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    "textoCompleto" => "<span class="elsevierStyleSections"><p id="par0005" class="elsevierStylePara elsevierViewall">Laughter is an inherent and essential part of human behaviour&#46; Its impact throughout our culture may be observed from the texts of the great civilisations&#44; from classical Greece&#44; to today&#39;s popular culture&#46; The beneficial impact of laughter in medicine has been widely demonstrated in&#44; for example&#44; cardiovascular health&#44; behaviour patterns and learning&#46;<a class="elsevierStyleCrossRefs" href="#bib0045"><span class="elsevierStyleSup">1&#8211;4</span></a></p><p id="par0010" class="elsevierStylePara elsevierViewall">On the other end of the laughter type classification spectrum is abnormal laughter&#46; This is defined as something which occurs without any previous stimulus and which is of inappropriate intensity&#46; Similarly&#44; to the first type of laughter&#44; there are many references to abnormal laughter&#46; One recent reference is the <span class="elsevierStyleItalic">Joker</span> antihero&#44; whose distinctive trait is his scandalous&#44; abnormal&#44; and on occasions&#44; malevolent laughter&#46;</p><p id="par0015" class="elsevierStylePara elsevierViewall">The aetiology of abnormal laughter&#44; which is a component of pseudobulbar syndrome&#44; is extensive and includes neurodegenerative&#44; tumoral&#44; traumatic and even vascular diseases&#46; In Beh&#231;et&#39;s disease&#44; primary systemic vasculitis of variably sized blood vessels and involvement of the central nervous system at disease onset is rare and occurs less frequently than that of other organs&#46; Inappropriate laughter as part of the neuropsychiatric symptoms has been infrequently reported&#46;<a class="elsevierStyleCrossRefs" href="#bib0065"><span class="elsevierStyleSup">5&#8211;7</span></a></p><p id="par0020" class="elsevierStylePara elsevierViewall">We report 2 cases of patients with Beh&#231;et&#39;s disease and with &#8220;joker syndrome&#8221; traits&#46;</p><span id="sec0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0005">Case 1</span><p id="par0025" class="elsevierStylePara elsevierViewall">Male&#44; 23 years of age&#44; previously healthy&#44; with 6-month onset of constitutional symptoms&#44; ataxia&#44; dysmetria and dysarthria&#46; Three months later additional symptoms of polyarthritis&#44; pustular dermatosis&#44; and oral and genital ulcers presented&#46; Diagnosis ruled out infectious aetiologies and confirmed the existence of retinal vasculitis in fluorescein angiography and in magnetic resonance imaging&#44; which showed predominantly mesencephalic inflammatory lesions&#46; Beh&#231;et&#39;s disease was considered and was treated with methylprednisolone and cyclophosphamide with favourable response&#46; During the course of the disease the patient again presented with ataxia&#44; dysphagia&#44; and dysarthria&#44; accompanied by episodes of intense laughter&#46; His mother reported that the laughter even occurred in situations such as when he was bathing or eating and when his attention was captured&#46; The patient again received methylprednisolone by IV and cyclophosphamide&#44; with subsequent maintenance therapy of azathioprine with partial response&#46; Gait disorders persisted which led to the patient becoming bed-ridden&#44; with dysphasia and a continuation of the inappropriate laughter&#46; Treatment was changed to anti-TNF&#44; but shortly after initiation of treatment the patient died from respiratory infectious complications&#46;</p></span><span id="sec0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0010">Case 2</span><p id="par0030" class="elsevierStylePara elsevierViewall">Male&#44; 39 years of age&#44; previously healthy&#46; He presented with a 4-month history of constitutional symptoms of low-grade fever&#44; polyarthritis&#44; oral and genital ulcers&#46; Beh&#231;et&#39;s disease was diagnosed for which the patient was treated with glucocorticoids and cyclophosphamide&#46; During follow-up&#44; his family members&#44; and the patient himself commented upon changes to his behaviour characterized by the appearance of involuntary and irrepressible laughter&#44; even in serious situations or without any stimuli&#46; The most dramatic event presented during the funeral of his daughter aged 12 where&#44; despite being grief stricken&#44; the patient burst into uncontrollable laughter and preferred to vacate the premises&#46; The patient continued with