Skip to main content
Top
Published in: Italian Journal of Pediatrics 1/2017

Open Access 01-12-2017 | Research

Acute cerebellitis in children: an eleven year retrospective multicentric study in Italy

Authors: Laura Lancella, Susanna Esposito, Maria Luisa Galli, Elena Bozzola, Valeria Labalestra, Elena Boccuzzi, Andrzej Krzysztofiak, Laura Cursi, Guido Castelli Gattinara, Nadia Mirante, Danilo Buonsenso, Claudia Tagliabue, Luca Castellazzi, Carlotta Montagnani, Chiara Tersigni, Piero Valentini, Michele Capozza, Davide Pata, Maria Di Gangi, Piera Dones, Silvia Garazzino, Luca Baroero, Alberto Verrotti, Maria Luisa Melzi, Michele Sacco, Michele Germano, Filippo Greco, Elena Uga, Giovanni Crichiutti, Alberto Villani

Published in: Italian Journal of Pediatrics | Issue 1/2017

Login to get access

Abstract

Background

Acute cerebellitis (AC) and acute cerebellar ataxia (ACA) are the principal causes of acute cerebellar dysfunction in childhood. Nevertheless. there is no accepted consensus regarding the best management of children with AC/ACA: the aim of the study is both to assess clinical, neuroimaging and electrophysiologic features of children with AC/ACA and to evaluate the correlation between clinical parameters, therapy and outcome.

Methods

A multicentric retrospective study was conducted on children ≤ 18 years old admitted to 12 Italian paediatric hospitals for AC/ACA from 01/01/2003 to 31/12/2013. A score based on both cerebellar and extracerebellar signs/symptoms was computed for each patient. One point was given for each sign/symptom reported. Severity was divided in three classes: low, moderate, severe.

Results

A total of 124 children were included in the study. Of these, 118 children received a final diagnosis of ACA and 6 of AC. The most characteristic finding of AC/ACA was a broad-based gait disturbance. Other common symptoms included balance disturbances, slurred speech, vomiting, headache and fever. Neurological sequelae were reported in 6 cases (5%) There was no correlation among symptoms, cerebrospinal fluid findings, clinical outcome. There was no correlation between clinical manifestations and clinical score on admission and length of hospital stay, sex, age and EEG findings with sequelae (P > 0.05).
Children with pathological magnetic resonance imaging (MRI) or computed tomography (CT) had a higher probability of having clinical sequelae.
Treatment was decided independently case by case. Patients with a higher clinical score on admission had a higher probability of receiving intravenous steroids.

