Case report
General anesthesia considerations in CADASIL disease
Consideraciones para anestesia general en la enfermedad de CADASIL
M. Bermúdez-Triano, R. Guerrero-Domínguez
, A. Martínez-Saniger, I. Jiménez
Corresponding author
Servicio de Anestesiología y Reanimación, Hospital de Rehabilitación y Traumatología, Hospital Universitario Virgen del Rocío, Sevilla, Spain
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Bermúdez-Triano, R. Guerrero-Domínguez, A. Martínez-Saniger, I. Jiménez" "autores" => array:4 [ 0 => array:2 [ "nombre" => "M." "apellidos" => "Bermúdez-Triano" ] 1 => array:4 [ "nombre" => "R." "apellidos" => "Guerrero-Domínguez" "email" => array:1 [ 0 => "rosanabixi7@hotmail.com" ] "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">*</span>" "identificador" => "cor0005" ] ] ] 2 => array:2 [ "nombre" => "A." "apellidos" => "Martínez-Saniger" ] 3 => array:2 [ "nombre" => "I." "apellidos" => "Jiménez" ] ] "afiliaciones" => array:1 [ 0 => array:2 [ "entidad" => "Servicio de Anestesiología y Reanimación, Hospital de Rehabilitación y Traumatología, Hospital Universitario Virgen del Rocío, Sevilla, Spain" "identificador" => "aff0005" ] ] "correspondencia" => array:1 [ 0 => array:3 [ "identificador" => "cor0005" "etiqueta" => "⁎" "correspondencia" => "Corresponding author." ] ] ] ] "titulosAlternativos" => array:1 [ "es" => array:1 [ "titulo" => "Consideraciones para anestesia general en la enfermedad de CADASIL" ] ] "textoCompleto" => "<span class="elsevierStyleSections"><span id="sec0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0025">Introduction</span><p id="par0005" class="elsevierStylePara elsevierViewall">CADASIL (cerebral autosomal dominant arteriopathy with sub-cortical infarcts and leukoencephalopathy) syndrome is a rare hereditary systemic angiopathy that primarily affects small and medium-size brain vessels.<a class="elsevierStyleCrossRef" href="#bib0050"><span class="elsevierStyleSup">1</span></a> Symptoms include: migraine attacks (with or without aura), recurrent ischaemic strokes, subcortical dementia, and neuropsychiatric disorders.<a class="elsevierStyleCrossRef" href="#bib0055"><span class="elsevierStyleSup">2</span></a> It is a progressive disease in which cerebrovascular accidents are the primary cause of mortality.</p><p id="par0010" class="elsevierStylePara elsevierViewall">In these patients, anesthesia must be carefully planned to prevent new ischaemic and vasospastic phenomena.</p></span><span id="sec0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0030">Case report</span><p id="par0015" class="elsevierStylePara elsevierViewall">We present the case of a 55-year-old patient with CADASIL syndrome who, after an episode of left pontine haemorrhage with rupture into ventricles and secondary hydrocephalus, was scheduled for ventriculoperitoneal shunt. His personal history was significant for high blood pressure, hypercholesterolaemia, several episodes of lacunar stroke and tracheostomy due to prolonged admission to the intensive care unit. His medical treatment included paroxetine, amitriptyline, atorvastatin, diazepam, omeprazole, folic acid, aspirin and olmesartan.</p><p id="par0020" class="elsevierStylePara elsevierViewall">On physical examination he presented low level of consciousness with a Glasgow Coma Scale (GCS) score of 11 (eye response 4, verbal 1 and motor 6) and cardiorespiratory auscultation with generalised inspiratory rhonchi. The rest of the examination was unremarkable. Additional tests included biochemistry, blood count, coagulation study, electrocardiogram, and chest X-ray, all of which were normal (<a class="elsevierStyleCrossRef" href="#tbl0005">Table 1</a>).</p><elsevierMultimedia ident="tbl0005"></elsevierMultimedia><p id="par0025" class="elsevierStylePara elsevierViewall">On arrival in the operating room, monitoring with non-invasive blood pressure, peripheral oxygen saturation, electrocardiogram and bladder temperature probe was started. Anesthesia was induced with 1.5<span class="elsevierStyleHsp" style=""></span>mg<span class="elsevierStyleHsp" style=""></span>kg<span class="elsevierStyleSup">−1</span> propofol, 1<span class="elsevierStyleHsp" style=""></span>μg<span class="elsevierStyleHsp" style=""></span>kg<span class="elsevierStyleSup">−1</span>, fentanyl, 0.5<span class="elsevierStyleHsp" style=""></span>mg<span class="elsevierStyleHsp" style=""></span>kg<span class="elsevierStyleSup">−1</span> rocuronium, and 4<span class="elsevierStyleHsp" style=""></span>mg dexamethasone. Volume controlled mechanical ventilation was delivered through a non-fenestrated, cuffed tracheostomy tube. A left radial artery line was placed for continuous blood pressure monitoring. For surgery, the patient was placed supine with the head raised at a 30° angle. He was covered with a thermal blanket and fluids were warmed prior to infusion.