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Letter to the Editor
Asymptomatic colpocephaly and partial agenesis of corpus callosum
Colpocefalia y agenesia parcial de cuerpo calloso asintomáticos
E.L. Bartoloméa, J.C. Cotturab, R. Britos Fresciac, R.O. Domínguezd,
Corresponding author
a Departamento de Neurología, Hospital Sirio Libanés, Facultad de Medicina de la Universidad de Buenos Aires, Buenos Aires, Argentina
b Departamento de Medicina Interna, Hospital Sirio Libanés, Buenos Aires, Argentina
c Departamento de Medicina Interna, Hospital Sirio Libanés, Buenos Aires, Argentina
d Departamento de Neurología, Hospital Sirio Libanés, Facultad de Medicina de la Universidad de Buenos Aires, Buenos Aires, Argentina
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          "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">Head MRI scan of case 1&#46; a&#41; Axial FLAIR sequence&#46; Frontal ventricles &#40;dotted arrow&#41; are small&#44; and the frontal parenchyma features small hyperintense images &#40;arrow&#41; that could be associated with arterial hypertension&#46; The third ventricle and occipital horns are dilated &#40;CC&#41;&#46; b&#41; Axial FLAIR sequence&#46; The third ventricle appears dilated &#40;arrow&#41; and the upper part of the corpus callosum is very thin&#46; c&#41; T1-weighted sagittal slice&#58; the genu and trunk of the corpus callosum &#40;arrow&#41; seem faint&#46; The fourth ventricle is normal-sized&#46; d&#41; T2-weighted axial slice&#46; Frontal sulci are much smaller than cortical sulci on the posterior part of the brain&#46;</p>"
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    "textoCompleto" => "<span class="elsevierStyleSections"><p id="par0005" class="elsevierStylePara elsevierViewall">Colpocephaly &#40;CC&#41; can be observed in radiological findings from brain computed tomography &#40;BCT&#41; and magnetic resonance imaging &#40;MRI&#41;&#46; These images show dilated lateral ventricles&#44; specifically in the occipital and temporal horns&#44; and the third ventricle&#46; Frontal horns retain their normal size&#46; This type of hydrocephalus of the posterior half of the brain is a congenital malformation&#46; It can be associated with myelocele&#44; microgyria&#44; cerebellar atrophy&#44; total or partial absence of the corpus callosum&#44; and other structural anomalies&#46; Neurological manifestations occur mainly in children&#44; and include cognitive and motor developmental delay&#44; and epileptic seizures&#46; Diagnosis in adult patients who have learned to perform activities of daily living normally is very infrequent&#46; Imaging studies will show malformations&#44; and differential diagnosis is required to rule out obstructive and non-obstructive ventriculomegaly&#46;<a class="elsevierStyleCrossRefs" href="#bib0055"><span class="elsevierStyleSup">1&#8211;5</span></a> Case 1 is a 67-year-old man who presented sudden loss of consciousness with no seizures or loss of sphincter control&#46; He regained consciousness&#44; cognitive functions&#44; and motor functions after 1 or 2<span class="elsevierStyleHsp" style=""></span>minutes&#46; According to his personal history&#44; his developmental progression was normal and he had been working as administrative clerk until his retirement&#46; Ten years before the event&#44; he was diagnosed with arterial hypertension and had since been treated with enalapril dosed at 5<span class="elsevierStyleHsp" style=""></span>mg twice a day&#46; Clinical&#44; cardiological&#44; and neurological examinations did not show any abnormalities 2<span class="elsevierStyleHsp" style=""></span>hours after the event&#44; and neither did the electrocardiogram and blood test&#46; The BCT performed in the emergency department revealed very pronounced posterior ventricular dilatation&#46; The patient was then admitted for subsequent studies&#46; A head MRI revealed CC and partial agenesis of the corpus callosum&#46; The electroencephalogram did not show any abnormalities&#44; thus ruling out adult onset of epileptic seizures&#46;<a class="elsevierStyleCrossRef" href="#bib0080"><span class="elsevierStyleSup">6</span></a> On the following day&#44; cognitive tests and exploration of ideomotor&#44; ideational&#44; and limb-kinetic praxis showed normal results&#44; and no interhemispheric disconnection syndrome could be detected&#46;<a class="elsevierStyleCrossRefs" href="#bib0085"><span class="elsevierStyleSup">7&#44;8</span></a> A lumbar puncture did not reveal any inflammatory or infectious processes&#46; Given the normal structure of frontal ventricles&#44; we ruled out normal pressure hydrocephalus &#40;gait&#44; sphincter control&#44; and cognition were unaltered&#41;&#46; The transient nature of the clinical manifestation led us to consider syncope as the probable diagnosis&#44; regardless of any brain malformations&#46; <a class="elsevierStyleCrossRef" href="#fig0005">Fig&#46; 1</a> shows findings of CC and agenesis of the corpus callosum&#46; Case 2 is a 60-year-old man admitted to our hospital due to respiratory tract infection&#46; He presented confusional syndrome during a hyperthermic