Conclusions

We confirmed the literature data about the benign course of AC/ACA in most cases but we also highlighted a considerable rate of patients with neurological sequelae (5%). Pathological MRI or CT findings at admission correlate to neurological sequelae. These findings suggest the indication to perform an instrumental evaluation in all patients with AC/ACA at admission to identify those at higher risk of neurological outcome. These patients may benefit from a more aggressive therapeutic strategy and should have a closer follow-up. Randomized controlled trials are needed to confirm these observations. The ultimate goal of these studies could be to develop a standardized protocol on AC/ACA. The MRI/CT data, associated with the clinical manifestations, may allow us to define the class risk of patients for a neurological outcome.
Literature
2.
go back to reference Amador N, Scheithauer BW, Giannini C, Raffel C. Acute cerebellitis presenting as tumor. Report of two cases. J Neurosurg 2007; 107(1)(Suppl)57–61. Amador N, Scheithauer BW, Giannini C, Raffel C. Acute cerebellitis presenting as tumor. Report of two cases. J Neurosurg 2007; 107(1)(Suppl)57–61.
3.
go back to reference De Bruecker Y, Claus F, Demaerel P, et al. MRI findings in acute cerebellitis. Eur Radiol. 2004;14(8):1478–83.CrossRefPubMed De Bruecker Y, Claus F, Demaerel P, et al. MRI findings in acute cerebellitis. Eur Radiol. 2004;14(8):1478–83.CrossRefPubMed
4.
go back to reference Adachi M, Kawanami T, Ohshima H, Hosoya T. Cerebellar atrophy attributed to cerebellitis in two patients. Magn Reson Med Sci. 2005;4(2):103–7.CrossRefPubMed Adachi M, Kawanami T, Ohshima H, Hosoya T. Cerebellar atrophy attributed to cerebellitis in two patients. Magn Reson Med Sci. 2005;4(2):103–7.CrossRefPubMed
5.
go back to reference Kamate M, Chetal V, Hattiholi V. Fulminant cerebellitis: a fatal, clinically isolated syndrome. Pediatr Neurol. 2009;41(3):220–2.CrossRefPubMed Kamate M, Chetal V, Hattiholi V. Fulminant cerebellitis: a fatal, clinically isolated syndrome. Pediatr Neurol. 2009;41(3):220–2.CrossRefPubMed
6.
go back to reference Shiihara T, Kato M, Konno A, Takahashi Y, Hayasaka K. Acute cerebellar ataxia and consecutive cerebellitis produced by glutamate receptor delta2 autoantibody. Brain and Development. 2007;29(4):254–6.CrossRefPubMed Shiihara T, Kato M, Konno A, Takahashi Y, Hayasaka K. Acute cerebellar ataxia and consecutive cerebellitis produced by glutamate receptor delta2 autoantibody. Brain and Development. 2007;29(4):254–6.CrossRefPubMed
7.
go back to reference Levy EI, Harris AE, Omalu BI, Hamilton RL, Branstetter BF IV, Pollack IF. Sudden death from fulminant acute cerebellitis. Pediatr Neurosurg. 2001;35(1):24–8.CrossRefPubMed Levy EI, Harris AE, Omalu BI, Hamilton RL, Branstetter BF IV, Pollack IF. Sudden death from fulminant acute cerebellitis. Pediatr Neurosurg. 2001;35(1):24–8.CrossRefPubMed
9.
go back to reference Hayakawa H, Katoh T. Severe cerebellar atrophy following acute cerebellitis. Pediatr Neurol. 1995;12:159–61.CrossRefPubMed Hayakawa H, Katoh T. Severe cerebellar atrophy following acute cerebellitis. Pediatr Neurol. 1995;12:159–61.CrossRefPubMed
10.
go back to reference Yiş U, Kurul SH, Cakmakçi H, Dirik E. Acute cerebellitis with cerebellar swelling successfully treated with standard dexamethasone treatment. Cerebellum. 2008;7(3):430–2.CrossRefPubMed Yiş U, Kurul SH, Cakmakçi H, Dirik E. Acute cerebellitis with cerebellar swelling successfully treated with standard dexamethasone treatment. Cerebellum. 2008;7(3):430–2.CrossRefPubMed
11.
go back to reference de Ribaupierre S, Meagher-Villemure K, Villemure JG, et al. The role of posterior fossa decompression in acute cerebellitis. Childs Nerv Syst. 2005;21(11):970–4.CrossRefPubMed de Ribaupierre S, Meagher-Villemure K, Villemure JG, et al. The role of posterior fossa decompression in acute cerebellitis. Childs Nerv Syst. 2005;21(11):970–4.CrossRefPubMed
12.
go back to reference Hamada H, Kurimoto M, Masuoka T, Hirashima Y, Endo S, Harada J. A case of surgically treated acute cerebellitis with hydrocephalus. Childs Nerv Syst. 2001;17(8):500–2.CrossRefPubMed Hamada H, Kurimoto M, Masuoka T, Hirashima Y, Endo S, Harada J. A case of surgically treated acute cerebellitis with hydrocephalus. Childs Nerv Syst. 2001;17(8):500–2.CrossRefPubMed
