Letter to the Editor
Agenesis of posterior inferior cerebellar arteries in an asymptomatic adult with Dandy–Walker malformation
Agenesia de arterias cerebelosas postero-inferiores en adulto asintomático con malformación de Dandy Walker
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Domínguez, S.E. González, A. Saenz Valiente, E. Pinkala" "autores" => array:4 [ 0 => array:4 [ "nombre" => "R.O." "apellidos" => "Domínguez" "email" => array:1 [ 0 => "neuroldominguez@yahoo.com.ar" ] "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" => "S.E." "apellidos" => "González" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] ] ] 2 => array:3 [ "nombre" => "A." "apellidos" => "Saenz Valiente" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] ] ] 3 => array:3 [ "nombre" => "E." "apellidos" => "Pinkala" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">b</span>" "identificador" => "aff0010" ] ] ] ] "afiliaciones" => array:2 [ 0 => array:3 [ "entidad" => "Departamento de Neurología, Hospital Sirio Libanés, Buenos Aires, Argentina" "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] 1 => array:3 [ "entidad" => "Departamento de Medicina Interna, Hospital Sirio Libanés, Buenos Aires, Argentina" "etiqueta" => "<span class="elsevierStyleSup">b</span>" "identificador" => "aff0010" ] ] "correspondencia" => array:1 [ 0 => array:3 [ "identificador" => "cor0005" "etiqueta" => "⁎" "correspondencia" => "Corresponding author." ] ] ] ] "titulosAlternativos" => array:1 [ "es" => array:1 [ "titulo" => "Agenesia de arterias cerebelosas postero-inferiores en adulto asintomático con malformación de Dandy Walker" ] ] "resumenGrafico" => array:2 [ "original" => 0 "multimedia" => array:7 [ "identificador" => "fig0010" "etiqueta" => "Figure 2" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr2.jpeg" "Alto" => 795 "Ancho" => 1299 "Tamanyo" => 135959 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">MR angiography images in the posterior fossa (see description in text).</p>" ] ] ] "textoCompleto" => "<span class="elsevierStyleSections"><p id="par0005" class="elsevierStylePara elsevierViewall">Dandy–Walker malformation (DWM) is an uncommon condition characterised by agenesis or hypoplasia of the cerebellar vermis, the cerebellar hemispheres, and cystic dilation of the fourth ventricle. There are very few records of asymptomatic adults with DWM. Hypotonia and motor and mental developmental delay, together with supratentorial hydrocephalus, malformation of the corpus callosum, and macrocephaly, appear in children.<a class="elsevierStyleCrossRefs" href="#bib0005"><span class="elsevierStyleSup">1,2</span></a> This case history describes arterial agenesis associated with abnormal brain embryogenesis.</p><p id="par0010" class="elsevierStylePara elsevierViewall">A 61-year-old female visited due to syncope. She had been taking medication for high blood pressure for 5<span class="elsevierStyleHsp" style=""></span>years (25<span class="elsevierStyleHsp" style=""></span>mg/day atenolol). Her medical history reported no motor or cognitive abnormalities. She was able to work and perform daily living activities normally. Blood tests, a 24-hour ECG, 24-hour blood pressure monitor test, a tilt test to measure response in terms of heart rate and blood pressure, and the EEG all delivered normal results. Findings from the neurological