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Letter to the Editor
Migraine triggered by laughing as a form of presentation of a Chiari Type I malformation
Cefalea desencadenada por la risa como forma de presentación de una malformación de CHIARI tipo I
P.E. Jiménez Caballero
Corresponding author
pjimenez1010j@yahoo.es

Corresponding author.
, M.L. Calle Escobar, J.C. Portilla Cuenca, I. Casado Naranjo
Sección de Neurología, Hospital San Pedro de Alcántara, Cáceres, Spain
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    "textoCompleto" => "<span class="elsevierStyleSections"><p id="par0005" class="elsevierStylePara elsevierViewall">Chiari malformation type I &#40;CM1&#41; is defined as the extrusion of the cerebellar tonsils through the foramen magnum and into the spinal canal&#46; It can be confirmed by a radiograph showing descent of brain tissue 5<span class="elsevierStyleHsp" style=""></span>mm or more&#44; lending a pointed appearance to the cerebellar tonsils and decreasing the subarachnoid space at the craniocervical junction&#46;<a class="elsevierStyleCrossRef" href="#bib0005"><span class="elsevierStyleSup">1</span></a> The clinical manifestations of CM-I are associated with direct compression of the brainstem and cervical spinal cord&#44; or with changes in cerebrospinal fluid&#46;</p><p id="par0010" class="elsevierStylePara elsevierViewall">A 38-year-old man with no relevant medical history described headaches occurring throughout the preceding year&#46; Pain was dull and moderately intense &#40;VAS score 6&#41; and located in the occipital region radiating to the frontal region&#46; Headache was triggered by laughing&#44; but not by Valsalva manoeuvres or physical exercise&#46; Episodes lasted 10 to 30<span class="elsevierStyleHsp" style=""></span>minutes and were not accompanied by focal neurological signs&#46; In the preceding month&#44; pain had grown more intense &#40;VAS 8&#41; and was triggered not only by laughing&#44; but also by minimal exertion and Valsalva manoeuvres such as coughing or defecating&#46; Lateral and rotational movements of the head also caused headache episodes&#46; The general examination was normal&#59; a neurological examination revealed left-beating horizontal nystagmus with no other relevant findings&#46;</p><p id="par0015" class="elsevierStylePara elsevierViewall">Brain and cervical MRI &#40;<a class="elsevierStyleCrossRef" href="#fig0005">Fig&#46; 1</a>&#41; showed that cerebellar tonsils had descended 16<span class="elsevierStyleHsp" style=""></span>mm below the foramen magnum&#44; forming a point&#46; The medulla had taken on an angled shape and the subarachnoid space at the craniocervical junction was reduced&#46; Signs were consistent with a diagnosis of type I Chiari malformation&#46;</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><p id="par0020" class="elsevierStylePara elsevierViewall">The patient was initially treated with 600<span class="elsevierStyleHsp" style=""></span>mg ibuprofen every 8<span class="elsevierStyleHsp" style=""></span>hours but did not respond&#46; Treatment was changed to 25<span class="elsevierStyleHsp" style=""></span>mg indometacin every 8<span class="elsevierStyleHsp" style=""></span>hours&#44; still with no response&#46; As the patient&#39;s headaches were incapacitating&#44; he underwent decompressive occipital surgery associated with C1 laminectomy&#46; The headaches&#44; vertigo&#44; and nystagmus all resolved and have not returned in 2 years of follow-up&#46;</p><p id="par0025" class="elsevierStylePara elsevierViewall">Our patient&#39;s pain met criteria for headaches caused by type 1 Chiari malformation according to the second edition of the International Headache Classification &#40;<a class="elsevierStyleCrossRef" href="#tbl0005">Table 1</a>&#41;&#46;<a class="elsevierStyleCrossRef" href="#bib0010"><span class="elsevierStyleSup">2</span></a> There have been cases in which headaches triggered by laughing&#44;<a class="elsevierStyleCrossRef" href="#bib0015"><span class="elsevierStyleSup">3</span></a> one of which was prolonged&#44; signalled the presence of CM1&#46;<a class="elsevierStyleCrossRef" href="#bib0020"><span class="elsevierStyleSup">4</span></a> In another published case of a patient with CM1 and IV-ventricle ependymoma without hydrocephalus&#44; headaches triggered by laughing resolved once the tumour had been removed&#46;<a class="elsevierStyleCrossRef" href="#bib0025"><span class="elsevierStyleSup">5</span></a> Although the phenomenon of headache caused by laughing is exceptional&#44; and would rarely appear as the first manifestation of CM1&#44; this did occur in our patient&#46;</p><elsevierMultimedia