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is the gold standard of diagnosis&#46; Computed tomography &#40;CT&#41; can be useful during the acute phase to rule out other aetiologies and to detect the development of acute hydrocephalus or severe compression of the brainstem&#46;</p><p id="par0015" class="elsevierStylePara elsevierViewall">Acute cerebellitis is an infrequent process and its diagnosis is challenging&#44; as its presentation and course are widely heterogeneous&#46; Early intervention is essential to optimise outcomes&#44; so this disease should be suspected in patients with symptoms suggestive of posterior fossa involvement&#46;</p><p id="par0020" class="elsevierStylePara elsevierViewall">Cerebellar inflammation can lead to compression of the brainstem and cause alterations in the level of consciousness that may mask the initial manifestations of cerebellar involvement&#44; as patients may even present with coma and autonomic dysfunction&#46; This presentation&#44; in which raised intracranial pressure &#40;RICP&#41; symptoms predominate over cerebellar symptoms and associated with significant inflammation&#44; is known as fulminant acute cerebellitis<a class="elsevierStyleCrossRef" href="#bib0035"><span class="elsevierStyleSup">1</span></a> and should be considered in the differential diagnosis of patients with RICP of sudden onset&#46;<a class="elsevierStyleCrossRef" href="#bib0035"><span class="elsevierStyleSup">1</span></a> This form of disease carries an increased risk of permanent sequelae and even death&#46;<a class="elsevierStyleCrossRefs" href="#bib0035"><span class="elsevierStyleSup">1&#44;4&#44;6</span></a></p><p id="par0025" class="elsevierStylePara elsevierViewall">Due to the variable natural history of acute cerebellitis&#44; its management needs to be individualised&#46;<a class="elsevierStyleCrossRef" href="#bib0050"><span class="elsevierStyleSup">4</span></a> In mild cases without clinical progression or neuroimaging findings suggestive of a fulminant course&#44; a conservative approach with close monitoring may be sufficient&#46; In moderate to severe cases&#44; steroid drugs are the first-line treatment to reduce the mass effect of inflammation&#44; and placement of an external ventricular drain &#40;EVD&#41; may be necessary to manage hydrocephalus&#46;</p><p id="par0030" class="elsevierStylePara elsevierViewall">We now present the cases of 3 patients aged 7&#8211;12 years that received a diagnosis of AC&#44; none of whom had a personal or family history of interest&#46;</p><p id="par0035" class="elsevierStylePara elsevierViewall">The first patient sought care due to vomiting and malaise&#44; and presented on arrival with vasovagal syndrome&#44; with altered level of consciousness&#44; hypotonia and neurologic impairment&#46; A cranial CT scan was performed&#44; and the images showed hypodensity in the subcortical region of the left cerebellar hemisphere &#40;<a class="elsevierStyleCrossRef" href="#fig0005">Fig&#46; 1</a>A&#41;&#46; The patient was admitted to the intensive care unit&#44; and a cranial MRI was ordered to make the differential diagnosis between ischaemic injury of the posterior fossa&#44; encephalitis and AC &#40;<a class="elsevierStyleCrossRef" href="#fig0005">Fig&#46; 1</a>B&#41;&#44; while the patient remained under continuous monitoring &#40;including intracranial pressure&#41; and started treatment with acyclovir and antiplatelet and steroid therapy&#46; At 12<span class="elsevierStyleHsp" style=""></span>h from admission&#44; the patient developed raised intracranial pressure and anisocoria&#44; which led to performance of a cranial CT scan&#46; Based on the results of the scan&#44; we decided to perform a decompressive craniectomy with placement of an EVD&#44; which achieved stabilisation&#46; The patient subsequently started rehabilitation and showed neurologic improvement&#44; although sequelae were still present at the 4-month follow-up evaluation&#44; including dysarthria&#44; hypotonia&#44; inability to stand and right hemiparesis&#46; The follow-up cranial MRI scan revealed that the mass effect had resolved&#44; but also significant cerebellar atrophy&#46;</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><p id="par0040" class="elsevierStylePara elsevierViewall">The second and third patients both sought care for headache of approximately a week&#39;s duration that had intensified&#44; interfering with daily activities and disturbing sleep&#44; and accompanied by vomiting&#46; The findings of the physical examination and the CT scan were normal in both&#44; and they were both admitted to hospital for analgesia&#46; Due to lack of response&#44; they underwent evaluation by cranial MRI &#40;<a class="elsevierStyleCrossRef" href="#fig0010">Fig&#46; 2</a>&#41;&#44; which led to diagnosis of AC&#46; The management of AC consisted of monitoring and steroid therapy&#46; The patients responded well&#44; with improvement of symptoms and no complications&#44; so they were discharged with a prescription for a tapering course of steroids&#46; There was no evidence of sequelae in the follow-up evaluations&#46; The follow-up MRI confirmed the resolution of the mass effect&#46;</p><elsevierMultimedia ident="fig0010"></elsevierMultimedia><p id="par0045" class="elsevierStylePara elsevierViewall">In the 3 cases described here&#44; symptoms associated with RICP dominated the presentation &#40;headache and vomiting&#41;&#46; 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Scientific Letter
Fulminant acute cerebellitis: An under-diagnosed condition?