and cognitive tests were normal. Brain neuroimaging studies were performed. In <a class="elsevierStyleCrossRef" href="#fig0005">Fig. 1</a>, the sagittal brain MR image on the A side showed DWM. The supratentorial ventricles, cerebral cortex, corpus callosum, brainstem, and mesencephalic duct all appeared to be normal. On the medial slice of side A, Arrow 1 points to the entire length of the basilar artery. The cerebellar vermis is absent, and we see a small wing-shaped part of the upper cerebellum. In a paramedial slice on side B, arrows 2 and 3 indicate the anterior, superior, and inferior cerebellar parenchyma, respectively.</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><p id="par0015" class="elsevierStylePara elsevierViewall">Angio-RM revealed that neck and supratentorial arteries were normal. Side C of <a class="elsevierStyleCrossRef" href="#fig0010">Fig. 2</a> shows a frontal view, and side D, an oblique view, of the arteries of the posterior fossa. Arrow 1, along the final segment of the vertebral arteries, shows the lack of posterior inferior cerebellar arteries (PICA). Arrow 2 indicates the basilar trunk; Arrow 3 shows the posterior cerebral terminal branches; Arrow 4 shows the superior cerebellar colateral branches; and Arrow 5, the anterior inferior cerebellar arteries (AICA).</p><elsevierMultimedia ident="fig0010"></elsevierMultimedia><p id="par0020" class="elsevierStylePara elsevierViewall">One year after the syncopic episode, the patient had not experienced symptom recurrence, and was able to carry out daily activities normally.</p><p id="par0025" class="elsevierStylePara elsevierViewall">Descriptions of DWM state that children hardly ever reach adulthood without clinical neurological signs of the syndrome. There are fewer than a dozen published accounts of asymptomatic adults, most of which describe females.</p><p id="par0030" class="elsevierStylePara elsevierViewall">One article provides a case history of a woman with asymptomatic DWM who died of breast cancer at the age of 52. This was the first published anatomical pathology study in asymptomatic DWM. Macroscopic examination revealed hypoplasia of the vermis and both cerebellar hemispheres, cystic dilation of the fourth ventricle, and no supratentorial hydrocephalus. The external and medial walls of the cyst were formed by arachnoidal cells and internal ependymal cells. The cerebellar cortex had only 2 small areas with dysplasia. The cerebral cortex showed no neural losses, ectopias, vascular lesions, or degenerative lesions.<a class="elsevierStyleCrossRef" href="#bib0015"><span class="elsevierStyleSup">3</span></a> In our case, the lack of other structural brain lesions concomitant with DWM could explain the patient's lack of symptoms in adulthood.</p><p id="par0035" class="elsevierStylePara elsevierViewall">Other published articles on DWM present 4 women and 1 man aged 30–65<span class="elsevierStyleHsp" style=""></span>years with a normal ability to engage in activities of daily life who rapidly developed symptoms compatible with vascular lesions, including headache, nausea, vomiting, ataxic gait, diplopia, and reduced awareness.