ident="tbl0005"></elsevierMultimedia><p id="par0030" class="elsevierStylePara elsevierViewall">Laughter constitutes a Valsalva manoeuvre&#44; and&#44; like coughing&#44; it raises intracranial pressure&#46; Coughing and other activities involving Valsalva manoeuvres may cause primary cough headache&#46; They may also trigger a headache in the context of CM1&#46;<a class="elsevierStyleCrossRefs" href="#bib0030"><span class="elsevierStyleSup">6&#44;7</span></a> The difference between the headaches is that primary cough headache has an older age at onset &#40;&#62;60 years&#41; and a shorter duration &#40;seconds rather than minutes&#41;&#46;<a class="elsevierStyleCrossRef" href="#bib0040"><span class="elsevierStyleSup">8</span></a> This trend can be seen in our patient&#59; since he is a young adult with headaches lasting several minutes&#44; symptoms are consistent with CM1&#46; The pathophysiology of headache secondary to CM1 is uncertain&#46; The postulated mechanism is a temporary increase in the pressure difference between the intracranial and intramedullary compartments&#46; This would further force the cerebellar tonsils against the foramen magnum&#44; causing pain due to traction on pain-sensitive structures&#46;<a class="elsevierStyleCrossRef" href="#bib0045"><span class="elsevierStyleSup">9</span></a> In some case series&#44; the resolution rate for headaches after surgery reaches 88&#37;&#46;<a class="elsevierStyleCrossRef" href="#bib0050"><span class="elsevierStyleSup">10</span></a> Headache triggered by laughing may be primary&#44; but it may also indicate diseases of the posterior fossa&#46; Age at onset&#44; pain duration&#44; and the presence of neurological signs and symptoms may point to a structural cause&#46;</p><p id="par0035" class="elsevierStylePara elsevierViewall">Headache secondary to CM1 is often described as being similar to primary cough headache except that it may last longer &#40;minutes instead of seconds&#41;&#46; One theory holds that primary cough headache responds better than secondary headache to indometacin&#46; However&#44; distinguishing between primary and secondary headaches may be difficult&#46; A brain MRI is not only useful&#44; but absolutely necessary in cases of headache provoked by the Valsalva manoeuvre&#46; Headache is the most frequent symptom of CM1&#44; but patients may have localised vestibulo-ocular disorders &#40;in 74&#37; of cases&#41;&#59; cranial nerve&#44; brainstem&#44; or cerebellar disorders &#40;in 50&#37;&#41;&#59; and&#47;or cervical spinal cord disorders indicating syringomyelia &#40;in 66&#37;&#41;&#46; These symptoms are the most indicative of a secondary headache&#46;</p><p id="par0040" class="elsevierStylePara elsevierViewall">In addition to being associated with better surgical results&#44; early diagnosis and early treatment can prevent development of more critical cerebellar&#44; brainstem&#44; or cervical symptoms&#46;</p></span>"
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      0 => array:2 [
        "etiqueta" => "&#9734;"
        "nota" => "<p class="elsevierStyleNotepara" id="npar0005">Please cite this article as&#58; Jim&#233;nez Caballero PE&#44; Calle Escobar ML&#44; Portilla Cuenca JC&#44; Casado Naranjo I&#46; Cefalea desencadenada por la risa como forma de presentaci&#243;n de una malformaci&#243;n de CHIARI tipo I&#46; Neurolog&#237;a&#46; 2013&#59;28&#58;446&#8211;447&#46;</p>"
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          "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">Sagittal&#44; fat-suppressed cervical MR image revealing ectopia of the cerebellar tonsils 16<span class="elsevierStyleHsp" style=""></span>mm below the foramen magnum&#44; angled medulla&#44; and decreased subarachnoid space at the craniocervical junction&#46;</p>"
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                  <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="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t"><span class="elsevierStyleBold">A&#46; Headache fulfilling at least 1 of the following conditions as well as condition D&#58;</span>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
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                  \t\t\t\t"><span class="elsevierStyleHsp" style=""></span><span class="elsevierStyleItalic">1&#46; Triggered by cough and&#47;or Valsalva manoeuvres</span>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t"><span class="elsevierStyleHsp" style=""></span><span class="elsevierStyleItalic">2&#46; Prolonged occipital and&#47;or suboccipital headache &#40;lasting hours to days&#41;</span>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