</p><p id="par0030" class="elsevierStylePara elsevierViewall">Before making the surgical incision, the site was infiltrated with 20<span class="elsevierStyleHsp" style=""></span>ml of 0.25% bupivacaine. Anesthesia was maintained with 0.7 MAC desflurane, with 1<span class="elsevierStyleHsp" style=""></span>μg<span class="elsevierStyleHsp" style=""></span>kg<span class="elsevierStyleSup">−1</span> fentanyl and strict haemodynamic control to maintain mean arterial pressure (MAP) at 80<span class="elsevierStyleHsp" style=""></span>mmHg, normocarbia and normothermia. Surgery, which lasted 40<span class="elsevierStyleHsp" style=""></span>min, was uneventful.</p><p id="par0035" class="elsevierStylePara elsevierViewall">After surgery, paracetamol 1<span class="elsevierStyleHsp" style=""></span>g and ondansetron 6<span class="elsevierStyleHsp" style=""></span>mg were administered, and 200<span class="elsevierStyleHsp" style=""></span>mg sugammadex to reverse neuromuscular blockade.</p><p id="par0040" class="elsevierStylePara elsevierViewall">The postoperative examination showed progressive neurological improvement and no new signs of focality. Intravenous paracetamol 1<span class="elsevierStyleHsp" style=""></span>g every 8<span class="elsevierStyleHsp" style=""></span>h was the chosen postoperative analgesia. This gave satisfactory pain relief, with a score of less than 3 on a visual analogue scale (VAS).</p></span><span id="sec0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0035">Discussion</span><p id="par0045" class="elsevierStylePara elsevierViewall">CADASIL syndrome is caused by mutations in the NOTCH3 gene located on chromosome 19.<a class="elsevierStyleCrossRef" href="#bib0050"><span class="elsevierStyleSup">1</span></a> This family of genes encodes transmembrane receptors involved in cell growth and differentiation during development. Over 300 NOTCH3 gene mutations associated with CADASIL disease have been described, of which approximately 95% are due to the addition or elimination of cysteine residues within EGF repeats in the extracellular domains.<a class="elsevierStyleCrossRef" href="#bib0055"><span class="elsevierStyleSup">2</span></a> This results in the aberrant polymerisation and abnormal deposits of NOTCH3. Histologically, granular osmiophilic material (GOM) deposition around vascular smooth muscle cells is a specific diagnostic feature of CADASIL.<a class="elsevierStyleCrossRefs" href="#bib0050"><span class="elsevierStyleSup">1,3</span></a> Recent investigations using immunoelectron microscopy analysis with antibodies against the extra- and intracellular portions of NOTCH3, showed the NOTCH3 ectodomain to be a major component of GOM.<a class="elsevierStyleCrossRef" href="#bib0065"><span class="elsevierStyleSup">4</span></a> Despite the latest findings, the reason the systemic arteriopathy specifically occurs in the central nervous system is still unclear.</p><p id="par0050" class="elsevierStylePara elsevierViewall">Symptoms usually start at around 45 years of age, and consist of migraine attacks, psychiatric disorders (depression and behavioural disorders, among others), recurrent ischaemic strokes and cognitive deterioration that progresses to dementia. Only about 500 families are affected worldwide, most of them in Europe.<a class="elsevierStyleCrossRef" href="#bib0070"><span class="elsevierStyleSup">5</span></a></p><p id="par0055" class="elsevierStylePara elsevierViewall">Reports of anesthesia management in patients with CADASIL syndrome are scant. We searched Cochrane Library, DOCUMED, ERIC (USDE), IBECS, IME-Biomedicine, LILACS, MEDLINE, Pubmed, PubPsych and SciELo databases in Spanish and English, using the terms “CADASIL” and “anesthesia” with no date limit. This retrieved 5 cases, only one of them involving general anesthaesia,<a class="elsevierStyleCrossRef" href="#bib0075"><span class="elsevierStyleSup">6</span></a> and none involving brain surgery, despite the high association of this syndrome with neurosurgical procedures.