episode&#46; Throughout the patient&#39;s personal history&#44; he had been independent for activities of daily living and was able to work satisfactorily as keeper and doorman in a condominium&#46; A neurological examination of motor function and cranial nerves yielded no anomalous results&#46; Cardiological examination and blood count results were also within normal limits&#46; No inflammatory activity was detected in CSF extracted by lumbar puncture&#46; A head MRI revealed CC and partial agenesis of the corpus callosum &#40;<a class="elsevierStyleCrossRef" href="#fig0010">Fig&#46; 2</a>&#41;&#46; Once hyperthermia and the confusional syndrome had resolved&#44; cognitive and praxis assessments yielded normal results&#44; as in case 1&#46; The patient was discharged after one week of hospitalisation&#46; He displayed normal gait and sphincter control&#44; which ruled out normal pressure hydrocephalus&#46; As in the other case&#44; our clinical assessment did not link cerebral malformation to the confusional syndrome&#46; In both cases&#44; the posterior to anterior ratio described by Noorani et al&#46;<a class="elsevierStyleCrossRef" href="#bib0070"><span class="elsevierStyleSup">4</span></a> was higher than 3 &#40;CC&#41;&#44; indicating a larger diameter of occipital ventricles than of frontal ventricles&#46; In case 1&#44; frontal sulci were found to be strikingly smaller than those in the posterior half of the brain&#44; but no frontal microgyria could be diagnosed&#46; An examination of these cases admitted in 2013 found no other similar cases in the hospital&#39;s records in the preceding 30 years&#46; Whipple disease was considered in both cases since it can cause symmetric or asymmetric hydrocephalus&#46; However&#44; no history of this disease was reported for either patient&#44; and CSF analysis showed normal results&#46;<a class="elsevierStyleCrossRef" href="#bib0095"><span class="elsevierStyleSup">9</span></a> A recently published study reported the case of an 88-year-old patient diagnosed with agenesis of the corpus callosum and CC&#46; The patient had been asymptomatic until that moment&#44; and the absence of clinical symptoms related to his malformations was thought to be due to neuroplasticity&#46;<a class="elsevierStyleCrossRef" href="#bib0100"><span class="elsevierStyleSup">10</span></a> CC is a congenital malformation which causes enlargement of the posterior half of the brain ventricles&#59; it is frequently associated with total or partial agenesis of the corpus callosum&#46; Manifestations are heterogeneous during childhood&#44; but CC can also manifest incidentally in asymptomatic adults&#46; Differential diagnosis to rule out normal pressure hydrocephalus should be performed before proceeding to surgical treatment&#44; since the marked difference in diameter between the occipital and frontal ventricles will help identify the congenital malformation&#46;</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><elsevierMultimedia ident="fig0010"></elsevierMultimedia><span id="sec0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0005">Funding</span><p id="par0010" class="elsevierStylePara elsevierViewall">This study has not received funding from any sources&#46; All authors are aware of the manuscript content and have approved it for publication&#46;</p></span></span>"
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          "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">Head MRI scan of case 1&#46; a&#41; Axial FLAIR sequence&#46; Frontal ventricles &#40;dotted arrow&#41; are small&#44; and the frontal parenchyma features small hyperintense images &#40;arrow&#41; that could be associated with arterial hypertension&#46; The third ventricle and occipital horns are dilated &#40;CC&#41;&#46; b&#41; Axial FLAIR sequence&#46; The third ventricle appears dilated &#40;arrow&#41; and the upper part of the corpus callosum is very thin&#46; c&#41; T1-weighted sagittal slice&#58; the genu and trunk of the corpus callosum &#40;arrow&#41; seem faint&#46; The fourth ventricle is normal-sized&#46; d&#41; T2-weighted axial slice&#46; Frontal sulci are much smaller than cortical sulci on the posterior part of the brain&#46;</p>"
        ]
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          "en" => "<p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">Head MRI scan of case 2&#46; a&#41; T2-weighted sagittal sequence&#46; We can observe a great difference in size between the normal anterior horn of the lateral ventricle and the very enlarged portion &#40;from the arrow onwards&#41; in the occipital region&#46; b&#41; T1-weighted axial slice&#46; View of the third ventricle with an arrow indicating the thin genu of the corpus callosum&#46; c&#41; T1-weighted coronal slice&#46; The splenium of the corpus callosum &#40;arrow&#41; is shown crossing the midline at the posterior fossa level &#40;dotted arrow over the cerebellar tentorium&#41;&#46; d&#41; T2-weighted sagittal slice&#58; Frontal and occipital cortical sulci show similar degrees of dilatation&#44; unlike in case 1&#46;</p>"
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