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Parinaud “plus” syndrome in a patient with dysgerminoma
Síndrome de Parinaud «plus» en paciente con disgerminoma
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Burgueño-Montañés, C. Santalla-Castro, J. Peña-Suárez" "autores" => array:3 [ 0 => array:4 [ "nombre" => "C." "apellidos" => "Burgueño-Montañés" "email" => array:1 [ 0 => "casamata@gmail.com" ] "referencia" => array:2 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] 1 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">*</span>" "identificador" => "cor0005" ] ] ] 1 => array:3 [ "nombre" => "C." "apellidos" => "Santalla-Castro" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] ] ] 2 => array:3 [ "nombre" => "J." "apellidos" => "Peña-Suárez" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">b</span>" "identificador" => "aff0010" ] ] ] ] "afiliaciones" => array:2 [ 0 => array:3 [ "entidad" => "Servicio de Oftalmología, Hospital Universitario Central de Asturias, Oviedo, Asturias, Spain" "etiqueta" => "a" "identificador" => "aff0005" ] 1 => array:3 [ "entidad" => "Servicio de Radiodiagnóstico, Hospital Universitario Central de Asturias, Oviedo, Asturias, Spain" "etiqueta" => "b" "identificador" => "aff0010" ] ] "correspondencia" => array:1 [ 0 => array:3 [ "identificador" => "cor0005" "etiqueta" => "⁎" "correspondencia" => "<span class="elsevierStyleItalic">Corresponding author</span>." ] ] ] ] "titulosAlternativos" => array:1 [ "es" => array:1 [ "titulo" => "Síndrome de Parinaud «plus» en paciente con disgerminoma" ] ] "resumenGrafico" => array:2 [ "original" => 0 "multimedia" => array:7 [ "identificador" => "fig0010" "etiqueta" => "Fig. 2" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr2.jpeg" "Alto" => 527 "Ancho" => 1400 "Tamanyo" => 114046 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0030" class="elsevierStyleSimplePara elsevierViewall">Fundus examination: prepapillary gliosis, left papillary pallor and folds in the papillomacular beam which, in the LE, reach the macula.</p>" ] ] ] "textoCompleto" => "<span class="elsevierStyleSections"><span id="sec0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0045">Introduction</span><p id="par0005" class="elsevierStylePara elsevierViewall">Parinaud's syndrome (PS) or dorsal midbrain syndrome is a supranuclear palsy of the vertical gaze due to compression of or ischaemic damage to the midbrain <span class="elsevierStyleItalic">tectum</span>.</p><p id="par0010" class="elsevierStylePara elsevierViewall">PS “plus” manifests with other neuro-ophthalmological signs secondary to involvement of adjacent structures, with lesions at the nuclei or ganglia of cranial nerves III, IV, VI and VII, horizontal gaze palsy, Foville syndrome, one and a half syndrome; or distant structures, such as lesion of the II cranial nerve due to hydrocephalus when the aqueduct of Sylvius is obstructed.</p></span><span id="sec0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0050">Case report</span><p id="par0015" class="elsevierStylePara elsevierViewall">This was a 33-year-old male with a 2-year history of occasional diplopia who consulted due to constant diplopia, altered visual field, loss of balance and urinary incontinence in the previous few weeks. He had no ophthalmological history of interest or previous amblyopia. One year earlier, he had attended A&E and had a computed tomography (CT) scan of the brain which was reported as normal. He had slightly diminished tendon reflexes on the left side of the body and unsteady gait. Pituitary function was normal, and ophthalmology examination revealed:<ul class="elsevierStyleList" id="lis0005"><li class="elsevierStyleListItem" id="lsti0005"><span class="elsevierStyleLabel">–</span><p id="par0020" class="elsevierStylePara elsevierViewall">Best corrected visual acuity (BCVA) RE 0.8 and LE 0.5. Upper gaze palsy, convergence-retraction nystagmus (PS). Exotropia of LE of −50Δ. No limitation in adduction or abduction (<a class="elsevierStyleCrossRef" href="#fig0005">Fig. 1</a>). Fundus examination (FE): prepapillary gliosis and folds in the residual papillomacular beam after major papillary and peripapillary oedema, and papillary pallor in the LE (<a class="elsevierStyleCrossRef" href="#fig0010">Fig. 2</a>). Visual field (VF) 30-2: RE paracentral defects, LE superior arciform scotoma (<a class="elsevierStyleCrossRef" href="#fig0015">Fig. 3</a>). The optical coherence tomography (OCT) of the optic nerve fibre layer was 75 (borderline) in RE and 68 (abnormal) in LE (<a class="elsevierStyleCrossRef" href="#fig0020">Fig. 4</a>).