</p><p id="par0060" class="elsevierStylePara elsevierViewall">The primary objective of anesthesia management in this disease is to maintain adequate cerebral perfusion pressure (CPP).<a class="elsevierStyleCrossRefs" href="#bib0070"><span class="elsevierStyleSup">5–7</span></a> For this purpose, MAP must be closely monitored using an arterial line in order to maintain pressure levels within the limits of cerebral auto-regulation. In our case, we aimed for a MAP greater than 80<span class="elsevierStyleHsp" style=""></span>mmHg; given the patient's history of arterial hypertension, the cerebral blood flow autoregulation curve would be right-shifted. It is also important to monitor and maintain normothermia and normocapnia in order to avoid vasospastic phenomena.</p><p id="par0065" class="elsevierStylePara elsevierViewall">In our patient, we opted for balanced inhalation anesthesia with desflurane, since it provides greater haemodynamic stability than total intravenous anesthesia with propofol. Titration of this agent is simpler, and at doses lower than or equal to minimum alveolar concentration (MAC) it does not alter the self-regulation of cerebral blood flow.<a class="elsevierStyleCrossRefs" href="#bib0075"><span class="elsevierStyleSup">6,8</span></a> In addition, given the characteristics of the patient, the blood/gas partition coefficient of desflurane (0.42) allows rapid reduction and early neurological examination. The use of sevoflurane can produce epileptiform electroencephalographic patterns in frail, predisposed patients.<a class="elsevierStyleCrossRefs" href="#bib0085"><span class="elsevierStyleSup">8,9</span></a></p><p id="par0070" class="elsevierStylePara elsevierViewall">We administered 75<span class="elsevierStyleHsp" style=""></span>μg intravenous fentanyl (1<span class="elsevierStyleHsp" style=""></span>μg<span class="elsevierStyleHsp" style=""></span>kg<span class="elsevierStyleSup">−1</span>) for anesthesia prior to inserting the ventriculoperitoneal shunt. This preserved haemodynamic stability and provided good residual analgesia in the immediate postoperative period. In this case, we ruled out continuous infusion of remifentanil given the short duration of surgery (40<span class="elsevierStyleHsp" style=""></span>min), low analgesic requirement, and the need for an opioid that would provide analgesic in the postoperative period. If vasoconstrictors are needed to guarantee a certain MAP, direct-acting drugs such as noradrenaline or phenylephrine appear to be more appropriate, although the primary concern is to optimise fluid therapy to avoid hypovolaemia.<a class="elsevierStyleCrossRefs" href="#bib0075"><span class="elsevierStyleSup">6,7</span></a> If hypertensive crises should occur, nimodipine would by the antihypertensive of choice, due to its prophylactic effect on cerebral vasospasm.<a class="elsevierStyleCrossRef" href="#bib0075"><span class="elsevierStyleSup">6</span></a></p><p id="par0075" class="elsevierStylePara elsevierViewall">Another factor that will prevent intracranial pressure (IP) build-up and the consequent decrease in CPP is the surgical position. The head should be raised to an angle of 30° to avoid obstruction of cerebral venous drainage.<a class="elsevierStyleCrossRefs" href="#bib0070"><span class="elsevierStyleSup">5,6</span></a> It is also important to prevent postoperative nausea and vomiting that could contribute to an increase in intracranial pressure.</p><p id="par0080" class="elsevierStylePara elsevierViewall">Postoperative analgesia with paracetamol was satisfactory in our patient. If additional analgesia is required, 1<span class="elsevierStyleHsp" style=""></span>mg<span class="elsevierStyleHsp" style=""></span>kg<span class="elsevierStyleSup">−1</span> tramadol is a good alternative that does not alter IP or CPP.<a class="elsevierStyleCrossRef" href="#bib0075"><span class="elsevierStyleSup">6</span></a> Major opioids, given their potential for respiratory depression, should be used with caution in order to avoid hypercapnia.</p><p id="par0085" class="elsevierStylePara elsevierViewall">In conclusion, we describe for the first time in the literature the anaesthetic management of an intracranial neurosurgical procedure in a patient with CADASIL syndrome. Based on our experience, we consider invasive MAP monitoring to be essential for ensuring adequate CPP. Normocapnia and normothermia should also be monitored. However, many aspects of preserving cerebral autoregulation in these patients are unknown, and future anesthesia studies in this context are required.