]]></surname>
<given-names><![CDATA[E]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Guidelines for treatment of Susac syndrome - An update]]></article-title>
<source><![CDATA[International Journal of Stroke]]></source>
<year>2020</year>
<volume>15</volume>
<page-range>484-94</page-range><publisher-name><![CDATA[SAGE Publications Inc.]]></publisher-name>
</nlm-citation>
</ref>
<ref id="B2">
<nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Wilf-Yarkoni]]></surname>
<given-names><![CDATA[A]]></given-names>
</name>
<name>
<surname><![CDATA[Zmira]]></surname>
<given-names><![CDATA[O]]></given-names>
</name>
<name>
<surname><![CDATA[Tolkovsky]]></surname>
<given-names><![CDATA[A]]></given-names>
</name>
<name>
<surname><![CDATA[Pflantzer]]></surname>
<given-names><![CDATA[B]]></given-names>
</name>
<name>
<surname><![CDATA[Gofrit]]></surname>
<given-names><![CDATA[SG]]></given-names>
</name>
<name>
<surname><![CDATA[Kleffner]]></surname>
<given-names><![CDATA[I]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Clinical Characterization and Ancillary Tests in Susac Syndrome: A Systematic Review]]></article-title>
<source><![CDATA[Neurology(R) neuroimmunology &amp; neuroinflammation]]></source>
<year>2024</year>
<volume>11</volume>
</nlm-citation>
</ref>
<ref id="B3">
<nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Pace]]></surname>
<given-names><![CDATA[GM]]></given-names>
</name>
<name>
<surname><![CDATA[Canali]]></surname>
<given-names><![CDATA[L]]></given-names>
</name>
<name>
<surname><![CDATA[Villari]]></surname>
<given-names><![CDATA[D]]></given-names>
</name>
<name>
<surname><![CDATA[Spriano]]></surname>
<given-names><![CDATA[G]]></given-names>
</name>
<name>
<surname><![CDATA[Mercante]]></surname>
<given-names><![CDATA[G]]></given-names>
</name>
<name>
<surname><![CDATA[Mosnier]]></surname>
<given-names><![CDATA[I]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Síndrome de Susac: una revisión sistemática y análisis de supervivencia]]></article-title>
<source><![CDATA[Clin Rev Allergy Immunol]]></source>
<year>2025</year>
<volume>68</volume>
<page-range>73</page-range></nlm-citation>
</ref>
<ref id="B4">
<nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Susac]]></surname>
<given-names><![CDATA[JO]]></given-names>
</name>
<name>
<surname><![CDATA[Hardman]]></surname>
<given-names><![CDATA[JM]]></given-names>
</name>
<name>
<surname><![CDATA[Selhorst]]></surname>
<given-names><![CDATA[JB]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Microangiopathy of the brain and retina with hearing loss]]></article-title>
<source><![CDATA[Neurology]]></source>
<year>1979</year>
<volume>29</volume>
<numero>3</numero>
<issue>3</issue>
<page-range>313-6</page-range></nlm-citation>
</ref>
<ref id="B5">
<nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[David]]></surname>
<given-names><![CDATA[C]]></given-names>
</name>
<name>
<surname><![CDATA[Sacré]]></surname>
<given-names><![CDATA[K]]></given-names>
</name>
<name>
<surname><![CDATA[Henri-Feugeas]]></surname>
<given-names><![CDATA[MC]]></given-names>
</name>
<name>
<surname><![CDATA[Klein]]></surname>
<given-names><![CDATA[I]]></given-names>
</name>
<name>
<surname><![CDATA[Doan]]></surname>
<given-names><![CDATA[S]]></given-names>
</name>
<name>
<surname><![CDATA[Cohen]]></surname>
<given-names><![CDATA[FA]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Susac syndrome: A scoping review]]></article-title>
<source><![CDATA[Autoimmun Rev]]></source>
<year>2022</year>
<volume>21</volume>
<numero>6</numero>
<issue>6</issue>
<page-range>103097</page-range></nlm-citation>
</ref>
<ref id="B6">
<nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Bose]]></surname>
<given-names><![CDATA[S]]></given-names>
</name>
<name>
<surname><![CDATA[Papathanasiou]]></surname>
<given-names><![CDATA[A]]></given-names>
</name>
<name>
<surname><![CDATA[Karkhanis]]></surname>
<given-names><![CDATA[S]]></given-names>
</name>
<name>
<surname><![CDATA[Appleton]]></surname>
<given-names><![CDATA[JP]]></given-names>
</name>
<name>
<surname><![CDATA[King]]></surname>
<given-names><![CDATA[D]]></given-names>
</name>
<name>
<surname><![CDATA[Batra]]></surname>
<given-names><![CDATA[R]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Susac syndrome: neurological update (clinical features, long-term observational follow-up and management of sixteen patients)]]></article-title>
<source><![CDATA[J Neurol]]></source>
<year>2023</year>
<volume>270</volume>
<numero>12</numero>
<issue>12</issue>
<page-range>6193-206</page-range></nlm-citation>
</ref>
<ref id="B7">
<nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Wilf-Yarkoni]]></surname>
<given-names><![CDATA[A]]></given-names>
</name>
<name>