</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><elsevierMultimedia ident="fig0010"></elsevierMultimedia><elsevierMultimedia ident="fig0015"></elsevierMultimedia><elsevierMultimedia ident="fig0020"></elsevierMultimedia></li><li class="elsevierStyleListItem" id="lsti0010"><span class="elsevierStyleLabel">–</span><p id="par0025" class="elsevierStylePara elsevierViewall">CT and magnetic resonance imaging (MRI) showed a tumour in the pineal gland of approximately 25<span class="elsevierStyleHsp" style=""></span>mm, obstructive hydrocephaly and incipient ependymal dissemination in the left lateral ventricle. “Pineal gland germinoma” diagnosed after stereotactic biopsy and immunohistochemical positivity for placental alkaline phosphatase (PLAP) and CD117. Ventriculoperitoneal shunt valve implanted and chemotherapy given (cisplatin-etoposide 5 days/4 cycles), with virtually complete tumour response, plus additional radical radiotherapy (48.6<span class="elsevierStyleHsp" style=""></span>Gy) (<a class="elsevierStyleCrossRef" href="#fig0025">Fig. 5</a>).</p><elsevierMultimedia ident="fig0025"></elsevierMultimedia></li></ul></p><p id="par0030" class="elsevierStylePara elsevierViewall">Correction of strabismus assessed but declined by the patient (prevents the diplopia but with drawbacks). The PS continues to persist a year after diagnosis.</p></span><span id="sec0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0055">Discussion</span><p id="par0035" class="elsevierStylePara elsevierViewall">PS is a set of disorders of eye movements and pupillary dysfunction characterised by any of the following signs<a class="elsevierStyleCrossRef" href="#bib0035"><span class="elsevierStyleSup">1</span></a>:<ul class="elsevierStyleList" id="lis0010"><li class="elsevierStyleListItem" id="lsti0015"><span class="elsevierStyleLabel">1.</span><p id="par0040" class="elsevierStylePara elsevierViewall">Supranuclear palsy of the conjugate upward gaze (the most characteristic) with eyes straight in the primary position.</p></li><li class="elsevierStyleListItem" id="lsti0020"><span class="elsevierStyleLabel">2.</span><p id="par0045" class="elsevierStylePara elsevierViewall">Pupils semi-dilated with light-near dissociation.</p></li><li class="elsevierStyleListItem" id="lsti0025"><span class="elsevierStyleLabel">3.</span><p id="par0050" class="elsevierStylePara elsevierViewall">Convergence-retraction nystagmus.</p></li><li class="elsevierStyleListItem" id="lsti0030"><span class="elsevierStyleLabel">4.</span><p id="par0055" class="elsevierStylePara elsevierViewall">Upper eyelid retraction (Collier's sign).</p></li><li class="elsevierStyleListItem" id="lsti0035"><span class="elsevierStyleLabel">5.</span><p id="par0060" class="elsevierStylePara elsevierViewall">Rarer signs include vergence dysfunction (usually convergence spasm, but sometimes paralysis), pseudoabducens palsy (thalamic esotropia) and exotropia.</p></li></ul></p><p id="par0065" class="elsevierStylePara elsevierViewall">PS is observed in tumours of the pineal gland, the midbrain or the 3rd ventricle, in multiple sclerosis and midbrain infarction or abscess.</p><p id="par0070" class="elsevierStylePara elsevierViewall">Pineal gland tumours and trauma most commonly cause PS “plus”.</p><p id="par0075" class="elsevierStylePara elsevierViewall">Pineal gland tumours are rare, less than 1% of intracranial tumours; 50% come from germ cells and of these, the majority (2/3) are germinomas.<a class="elsevierStyleCrossRefs" href="#bib0035"><span class="elsevierStyleSup">1–3</span></a></p><p id="par0080" class="elsevierStylePara elsevierViewall">Germinomas develop in midline and are most common in the pineal gland (50–65%). They affect males more than females (3:1), but in the pineal region, the ratio becomes 12:1. Mean age at diagnosis is 10–12 years, with 90% being under 30. It may take from several months to years between first symptoms and diagnosis.<a class="elsevierStyleCrossRefs" href="#bib0040"><span class="elsevierStyleSup">2,4</span></a> The association of neuro-ophthalmological and neuroendocrine signs (diabetes insipidus, hypopituitarism, abnormal pubertal development) is characteristic. The most common are signs of intracranial hypertension and PS (for many pathognomonic).