Cerebelitis aguda fulminante, ¿una entidad infradiagnosticada?
María Molina Corbachoa,
Corresponding author
mariamolcorba@gmail.com

Corresponding author.
, Fernando Martín Birlangaa, Nerea Sarrión Sosb, Pablo Gargallo Tataya, Miguel Tomás Vilac
a Departamento de Pediatría, Hospital Universitari i Politècnic La Fe, Valencia, Spain
b Departamento de Pediatría, Hospital General Universitario de Valencia, Valencia, Spain
c Departamento de Neuropediatría, Hospital Universitari i Politècnic La Fe, Valencia, Spain
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          "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">&#40;A&#41; Cranial CT scan&#44; axial plane&#58; subcortical hypodensity in left cerebellar hemisphere with nonspecific appearance&#46; &#40;B&#41; Head MRI&#44; T2-weighted axial view&#58; hyperintensity in both cerebellar hemispheres with restricted diffusion and cortical enhancement after administration of contrast&#46; &#40;C&#41; Cranial CT scan&#44; axial plane&#58; evidence of progression of cerebellar ischaemia associated with swelling and mass effect and with descent of the cerebellar tonsils to the foramen magnum and raised supratentorial ventricle&#46;</p>"
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    "textoCompleto" => "<span class="elsevierStyleSections"><p id="par0005" class="elsevierStylePara elsevierViewall">Acute cerebellitis &#40;AC&#41; is an inflammatory syndrome that causes acute cerebellar dysfunction &#40;ataxia&#44; nystagmus or dysmetria&#41; often in association with fever&#44; headache&#44; nausea and altered consciousness&#46;<a class="elsevierStyleCrossRefs" href="#bib0035"><span class="elsevierStyleSup">1&#44;2</span></a> It usually occurs in the context of infection or after infection or vaccination&#44; although there are cases in which a trigger is not identified&#46;<a class="elsevierStyleCrossRefs" href="#bib0045"><span class="elsevierStyleSup">3&#8211;5</span></a></p><p id="par0010" class="elsevierStylePara elsevierViewall">By consensus&#44; cerebellar ataxia is defined as cases with normal neuroimaging&#44; while AC is defined as cases presenting with imaging abnormalities&#44;<a class="elsevierStyleCrossRef" href="#bib0045"><span class="elsevierStyleSup">3</span></a> and magnetic resonance imaging &#40;MRI&#41; is the gold standard of diagnosis&#46; Computed tomography &#40;CT&#41; can be useful during the acute phase to rule out other aetiologies and to detect the development of acute hydrocephalus or severe compression of the brainstem&#46;</p><p id="par0015" class="elsevierStylePara elsevierViewall">Acute cerebellitis is an infrequent process and its diagnosis is challenging&#44; as its presentation and course are widely heterogeneous&#46; Early intervention is essential to optimise outcomes&#44; so this disease should be suspected in patients with symptoms suggestive of posterior fossa involvement&#46;</p><p id="par0020" class="elsevierStylePara elsevierViewall">Cerebellar inflammation can lead to compression of the brainstem and cause alterations in the level of consciousness that may mask the initial manifestations of cerebellar involvement&#44; as patients may even present with coma and autonomic dysfunction&#46; This presentation&#44; in which raised intracranial pressure &#40;RICP&#41; symptoms predominate over cerebellar symptoms and associated with significant inflammation&#44; is known as fulminant acute cerebellitis<a class="elsevierStyleCrossRef" href="#bib0035"><span class="elsevierStyleSup">1</span></a> and should be considered in the differential diagnosis of patients with RICP of sudden onset&#46;<a class="elsevierStyleCrossRef" href="#bib0035"><span class="elsevierStyleSup">1</span></a> This form of disease carries an increased risk of permanent sequelae and even death&#46;<a class="elsevierStyleCrossRefs" href="#bib0035"><span class="elsevierStyleSup">1&#44;4&#44;6</span></a></p><p id="par0025" class="elsevierStylePara elsevierViewall">Due to the variable natural history of acute cerebellitis&#44; its management needs to be individualised&#46;<a class="elsevierStyleCrossRef" href="#bib0050"><span class="elsevierStyleSup">4</span></a> In mild cases without clinical progression or neuroimaging findings suggestive of a fulminant course&#44; a conservative approach with close monitoring may be sufficient&#46; In moderate to severe cases&#44; steroid drugs are the first-line treatment to reduce the mass effect of inflammation&#44; and placement of an external ventricular drain &#40;EVD&#41; may be necessary to manage