</p></span><span id="sec0020" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0040">Conflicts of interest</span><p id="par0090" class="elsevierStylePara elsevierViewall">The authors declare that they have no conflicts of interest.</p></span></span>" "textoCompletoSecciones" => array:1 [ "secciones" => array:9 [ 0 => array:3 [ "identificador" => "xres1183077" "titulo" => "Abstract" "secciones" => array:1 [ 0 => array:1 [ "identificador" => "abst0005" ] ] ] 1 => array:2 [ "identificador" => "xpalclavsec1103666" "titulo" => "Keywords" ] 2 => array:3 [ "identificador" => "xres1183078" "titulo" => "Resumen" "secciones" => array:1 [ 0 => array:1 [ "identificador" => "abst0010" ] ] ] 3 => array:2 [ "identificador" => "xpalclavsec1103665" "titulo" => "Palabras clave" ] 4 => array:2 [ "identificador" => "sec0005" "titulo" => "Introduction" ] 5 => array:2 [ "identificador" => "sec0010" "titulo" => "Case report" ] 6 => array:2 [ "identificador" => "sec0015" "titulo" => "Discussion" ] 7 => array:2 [ "identificador" => "sec0020" "titulo" => "Conflicts of interest" ] 8 => array:1 [ "titulo" => "References" ] ] ] "pdfFichero" => "main.pdf" "tienePdf" => true "fechaRecibido" => "2018-08-08" "fechaAceptado" => "2018-10-31" "PalabrasClave" => array:2 [ "en" => array:1 [ 0 => array:4 [ "clase" => "keyword" "titulo" => "Keywords" "identificador" => "xpalclavsec1103666" "palabras" => array:5 [ 0 => "General anesthesia" 1 => "CADASIL disease" 2 => "Rare diseases" 3 => "Cerebral ischaemia" 4 => "Neurological diseases" ] ] ] "es" => array:1 [ 0 => array:4 [ "clase" => "keyword" "titulo" => "Palabras clave" "identificador" => "xpalclavsec1103665" "palabras" => array:5 [ 0 => "Anestesia general" 1 => "Enfermedad de CADASIL" 2 => "Enfermedades poco frecuentes" 3 => "Isquemia cerebral" 4 => "Enfermedades neurológicas" ] ] ] ] "tieneResumen" => true "resumen" => array:2 [ "en" => array:2 [ "titulo" => "Abstract" "resumen" => "<span id="abst0005" class="elsevierStyleSection elsevierViewall"><p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">CADASIL (cerebral arteriopathy, autosomal dominant, with subcortical infarcts and leukoencephalopathy) disease is an inherited systemic arterial disease that affects the small and medium calibre cerebral vessels. Around 500 families are affected in the world, most of them in Europe. It is characterised by migraine attacks, subcortical dementia, neuropsychiatric disorders, and recurrent ischaemic strokes.</p><p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">The objective of this article is to describe, for the first time in the literature, the management by general anesthesia of an intracranial neurosurgical procedure in a patient with CADASIL disease. Continuous monitoring of blood pressure is considered essential, as well as the maintenance of normocapnia and normothermia to avoid the development of new cerebrovascular accidents. This disease is relevant due to its anaesthetic implications and the few publications to date.</p></span>" ] "es" => array:2 [ "titulo" => "Resumen" "resumen" => "<span id="abst0010" class="elsevierStyleSection elsevierViewall"><p id="spar0015" class="elsevierStyleSimplePara elsevierViewall">La enfermedad de CADASIL (arteriopatía cerebral autosómica dominante con infartos subcorticales y leucoencefalopatía) es una angiopatía sistémica hereditaria que afecta fundamentalmente a los vasos cerebrales de pequeño y mediano calibre. Alrededor de 500 familias están afectadas en el mundo, la mayoría de ellas en Europa. Se caracteriza por presentar crisis de migraña, demencia subcortical, trastornos neuropsiquiátricos e ictus isquémicos de repetición.</p><p id="spar0020" class="elsevierStyleSimplePara elsevierViewall">Nuestro objetivo ha sido describir por primera vez en la literatura el manejo mediante anestesia general de un procedimiento neuroquirúrgico intracraneal en un paciente con la enfermedad de CADASIL. Consideramos esencial la monitorización continua de la presión arterial, así como el mantenimiento de normocapnia y normotermia para evitar el desarrollo de nuevos accidentes cerebrovasculares. Esta enfermedad resulta relevante debido a sus implicaciones anestésicas y las escasas publicaciones hasta la fecha.</p></span>" ] ] "NotaPie" => array:1 [ 0 => array:2 [ "etiqueta" => "☆" "nota" => "<p class="elsevierStyleNotepara" id="npar0005">Please cite this article as: Bermúdez-Triano M, Guerrero-Domínguez R, Martínez-Saniger A, Jiménez I. Consideraciones para anestesia general en la enfermedad de CADASIL. Rev Esp Anestesiol Reanim. 