immunosuppressant treatment based on methotrexate&#44; with remission of symptoms&#46;</p><p id="par0035" class="elsevierStylePara elsevierViewall">Beh&#231;et&#39;s disease&#44; a chronic multisystemic inflammatory disease&#44; is included in the Chapel Hill classification as vasculitis of blood vessels of varying size&#46;<a class="elsevierStyleCrossRef" href="#bib0080"><span class="elsevierStyleSup">8</span></a> An extremely high percentage of patients with this vasculitis&#44; ranging from 5&#37; to 50&#37;&#44; may present with neuropsychiatric symptoms&#46; There are two suggested mechanisms to explain the clinical symptoms&#58; parenchymatose inflammatory lesions &#40;with involvement of the brain stem&#44; mesodiencephalic union&#44; cerebellar and cerebral hemispheres&#41; and the thrombotic phenomenon&#46; Pseudobulbar paralysis syndrome has been previously reported in this disease&#44; but uncontrollable laughter being predominant as a symptom is extremely rare&#46; This finding presented in both patients&#46; In the first case there was mesodiencephalic involvement despite immunosuppressant treatment and evolution was not favourable&#46;</p><p id="par0040" class="elsevierStylePara elsevierViewall">Different disorders have received eponyms from other&#44; non-medical areas&#46; One such known case&#44; the Pickwickian syndrome&#44; was established by Charles Dickens in his novel <span class="elsevierStyleItalic">The Posthumous Papers of the Pickwick Club</span> published in 1837&#46; Might we then speak of the &#8220;Joker syndrome&#8221; in the case of the inappropriate laughter in our patients with this neuropsychiatric expression related to Behcet&#39;s disease&#63;</p></span></span>"
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Letter to the Editor
Is “Joker” syndrome a distinctive characteristic of neuro-Behçet's disease
¿Síndrome del Guasón, característica distintiva de neuro-Behçet?
Gerardo Toantiu Jaimes-Piñona, Ildefonso Rodrìguez-Leyvab,c, Carlos Abud Mendozab,c,
Corresponding author
cabudm@hotmail.com

Corresponding author.
a Hospital General número 50, Instituto Mexicano del Seguro Social (IMSS), San Luis Potosí, Mexico
b Facultad de Medicina, Universidad Autónoma San Luis Potosí (UASLP), San Luis Potosí, Mexico
c Hospital Central «Dr. Ignacio Morones Prieto», San Luis Potosí, Mexico
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    "textoCompleto" => "<span class="elsevierStyleSections"><p id="par0005" class="elsevierStylePara elsevierViewall">Laughter is an inherent and essential part of human behaviour&#46; Its impact throughout our culture may be observed from the texts of the great civilisations&#44; from classical Greece&#44; to today&#39;s popular culture&#46; The beneficial impact of laughter in medicine has been widely demonstrated in&#44; for example&#44; cardiovascular health&#44; behaviour patterns and learning&#46;<a class="elsevierStyleCrossRefs" href="#bib0045"><span class="elsevierStyleSup">1&#8211;4</span></a></p><p id="par0010" class="elsevierStylePara elsevierViewall">On the other end of the laughter type classification spectrum is abnormal laughter&#46; This is defined as something which occurs without any previous stimulus and which is of inappropriate intensity&#46; Similarly&#44; to the first type of laughter&#44; there are many references to abnormal laughter&#46; One recent reference is the <span class="elsevierStyleItalic">Joker</span> antihero&#44; whose distinctive trait is his scandalous&#44; abnormal&#44; and on occasions&#44; malevolent laughter&#46;</p><p id="par0015" class="elsevierStylePara elsevierViewall">The aetiology of abnormal laughter&#44; which is a component of pseudobulbar syndrome&#44; is extensive and includes neurodegenerative&#44; tumoral&#44; traumatic and even vascular diseases&#46; In Beh&#231;et&#39;s disease&#44; primary systemic vasculitis of variably sized blood vessels and involvement of the central nervous system at disease onset is rare and occurs less frequently than that of other organs&#46; Inappropriate laughter as part of the neuropsychiatric symptoms has been infrequently reported&#46;<a class="elsevierStyleCrossRefs" href="#bib0065"><span class="elsevierStyleSup">5&#8211;7</span></a></p><p id="par0020" class="elsevierStylePara elsevierViewall">We report 2 cases of patients with Beh&#231;et&#39;s disease and with &#8220;joker syndrome&#8221; traits&#46;</p><span id="sec0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0005">Case 1</span><p id="par0025" class="elsevierStylePara