<a class="elsevierStyleCrossRef" href="#bib0035"><span class="elsevierStyleSup">1</span></a> There are patients with a long history of symptoms such as movement disorders, enuresis, anorexia or psychiatric disorders. Sensitive to radiotherapy and chemotherapy, the standard treatments are constantly evolving with the aim of minimising the sequelae.<a class="elsevierStyleCrossRef" href="#bib0045"><span class="elsevierStyleSup">3</span></a> Survival at 5 years is 80–90%.<a class="elsevierStyleCrossRef" href="#bib0040"><span class="elsevierStyleSup">2</span></a></p><p id="par0085" class="elsevierStylePara elsevierViewall">The proximity of the pineal gland to the quadrigeminal bodies means that signs and symptoms of midbrain origin are prevalent.</p><p id="par0090" class="elsevierStylePara elsevierViewall">Lesions of the upper midbrain in the pretectum interrupt decussation of the supranuclear fibres when they pass the rostral interstitial nucleus of the medial longitudinal fasciculus in the midbrain (vertical gaze centre); they interrupt the retinotectal fibres, with preservation of the supranuclear pathways of the pupillary response to near stimulus, chromatic stimulus and sleep-related oscillations, producing a light-near dissociation<a class="elsevierStyleCrossRef" href="#bib0055"><span class="elsevierStyleSup">5</span></a>; and they affect the pretectal pupillary constriction nuclei.</p><p id="par0095" class="elsevierStylePara elsevierViewall">In a review of 18 patients with PS by Moguel-Ancheita et al.,<a class="elsevierStyleCrossRef" href="#bib0060"><span class="elsevierStyleSup">6</span></a> the most commonly-associated strabismus was exotropia (12 out of 18), and in 44.4%, the II cranial nerve was affected.</p><p id="par0100" class="elsevierStylePara elsevierViewall">Treatment of PS is directed at the cause. Ocular signs slowly improve once the causal factor has been resolved, and this often persists even after therapy. The associated strabismus is treated with botulinum toxin,<a class="elsevierStyleCrossRef" href="#bib0060"><span class="elsevierStyleSup">6</span></a> and if there is no response or in residual strabismus, surgery can be an option.</p></span><span id="sec0020" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0060">Conflicts of interest</span><p id="par0105" class="elsevierStylePara elsevierViewall">The authors declare that there are no conflicts of interest.</p></span></span>" "textoCompletoSecciones" => array:1 [ "secciones" => array:9 [ 0 => array:3 [ "identificador" => "xres676665" "titulo" => "Abstract" "secciones" => array:2 [ 0 => array:2 [ "identificador" => "abst0005" "titulo" => "Clinical case" ] 1 => array:2 [ "identificador" => "abst0010" "titulo" => "Discussion" ] ] ] 1 => array:2 [ "identificador" => "xpalclavsec682697" "titulo" => "Keywords" ] 2 => array:3 [ "identificador" => "xres676666" "titulo" => "Resumen" "secciones" => array:2 [ 0 => array:2 [ "identificador" => "abst0015" "titulo" => "Caso clínico" ] 1 => array:2 [ "identificador" => "abst0020" "titulo" => "Discusión" ] ] ] 3 => array:2 [ "identificador" => "xpalclavsec682696" "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" => "2015-08-05" "fechaAceptado" => "2016-01-11" "PalabrasClave" => array:2 [ "en" => array:1 [ 0 => array:4 [ "clase" => "keyword" "titulo" => "Keywords" "identificador" => "xpalclavsec682697" "palabras" => array:5 [ 0 => "Parinaud's syndrome" 1 => "Dorsal midbrain syndrome" 2 => "Germinoma" 3 => "Dysgerminoma" 4 => "Pineal tumours" ] ] ] "es" => array:1 [ 0 => array:4 [ "clase" => "keyword" "titulo" => "Palabras clave" "identificador" => "xpalclavsec682696" "palabras" => array:5 [ 0 => "Síndrome de Parinaud" 1 => "Síndrome mesencefálico dorsal" 2 => "Germinoma" 3 => "Disgerminoma" 4 => "Tumores pineales" ] ] ] ] "tieneResumen" => true "resumen" => array:2 [ "en" => array:3 [ "titulo" => "Abstract" "resumen" => "<span id="abst0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0010">Clinical case</span><p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">A 33-year-old male diagnosed with Parinaud's syndrome, exotropia and post-papillary oedema optic atrophy in his left eye. A pineal germinoma was diagnosed after performing neuroimaging scans and a stereotactic biopsy. He was treated with chemotherapy and radiotherapy, showing a complete pathological response. The Parinaud's syndrome persists one year after diagnosis and the patient has refused to have strabismus surgery.