2019;66:226–229.</p>" ] ] "multimedia" => array:1 [ 0 => array:8 [ "identificador" => "tbl0005" "etiqueta" => "Table 1" "tipo" => "MULTIMEDIATABLA" "mostrarFloat" => true "mostrarDisplay" => false "detalles" => array:1 [ 0 => array:3 [ "identificador" => "at1" "detalle" => "Table " "rol" => "short" ] ] "tabla" => array:2 [ "leyenda" => "<p id="spar0030" class="elsevierStyleSimplePara elsevierViewall"><span class="elsevierStyleItalic">Source</span>: Adapted from Guarnaschelli and Sotelo.<a class="elsevierStyleCrossRef" href="#bib0060"><span class="elsevierStyleSup">3</span></a></p>" "tablatextoimagen" => array:1 [ 0 => array:2 [ "tabla" => array:1 [ 0 => """ <table border="0" frame="\n \t\t\t\t\tvoid\n \t\t\t\t" class=""><tbody title="tbody"><tr title="table-row"><td class="td" title="table-entry " rowspan="9" align="left" valign="top">Probable CADASIL</td><td class="td" title="table-entry " align="left" valign="top">Age of onset younger than 50 years \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">At least 2 of the following clinical findings: \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">• Stroke-like episodes with permanent neurological signs \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">• Migraine \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">• Major mood disorder \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">• Subcortical dementia \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Absence of cardiovascular risk factors etiologically related to the deficit \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Evidence of autosomal dominant transmission \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Magnetic resonance imaging showing white matter lesions with not cortical infarcts \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Definitive CADASIL \t\t\t\t\t\t\n \t\t\t\t</td><td class="td" title="table-entry " align="left" valign="top">Probable CADASIL criteria<span class="elsevierStyleHsp" style=""></span>+<span class="elsevierStyleHsp" style=""></span>evidence of mutation in the NOTCH3 gene and/or pathological findings of small vessel arteriopathy with GOM deposits \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="table-entry " rowspan="7" align="left" valign="top">Possible CADASIL</td><td class="td" title="table-entry " align="left" valign="top">Late onset, after 50 years \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Stroke-like episodes without permanent signs: \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">• Minor mood disorder \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">• Global dementia \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Minor vascular risk factors: mild hypertension, mild hyperlipidaemia, smoking habit, oral contraceptives \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Unknown or incomplete family history \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Magnetic resonance showing atypical white matter lesions \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="table-entry " rowspan="4" align="left" valign="top">Exclusion criteria</td><td class="td" title="table-entry " align="left" valign="top">Age of onset >70 years \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Severe or complicated hypertension with cardiac or systemic vascular disease \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">No hereditary transmission \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="table-entry ; entry_with_role_rowhead " align="left" valign="top">Normal magnetic resonance in patients >35 years \t\t\t\t\t\t\n \t\t\t\t</td></tr></tbody></table> """ ] "imagenFichero" => array:1 [ 0 => "xTab2016396.png" ] ] ] ] "descripcion" => array:1 [ "en" => "<p id="spar0025" class="elsevierStyleSimplePara elsevierViewall">Diagnostic criteria for CADASIL syndrome.</p>" ] ] ] "bibliografia" => array:2 [ "titulo" => "References" "seccion" => array:1 [ 0 => array:2 [ "identificador" => "bibs0015" "bibliografiaReferencia" => array:9 [ 0 => array:3 [ "identificador" => "bib0050" "etiqueta" => "1" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Pathophysiology of CADASIL disease [article in Spanish]" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:3 [ 0 => "A. 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