<a class="elsevierStyleCrossRefs" href="#bib0020"><span class="elsevierStyleSup">4–8</span></a></p><p id="par0040" class="elsevierStylePara elsevierViewall">As we are well aware that stroke complications may arise in DWM, we ordered an MR angiography study to assess large cervical and intracranial arteries. The most relevant finding was agenesis in both PICAs, and this coincided with the absence of the bilateral posterior and inferior areas of the cerebellum. Cerebellar parenchyma was present in the AICA and superior cerebellar arterial territories. Pascual-Castroviejo's study on cutaneous vascular malformations and hemangiomas with other concomitant abnormalities mentions DWM in the context of variations in the arteries of the brain.<a class="elsevierStyleCrossRef" href="#bib0045"><span class="elsevierStyleSup">9</span></a></p><p id="par0045" class="elsevierStylePara elsevierViewall">We wished to highlight the well-illustrated PICA agenesis in our patient. No published articles report bilateral absence of these arteries, but the abnormality may be linked to the same embryogenic defect found in regions of the cerebellum. It is very surprising that the patient has reached an advanced age without displaying specific symptoms.</p></span>" "pdfFichero" => "main.pdf" "tienePdf" => true "NotaPie" => array:1 [ 0 => array:2 [ "etiqueta" => "☆" "nota" => "<p class="elsevierStyleNotepara">Please cite this article as: Domínguez RO, et al. Agenesia de arterias cerebelosas postero-inferiores en adulto asintomático con malformación de Dandy Walker. Neurología. 2012; 27:445–6.</p>" ] ] "multimedia" => array:2 [ 0 => array:7 [ "identificador" => "fig0005" "etiqueta" => "Figure 1" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr1.jpeg" "Alto" => 698 "Ancho" => 1401 "Tamanyo" => 168301 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">MR images in a sagittal slice (see description in text).</p>" ] ] 1 => array:7 [ "identificador" => "fig0010" "etiqueta" => "Figure 2" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr2.jpeg" "Alto" => 795 "Ancho" => 1299 "Tamanyo" => 135959 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">MR angiography images in the posterior fossa (see description in text).</p>" ] ] ] "bibliografia" => array:2 [ "titulo" => "References" "seccion" => array:1 [ 0 => array:2 [ "identificador" => "bibs0005" "bibliografiaReferencia" => array:9 [ 0 => array:3 [ "identificador" => "bib0005" "etiqueta" => "1" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "The Dandy Walker malformation: a review of 40 cases" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:5 [ 0 => "J.F. 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| Year/Month | Html | Total | |
|---|---|---|---|
| 2024 November | 3 | 1 | 4 |
| 2024 October | 19 | 5 | 24 |
| 2024 September | 43 | 9 | 52 |
| 2024 August | 40 | 7 | 47 |
| 2024 July | 51 | 6 | 57 |
| 2024 June | 36 | 4 | 40 |
| 2024 May | 27 | 5 | 32 |
| 2024 April | 33 | 3 | 36 |
| 2024 March | 39 | 3 | 42 |
| 2024 February | 71 | 8 | 79 |
| 2024 January | 68 | 5 | 73 |
| 2023 December | 47 | 9 | 56 |
| 2023 November | 86 | 9 | 95 |
| 2023 October | 91 | 11 | 102 |
| 2023 September | 44 | 7 | 51 |
| 2023 August | 55 | 11 | 66 |
| 2023 July | 65 | 9 | 74 |
| 2023 June | 51 | 11 | 62 |
| 2023 May | 80 | 9 | 89 |
| 2023 April | 60 | 1 | 61 |
| 2023 March | 63 | 7 | 70 |
| 2023 February | 78 | 13 | 91 |
| 2023 January | 39 | 5 | 44 |
| 2022 December | 43 | 6 | 49 |
| 2022 November | 50 | 12 | 62 |
| 2022 October | 54 | 14 | 68 |
| 2022 September | 54 | 12 | 66 |