elsevierViewall">Male&#44; 23 years of age&#44; previously healthy&#44; with 6-month onset of constitutional symptoms&#44; ataxia&#44; dysmetria and dysarthria&#46; Three months later additional symptoms of polyarthritis&#44; pustular dermatosis&#44; and oral and genital ulcers presented&#46; Diagnosis ruled out infectious aetiologies and confirmed the existence of retinal vasculitis in fluorescein angiography and in magnetic resonance imaging&#44; which showed predominantly mesencephalic inflammatory lesions&#46; Beh&#231;et&#39;s disease was considered and was treated with methylprednisolone and cyclophosphamide with favourable response&#46; During the course of the disease the patient again presented with ataxia&#44; dysphagia&#44; and dysarthria&#44; accompanied by episodes of intense laughter&#46; His mother reported that the laughter even occurred in situations such as when he was bathing or eating and when his attention was captured&#46; The patient again received methylprednisolone by IV and cyclophosphamide&#44; with subsequent maintenance therapy of azathioprine with partial response&#46; Gait disorders persisted which led to the patient becoming bed-ridden&#44; with dysphasia and a continuation of the inappropriate laughter&#46; Treatment was changed to anti-TNF&#44; but shortly after initiation of treatment the patient died from respiratory infectious complications&#46;</p></span><span id="sec0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0010">Case 2</span><p id="par0030" class="elsevierStylePara elsevierViewall">Male&#44; 39 years of age&#44; previously healthy&#46; He presented with a 4-month history of constitutional symptoms of low-grade fever&#44; polyarthritis&#44; oral and genital ulcers&#46; Beh&#231;et&#39;s disease was diagnosed for which the patient was treated with glucocorticoids and cyclophosphamide&#46; During follow-up&#44; his family members&#44; and the patient himself commented upon changes to his behaviour characterized by the appearance of involuntary and irrepressible laughter&#44; even in serious situations or without any stimuli&#46; The most dramatic event presented during the funeral of his daughter aged 12 where&#44; despite being grief stricken&#44; the patient burst into uncontrollable laughter and preferred to vacate the premises&#46; The patient continued with immunosuppressant treatment based on methotrexate&#44; with remission of symptoms&#46;</p><p id="par0035" class="elsevierStylePara elsevierViewall">Beh&#231;et&#39;s disease&#44; a chronic multisystemic inflammatory disease&#44; is included in the Chapel Hill classification as vasculitis of blood vessels of varying size&#46;<a class="elsevierStyleCrossRef" href="#bib0080"><span class="elsevierStyleSup">8</span></a> An extremely high percentage of patients with this vasculitis&#44; ranging from 5&#37; to 50&#37;&#44; may present with neuropsychiatric symptoms&#46; There are two suggested mechanisms to explain the clinical symptoms&#58; parenchymatose inflammatory lesions &#40;with involvement of the brain stem&#44; mesodiencephalic union&#44; cerebellar and cerebral hemispheres&#41; and the thrombotic phenomenon&#46; Pseudobulbar paralysis syndrome has been previously reported in this disease&#44; but uncontrollable laughter being predominant as a symptom is extremely rare&#46; This finding presented in both patients&#46; In the first case there was mesodiencephalic involvement despite immunosuppressant treatment and evolution was not favourable&#46;</p><p id="par0040" class="elsevierStylePara elsevierViewall">Different disorders have received eponyms from other&#44; non-medical areas&#46; One such known case&#44; the Pickwickian syndrome&#44; was established by Charles Dickens in his novel <span class="elsevierStyleItalic">The Posthumous Papers of the Pickwick Club</span> published in 1837&#46; Might we then speak of the &#8220;Joker syndrome&#8221; in the case of the inappropriate laughter in our patients with this neuropsychiatric expression related to Behcet&#39;s disease&#63;</p></span></span>"
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        "nota" => "<p class="elsevierStyleNotepara" id="npar0005">Please cite this article as&#58; Toantiu Jaimes-Pi&#241;on G&#44; Rodr&#236;guez-Leyva I&#44; Abud Mendoza C&#46; &#191;S&#237;ndrome del Guas&#243;n&#44; caracter&#237;stica distintiva de neuro-Beh&#231;et&#63; Reumatol Clin&#46; 2021&#59;17&#58;368&#8211;369&#46;</p>"
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Idiomas
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