hydrocephalus&#46;</p><p id="par0030" class="elsevierStylePara elsevierViewall">We now present the cases of 3 patients aged 7&#8211;12 years that received a diagnosis of AC&#44; none of whom had a personal or family history of interest&#46;</p><p id="par0035" class="elsevierStylePara elsevierViewall">The first patient sought care due to vomiting and malaise&#44; and presented on arrival with vasovagal syndrome&#44; with altered level of consciousness&#44; hypotonia and neurologic impairment&#46; A cranial CT scan was performed&#44; and the images showed hypodensity in the subcortical region of the left cerebellar hemisphere &#40;<a class="elsevierStyleCrossRef" href="#fig0005">Fig&#46; 1</a>A&#41;&#46; The patient was admitted to the intensive care unit&#44; and a cranial MRI was ordered to make the differential diagnosis between ischaemic injury of the posterior fossa&#44; encephalitis and AC &#40;<a class="elsevierStyleCrossRef" href="#fig0005">Fig&#46; 1</a>B&#41;&#44; while the patient remained under continuous monitoring &#40;including intracranial pressure&#41; and started treatment with acyclovir and antiplatelet and steroid therapy&#46; At 12<span class="elsevierStyleHsp" style=""></span>h from admission&#44; the patient developed raised intracranial pressure and anisocoria&#44; which led to performance of a cranial CT scan&#46; Based on the results of the scan&#44; we decided to perform a decompressive craniectomy with placement of an EVD&#44; which achieved stabilisation&#46; The patient subsequently started rehabilitation and showed neurologic improvement&#44; although sequelae were still present at the 4-month follow-up evaluation&#44; including dysarthria&#44; hypotonia&#44; inability to stand and right hemiparesis&#46; The follow-up cranial MRI scan revealed that the mass effect had resolved&#44; but also significant cerebellar atrophy&#46;</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><p id="par0040" class="elsevierStylePara elsevierViewall">The second and third patients both sought care for headache of approximately a week&#39;s duration that had intensified&#44; interfering with daily activities and disturbing sleep&#44; and accompanied by vomiting&#46; The findings of the physical examination and the CT scan were normal in both&#44; and they were both admitted to hospital for analgesia&#46; Due to lack of response&#44; they underwent evaluation by cranial MRI &#40;<a class="elsevierStyleCrossRef" href="#fig0010">Fig&#46; 2</a>&#41;&#44; which led to diagnosis of AC&#46; The management of AC consisted of monitoring and steroid therapy&#46; The patients responded well&#44; with improvement of symptoms and no complications&#44; so they were discharged with a prescription for a tapering course of steroids&#46; There was no evidence of sequelae in the follow-up evaluations&#46; The follow-up MRI confirmed the resolution of the mass effect&#46;</p><elsevierMultimedia ident="fig0010"></elsevierMultimedia><p id="par0045" class="elsevierStylePara elsevierViewall">In the 3 cases described here&#44; symptoms associated with RICP dominated the presentation &#40;headache and vomiting&#41;&#46; The method used for diagnosis was MRI&#46; We did not identify a likely aetiology in any case except the first&#44; in which enterovirus was isolated from 2 stool samples&#46; The approach to the management of AC was high-dose steroid therapy in all patients&#46;</p><p id="par0050" class="elsevierStylePara elsevierViewall">In conclusion&#44; fulminant AC requires urgent medical treatment and sometimes surgical intervention&#46; Thus&#44; it is important for clinicians to be knowledgeable of this disease so that they can suspect it and perform an urgent head MRI for diagnosis&#44; which allows early initiation of treatment&#46;</p></span>"
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        "nota" => "<p class="elsevierStyleNotepara" id="npar0005">Please cite this article as&#58; Molina Corbacho M&#44; Mart&#237;n Birlanga F&#44; Sarri&#243;n Sos N&#44; Gargallo Tatay P&#44; Tom&#225;s Vila M&#46; Cerebelitis aguda fulminante&#44; &#191;una entidad infradiagnosticada&#63;&#46; An Pediatr &#40;Barc&#41;&#46; 2019&#59;90&#58;188&#8211;190&#46;</p>"
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Article information
ISSN: 23412879
Original language: English
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Idiomas
Anales de Pediatría (English Edition)
es en

¿Es usted profesional sanitario apto para prescribir o dispensar medicamentos?

Are you a health professional able to prescribe or dispense drugs?