</p></span> <span id="abst0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0015">Discussion</span><p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">Parinaud's syndrome consists of a supranuclear vertical gaze palsy resulting from damage to the midbrain tectum. The involvement of adjacent structures leads to the “Parinaud-plus” syndrome. When a Parinaud's syndrome is accompanied by diplopia (“Parinaud-plus” syndrome), extension of the injury into adjacent areas must be considered.</p></span>" "secciones" => array:2 [ 0 => array:2 [ "identificador" => "abst0005" "titulo" => "Clinical case" ] 1 => array:2 [ "identificador" => "abst0010" "titulo" => "Discussion" ] ] ] "es" => array:3 [ "titulo" => "Resumen" "resumen" => "<span id="abst0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0025">Caso clínico</span><p id="spar0015" class="elsevierStyleSimplePara elsevierViewall">Varón de 33 años con síndrome de Parinaud, exotropía y atrofia óptica del OI postpapiledema. Tras las pruebas de neuroimagen y biopsia estereotáxica se diagnostica de germinoma pineal. Recibe quimio y radioterapia con respuesta completa del tumour. El síndrome de Parinaud persiste un año después del diagnóstico, y rechaza la corrección del estrabismo.</p></span> <span id="abst0020" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0030">Discusión</span><p id="spar0020" class="elsevierStyleSimplePara elsevierViewall">El síndrome de Parinaud consiste en una parálisis supranuclear de la mirada vertical por daño del <span class="elsevierStyleItalic">tectum</span> mesencefálico. El compromiso de las estructuras adyacentes da lugar al síndrome de Parinaud «plus». Ante un síndrome de Parinaud acompañado de diplopía (síndrome de Parinaud «plus») se debe pensar en la extensión de la lesión a otras áreas adyacentes.</p></span>" "secciones" => array:2 [ 0 => array:2 [ "identificador" => "abst0015" "titulo" => "Caso clínico" ] 1 => array:2 [ "identificador" => "abst0020" "titulo" => "Discusión" ] ] ] ] "NotaPie" => array:1 [ 0 => array:2 [ "etiqueta" => "☆" "nota" => "<p class="elsevierStyleNotepara" id="npar0005">Please cite this article as: Burgueño-Montañés C, Santalla-Castro C, Peña-Suárez J. Síndrome de Parinaud «plus» en paciente con disgerminoma. Arch Soc Esp Oftalmol. 2016;91:341–345.</p>" ] ] "multimedia" => array:5 [ 0 => array:7 [ "identificador" => "fig0005" "etiqueta" => "Fig. 1" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr1.jpeg" "Alto" => 1233 "Ancho" => 1754 "Tamanyo" => 359496 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0025" class="elsevierStyleSimplePara elsevierViewall">Upper gaze palsy. Exotropia of LE.</p>" ] ] 1 => array:7 [ "identificador" => "fig0010" "etiqueta" => "Fig. 2" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr2.jpeg" "Alto" => 527 "Ancho" => 1400 "Tamanyo" => 114046 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0030" class="elsevierStyleSimplePara elsevierViewall">Fundus examination: prepapillary gliosis, left papillary pallor and folds in the papillomacular beam which, in the LE, reach the macula.</p>" ] ] 2 => array:7 [ "identificador" => "fig0015" "etiqueta" => "Fig. 3" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr3.jpeg" "Alto" => 3054 "Ancho" => 2870 "Tamanyo" => 410246 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0035" class="elsevierStyleSimplePara elsevierViewall">Visual field (Carl Zeiss Meditec, Inc., Dublin, CA) 30-2.</p>" ] ] 3 => array:7 [ "identificador" => "fig0020" "etiqueta" => "Fig. 4" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr4.jpeg" "Alto" => 3088 "Ancho" => 3333 "Tamanyo" => 739312 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0040" class="elsevierStyleSimplePara elsevierViewall">OCT (Cirrus<span class="elsevierStyleSup">®</span> HD-OCT, Carl Zeiss Meditec, Inc., Dublin, CA) of the optic nerve fibre layers and the ganglion cells.</p>" ] ] 4 => array:7 [ "identificador" => "fig0025" "etiqueta" => "Fig. 5" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr5.jpeg" "Alto" => 1311 "Ancho" => 1902 "Tamanyo" => 353434 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0045" class="elsevierStyleSimplePara elsevierViewall">MRI pre- (below) and post-treatment (above).</p>" ] ] ] "bibliografia" => array:2 [ "titulo" => "References" "seccion" => array:1 [ 0 => array:2 [ "identificador" => "bibs0005" "bibliografiaReferencia" => array:6 [ 0 => array:3 [ "identificador" => "bib0035" "etiqueta" => "1" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Pineal region tumors: clinical symptoms and syndromes" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:4 [ 0 => "C. 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