<surname><![CDATA[Zmira]]></surname>
<given-names><![CDATA[O]]></given-names>
</name>
<name>
<surname><![CDATA[Tolkovsky]]></surname>
<given-names><![CDATA[A]]></given-names>
</name>
<name>
<surname><![CDATA[Pflantzer]]></surname>
<given-names><![CDATA[B]]></given-names>
</name>
<name>
<surname><![CDATA[Gofrit]]></surname>
<given-names><![CDATA[SG]]></given-names>
</name>
<name>
<surname><![CDATA[Kleffner]]></surname>
<given-names><![CDATA[I]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Clinical Characterization and Ancillary Tests in Susac Syndrome A Systematic Review]]></article-title>
<source><![CDATA[Neurology: Neuroimmunology and NeuroInflammation]]></source>
<year>2024</year>
<volume>11</volume>
<publisher-name><![CDATA[Lippincott Williams and Wilkins]]></publisher-name>
</nlm-citation>
</ref>
<ref id="B8">
<nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Marrodan]]></surname>
<given-names><![CDATA[M]]></given-names>
</name>
<name>
<surname><![CDATA[Fiol]]></surname>
<given-names><![CDATA[MP]]></given-names>
</name>
<name>
<surname><![CDATA[Correale]]></surname>
<given-names><![CDATA[J]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Susac syndrome: Challenges in the diagnosis and treatment]]></article-title>
<source><![CDATA[Brain]]></source>
<year>2022</year>
<volume>145</volume>
<page-range>858-71</page-range><publisher-name><![CDATA[Oxford University Press]]></publisher-name>
</nlm-citation>
</ref>
<ref id="B9">
<nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Kleffner]]></surname>
<given-names><![CDATA[I]]></given-names>
</name>
<name>
<surname><![CDATA[Dörr]]></surname>
<given-names><![CDATA[J]]></given-names>
</name>
<name>
<surname><![CDATA[Ringelstein]]></surname>
<given-names><![CDATA[M]]></given-names>
</name>
<name>
<surname><![CDATA[Gross]]></surname>
<given-names><![CDATA[CC]]></given-names>
</name>
<name>
<surname><![CDATA[Böckenfeld]]></surname>
<given-names><![CDATA[Y]]></given-names>
</name>
<name>
<surname><![CDATA[Schwindt]]></surname>
<given-names><![CDATA[W]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Diagnostic criteria for Susac syndrome]]></article-title>
<source><![CDATA[J Neurol Neurosurg Psychiatry]]></source>
<year>2016</year>
<volume>87</volume>
<numero>12</numero>
<issue>12</issue>
<page-range>1287-95</page-range></nlm-citation>
</ref>
<ref id="B10">
<nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Fuchs]]></surname>
<given-names><![CDATA[L]]></given-names>
</name>
<name>
<surname><![CDATA[Wilf-Yarkoni]]></surname>
<given-names><![CDATA[A]]></given-names>
</name>
<name>
<surname><![CDATA[Kolb]]></surname>
<given-names><![CDATA[H]]></given-names>
</name>
<name>
<surname><![CDATA[Vigiser]]></surname>
<given-names><![CDATA[I]]></given-names>
</name>
<name>
<surname><![CDATA[Regev]]></surname>
<given-names><![CDATA[K]]></given-names>
</name>
<name>
<surname><![CDATA[Zur]]></surname>
<given-names><![CDATA[D]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Clinical Characterization and Prognostic Risk Factors of Susac Syndrome A Retrospective Multicenter Study]]></article-title>
<source><![CDATA[Neurol Neuroimmunol Neuroinflamm]]></source>
<year>2024</year>
<volume>12</volume>
<numero>2</numero>
<issue>2</issue>
</nlm-citation>
</ref>
<ref id="B11">
<nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Portela]]></surname>
<given-names><![CDATA[M]]></given-names>
</name>
<name>
<surname><![CDATA[Nogueira]]></surname>
<given-names><![CDATA[V]]></given-names>
</name>
<name>
<surname><![CDATA[Liverani]]></surname>
<given-names><![CDATA[M]]></given-names>
</name>
<name>
<surname><![CDATA[Figueira]]></surname>
<given-names><![CDATA[L]]></given-names>
</name>
<name>
<surname><![CDATA[Jesus]]></surname>
<given-names><![CDATA[J]]></given-names>
</name>
<name>
<surname><![CDATA[Lorga]]></surname>
<given-names><![CDATA[T]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Susac syndrome - A Portuguese experience]]></article-title>
<source><![CDATA[The Pan-American Journal of Ophthalmology]]></source>
<year>2023</year>
<volume>5</volume>
<numero>1</numero>
<issue>1</issue>
</nlm-citation>
</ref>
<ref id="B12">
<nlm-citation citation-type="journal">
<person-group person-group-type="author">
<name>
<surname><![CDATA[Rennebohm]]></surname>
<given-names><![CDATA[RM]]></given-names>
</name>
<name>
<surname><![CDATA[Susac]]></surname>
<given-names><![CDATA[JO]]></given-names>
</name>
</person-group>
<article-title xml:lang=""><![CDATA[Treatment of Susac's syndrome]]></article-title>
<source><![CDATA[J Neurol Sci]]></source>
<year>2007</year>
<volume>257</volume>
<numero>1-2</numero>
<issue>1-2</issue>
<page-range>215-20</page-range></nlm-citation>
</ref>
</ref-list>
</back>
</article>
