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Images in Clinical Rheumatology
Confusional syndrome and retinal vasculitis
Síndrome confusional y vasculitis retiniana
Lucía Suárez-Péreza,
Corresponding author
luciasuarezp27@gmail.com

Corresponding author.
, Sara E. Herrero Suárezb, Jorge Peña Suárezc, Luis Caminal-Monteroa
a Unidad de Enfermedades Autoinmunes Sistémicas, Unidad de Gestión Clínica de Medicina Interna, Hospital Universitario Central de Asturias, Oviedo, Asturias, Spain
b Servicio de Neurología, Hospital Universitario Central de Asturias, Oviedo, Asturias, Spain
c Servicio de Radiología, Hospital Universitario Central de Asturias, Oviedo, Asturias, Spain
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    "textoCompleto" => "<span class="elsevierStyleSections"><p id="par0005" class="elsevierStylePara elsevierViewall">A 33-year-old male consulting due to behavioural disorders&#44; aphasia&#44; severe occipital headache&#44; unstable gait&#44; and hearing loss&#46; He denied substance abuse&#46;</p><p id="par0010" class="elsevierStylePara elsevierViewall">The following were highlighted on examination&#58; absence of meningeal signs&#44; sparse language with bradylalia&#44; bradypsychia&#44; amnesia and temporary disorientation&#46;</p><p id="par0015" class="elsevierStylePara elsevierViewall">Complementary studies&#58; haemogram&#44; general biochemistry&#44; C reactive protein and coagulation&#44; normal&#46; Lumbar puncture showed hyperproteinorrhoea&#44; with Gram&#44; panbacterial and myobacterial negative PCR&#46; Lues serologies&#44; HIV and neurotropic virus&#44; as well as ANA&#44; ANCA and HLA-B5 were negative&#46; A video-EEG showed signs of mild diffuse encephalopathy and cranial CT was normal&#46; Cranial MRI showed multiple supra-and intratentorial punctiform lesions in leptomeninges and &#8220;snowball&#8221; corpus callosum lesions &#40;<a class="elsevierStyleCrossRef" href="#fig0005">Fig&#46; 1</a>&#41;&#46;</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><p id="par0020" class="elsevierStylePara elsevierViewall">Treatment with steroids was initiated &#40;initial dose&#58; methylprednisolone 1&#8239;g&#47;24&#8239;h&#47;iv&#47;for 7 days&#44; later continued with 60&#8239;mg&#47;24&#8239;h oral prednisone in a decreasing regimen&#41; despite which he developed loss of visual acuity in both eyes&#44; and ischaemic vascular occlusions were found on fluorescein angiography &#40;FAG&#41; &#40;<a class="elsevierStyleCrossRef" href="#fig0010">Fig&#46; 2</a>&#41;&#46; In addition&#44; audiometry showed bilateral sensory hearing loss &#40;<a class="elsevierStyleCrossRef" href="#fig0015">Fig&#46; 3</a>&#41;&#46;</p><elsevierMultimedia ident="fig0010"></elsevierMultimedia><elsevierMultimedia ident="fig0015"></elsevierMultimedia><p id="par0025" class="elsevierStylePara elsevierViewall">Susac syndrome &#40;SS&#41; was diagnosed given the clinical triad of encephalopathy&#44; sensorineural hearing loss and retinal vasculitis with arterial occlusions&#46; SS is an immune-mediated&#44; pauci-inflammatory occlusive microvascular endotheliopathy that affects the brain&#44; the retina&#44; and the inner ear&#44; first described in 1979&#46;<a class="elsevierStyleCrossRefs" href="#bib0005"><span class="elsevierStyleSup">1&#44;2</span></a> Characteristic are snowball-shaped T2-FLAIR lesions on MRI at the level of the corpus callosum and occlusive retinal vasculitis on FAG&#46;<a class="elsevierStyleCrossRef" href="#bib0015"><span class="elsevierStyleSup">3</span></a></p><p id="par0030" class="elsevierStylePara elsevierViewall">The patient was treated with steroid boluses&#44; rituximab&#44; and acetylsalicylic acid and later&#44; as there was no improvement&#44; with immunoglobulins and mycophenolate&#44;<a class="elsevierStyleCrossRef" href="#bib0020"><span class="elsevierStyleSup">4</span></a> resulting in stabilisation of symptoms and then progressive reduction of steroid dose to 7&#46;5&#8239;mg&#47;24&#8239;h of prednisone after 9 months&#46;</p><span id="sec1040" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect1100">Funding</span><p id="par1180" class="elsevierStylePara elsevierViewall">There was no source of funding&#46;</p></span><span id="sec2040" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect2100">Conflict of interests</span><p id="par2180" class="elsevierStylePara elsevierViewall">The authors declare that they have no conflict of interests&#46;</p></span><span id="sec3040" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect3100">Acknowledgment</span><p id="par3180" class="elsevierStylePara elsevierViewall">In gratitude to Dra&#46; Miriam Garc&#237;a Fern&#225;ndez from the Service of Ophthalmology of the Hospital Universitario Central of Asturias for the AFG images&#46;</p></span></span>"
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Article information
ISSN: 21735743
Original language: English
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