| 2022 August | 51 | 25 | 76 |
| 2022 July | 25 | 7 | 32 |
| 2022 June | 21 | 7 | 28 |
| 2022 May | 29 | 11 | 40 |
| 2022 April | 35 | 11 | 46 |
| 2022 March | 45 | 10 | 55 |
| 2022 February | 42 | 6 | 48 |
| 2022 January | 40 | 6 | 46 |
| 2021 December | 30 | 15 | 45 |
| 2021 November | 59 | 9 | 68 |
| 2021 October | 40 | 12 | 52 |
| 2021 September | 47 | 15 | 62 |
| 2021 August | 36 | 3 | 39 |
| 2021 July | 28 | 7 | 35 |
| 2021 June | 34 | 11 | 45 |
| 2021 May | 39 | 10 | 49 |
| 2021 April | 92 | 22 | 114 |
| 2021 March | 63 | 10 | 73 |
| 2021 February | 38 | 11 | 49 |
| 2021 January | 32 | 9 | 41 |
| 2020 December | 35 | 12 | 47 |
| 2020 November | 28 | 11 | 39 |
| 2020 October | 27 | 6 | 33 |
| 2020 September | 21 | 9 | 30 |
| 2020 August | 19 | 9 | 28 |
| 2020 July | 19 | 17 | 36 |
| 2020 June | 15 | 3 | 18 |
| 2020 May | 27 | 11 | 38 |
| 2020 April | 16 | 3 | 19 |
| 2020 March | 17 | 3 | 20 |
| 2020 February | 16 | 9 | 25 |
| 2020 January | 26 | 4 | 30 |
| 2019 December | 21 | 13 | 34 |
| 2019 November | 14 | 10 | 24 |
| 2019 October | 5 | 4 | 9 |
| 2019 September | 18 | 5 | 23 |
| 2019 August | 7 | 4 | 11 |
| 2019 July | 9 | 14 | 23 |
| 2019 June | 45 | 39 | 84 |
| 2019 May | 93 | 48 | 141 |
| 2019 April | 32 | 35 | 67 |
| 2019 March | 8 | 8 | 16 |
| 2019 February | 4 | 8 | 12 |
| 2019 January | 8 | 6 | 14 |
| 2018 December | 6 | 6 | 12 |
| 2018 November | 4 | 7 | 11 |
| 2018 October | 19 | 10 | 29 |
| 2018 September | 6 | 5 | 11 |
| 2018 August | 1 | 2 | 3 |
| 2018 July | 0 | 3 | 3 |
| 2018 June | 1 | 7 | 8 |
| 2018 May | 7 | 10 | 17 |
| 2018 April | 8 | 4 | 12 |
| 2018 March | 0 | 1 | 1 |
| 2018 February | 1 | 4 | 5 |
| 2018 January | 7 | 3 | 10 |
| 2017 December | 4 | 10 | 14 |
| 2017 November | 7 | 6 | 13 |
| 2017 October | 10 | 4 | 14 |
| 2017 September | 9 | 4 | 13 |
| 2017 August | 14 | 8 | 22 |
| 2017 July | 14 | 7 | 21 |
| 2017 June | 8 | 9 | 17 |
| 2017 May | 12 | 14 | 26 |
| 2017 April | 7 | 5 | 12 |
| 2017 March | 8 | 32 | 40 |
| 2017 February | 8 | 6 | 14 |
| 2017 January | 9 | 6 | 15 |
| 2016 December | 13 | 13 | 26 |
| 2016 November | 8 | 17 | 25 |
| 2016 October | 26 | 22 | 48 |
| 2016 September | 15 | 14 | 29 |
| 2016 August | 16 | 12 | 28 |
| 2016 July | 11 | 10 | 21 |
| 2016 June | 25 | 19 | 44 |
| 2016 May | 17 | 22 | 39 |
| 2016 April | 14 | 21 | 35 |
| 2016 March | 24 | 28 | 52 |
| 2016 February | 23 | 19 | 42 |
| 2016 January | 14 | 15 | 29 |
| 2015 December | 12 | 14 | 26 |
| 2015 November | 20 | 14 | 34 |
| 2015 October | 24 | 26 | 50 |
| 2015 September | 15 | 20 | 35 |
| 2015 August | 42 | 13 | 55 |
| 2015 July | 34 | 13 | 47 |
| 2015 June | 33 | 4 | 37 |
| 2015 May | 31 | 9 | 40 |
| 2015 April | 45 | 24 | 69 |
| 2015 March | 18 | 18 | 36 |
| 2015 February | 7 | 8 | 15 |
| 2015 January | 31 | 16 | 47 |
| 2014 December | 38 | 6 | 44 |
| 2014 November | 21 | 6 | 27 |
| 2014 October | 23 | 2 | 25 |
| 2014 September | 38 | 2 | 40 |
| 2014 August | 42 | 6 | 48 |
| 2014 July | 39 | 6 | 45 |
| 2014 June | 25 | 2 | 27 |
| 2014 May | 18 | 3 | 21 |
| 2014 April | 17 | 2 | 19 |
| 2014 March | 18 | 6 | 24 |
| 2014 February | 22 | 5 | 27 |
| 2014 January | 14 | 5 | 19 |
| 2013 December | 27 | 5 | 32 |
| 2013 November | 30 | 3 | 33 |
| 2013 October | 43 | 4 | 47 |
| 2013 September | 40 | 4 | 44 |
| 2013 August | 42 | 4 | 46 |
| 2013 July | 29 | 4 | 33 |
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