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                  \t\t\t\t"><span class="elsevierStyleHsp" style=""></span><span class="elsevierStyleItalic">3&#46; Associated with signs or symptoms of cerebellar&#44; brainstem&#44; or cervical spinal cord dysfunction</span>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t"><span class="elsevierStyleVsp" style="height:0.5px"></span>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t"><span class="elsevierStyleBold">B&#46; Herniation of cerebellar tonsils meeting one of the following criteria as shown by craniocervical MRI&#58;</span>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t"><span class="elsevierStyleHsp" style=""></span><span class="elsevierStyleItalic">1&#46; Caudal extrusion of the cerebellar tonsils measuring 5</span><span class="elsevierStyleHsp" style=""></span><span class="elsevierStyleItalic">mm or more</span>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t"><span class="elsevierStyleHsp" style=""></span><span class="elsevierStyleItalic">2&#46; Caudal extrusion of the cerebellar tonsils measuring 3</span><span class="elsevierStyleHsp" style=""></span><span class="elsevierStyleItalic">mm or more&#44; plus at least 1 of the following indicators of overcrowding of the subarachnoid space at the craniocervical junction&#58;</span>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
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                  \t\t\t\t"><span class="elsevierStyleHsp" style=""></span><span class="elsevierStyleHsp" style=""></span>&#40;a&#41; Posterior and lateral compression of cerebrospinal fluid spaces&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t"><span class="elsevierStyleHsp" style=""></span><span class="elsevierStyleHsp" style=""></span>&#40;b&#41; Reduced height of the supraocciput&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t"><span class="elsevierStyleHsp" style=""></span><span class="elsevierStyleHsp" style=""></span>&#40;c&#41; Increased tentorial angle&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
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                  \t\t\t\t"><span class="elsevierStyleHsp" style=""></span><span class="elsevierStyleHsp" style=""></span>&#40;d&#41; Distortion of the medulla&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t"><span class="elsevierStyleVsp" style="height:0.5px"></span>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
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                  \t\t\t\t"><span class="elsevierStyleBold">C&#46; Evidence of dysfunction among posterior fossa structures&#44; based on at least 2 of the following criteria&#58;</span>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
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                  \t\t\t\t"><span class="elsevierStyleHsp" style=""></span><span class="elsevierStyleItalic">1&#46; Otoneurological signs and&#47;or symptoms &#40;for example&#44; dizziness&#44; poor balance&#44; perceived changes in ear pressure&#44; hearing loss or hyperacusis&#44; vertigo&#44; vertical nystagmus&#44; or oscillopsia&#41;</span>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
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                  \t\t\t\t"><span class="elsevierStyleHsp" style=""></span><span class="elsevierStyleItalic">2&#46; Transient visual symptoms &#40;scintillating photopsia&#44; blurry vision&#44; diplopia&#44; or transient visual field defect&#41;</span>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t"><span class="elsevierStyleHsp" style=""></span><span class="elsevierStyleItalic">3&#46; Discovery of clinical symptoms originating in the spinal cord&#44; brainstem&#44; or nerves in the base of the skull&#59; ataxia or dysmetria</span>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
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                  \t\t\t\t"><span class="elsevierStyleVsp" style="height:0.5px"></span>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t"><span class="elsevierStyleBold">D&#46; Headache resolves within 3 months of successful treatment of the Chiari malformation</span>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr></tbody></table>
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          "en" => "<p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">Diagnostic criteria for headaches caused by type I Chiari malformation&#46;</p>"
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      "titulo" => "References"
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ISSN: 21735808
Original language: English
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