13.
go back to reference Rack AL, Grote V, Streng A, Belohradsky BH, Heinen F, von Kries R, et al. Neurologic varicella complications before routine immunization in Germany. Pediatr Neurol. 2010;42(1):40–8.CrossRefPubMed Rack AL, Grote V, Streng A, Belohradsky BH, Heinen F, von Kries R, et al. Neurologic varicella complications before routine immunization in Germany. Pediatr Neurol. 2010;42(1):40–8.CrossRefPubMed
14.
go back to reference Cameron JC, Allan G, Johnston F, Finn A, Heath PT, Booy R. Severe complications of chickenpox in hospitalised children in the UK and Ireland. Arch Dis Child. 2007;92(12):1062–6.CrossRefPubMedPubMedCentral Cameron JC, Allan G, Johnston F, Finn A, Heath PT, Booy R. Severe complications of chickenpox in hospitalised children in the UK and Ireland. Arch Dis Child. 2007;92(12):1062–6.CrossRefPubMedPubMedCentral
15.
go back to reference Koturoglu G, Kurugöl Z, Cetin N, Hizarcioglu M, Vardar F, Helvaci M, et al. Complications of varicella in healthy children in Izmir. Turkey Pediatr Int. 2005;47(3):296–9.CrossRefPubMed Koturoglu G, Kurugöl Z, Cetin N, Hizarcioglu M, Vardar F, Helvaci M, et al. Complications of varicella in healthy children in Izmir. Turkey Pediatr Int. 2005;47(3):296–9.CrossRefPubMed
16.
go back to reference Losurdo G, Bertoluzzo L, Canale F, Timitilli A, Bondi E, Castagnola E, et al. Varicella and its complications as cause of hospitalization. Infez Med. 2005;13(4):229–34.PubMed Losurdo G, Bertoluzzo L, Canale F, Timitilli A, Bondi E, Castagnola E, et al. Varicella and its complications as cause of hospitalization. Infez Med. 2005;13(4):229–34.PubMed
17.
go back to reference Bozzola, et al. Acute cerebellitis in varicella: a ten year case series and systematic review of the literature. Italian Journal of Pediatrics. 2014;40:57.CrossRefPubMedPubMedCentral Bozzola, et al. Acute cerebellitis in varicella: a ten year case series and systematic review of the literature. Italian Journal of Pediatrics. 2014;40:57.CrossRefPubMedPubMedCentral
18.
go back to reference Gohlich-Ratmann G, Wallot M, Baethmann M, Schaper J, Roggendorf M, Roll C, et al. Acute cerebellitis with near-fatal cerebellar swelling and benign outcome under conservative treatment with high dose steroids. Eur J Paediatr Neurol. 1998;2:157–62.CrossRefPubMed Gohlich-Ratmann G, Wallot M, Baethmann M, Schaper J, Roggendorf M, Roll C, et al. Acute cerebellitis with near-fatal cerebellar swelling and benign outcome under conservative treatment with high dose steroids. Eur J Paediatr Neurol. 1998;2:157–62.CrossRefPubMed
19.
go back to reference Jabbour P, Samaha E, Abi-Lahoud G, Koussa S, Abadjian G, Nohra G, et al. Hemicerebellitis mimicking a tumour on MRI. Childs Nerv Syst. 2003;19:122–5.PubMed Jabbour P, Samaha E, Abi-Lahoud G, Koussa S, Abadjian G, Nohra G, et al. Hemicerebellitis mimicking a tumour on MRI. Childs Nerv Syst. 2003;19:122–5.PubMed
20.
go back to reference Gosalakkal JA. Epstein-Barr virus cerebellitis presenting as obstructive hydrocephalus. Clin Pediatr (Phila). 2001;40:229–31.CrossRef Gosalakkal JA. Epstein-Barr virus cerebellitis presenting as obstructive hydrocephalus. Clin Pediatr (Phila). 2001;40:229–31.CrossRef
21.
go back to reference Balfour HH Jr, Edelman CK, Anderson RS, Reed NV, Slivken RM, Marmor LH, et al. Controlled trial of acyclovir for chickenpox evaluating time of initiation and duration of therapy and viral resistance. Pediatr Infect Dis J. 2001;20(10):919–26.CrossRefPubMed Balfour HH Jr, Edelman CK, Anderson RS, Reed NV, Slivken RM, Marmor LH, et al. Controlled trial of acyclovir for chickenpox evaluating time of initiation and duration of therapy and viral resistance. Pediatr Infect Dis J. 2001;20(10):919–26.CrossRefPubMed
22.
go back to reference Uchibori A, Sakuta M, Kusunoki S, Chiba A. Autoantibodies in postinfectious acute cerebellar ataxia. Neurology. 2005;65:1114–6.CrossRefPubMed Uchibori A, Sakuta M, Kusunoki S, Chiba A. Autoantibodies in postinfectious acute cerebellar ataxia. Neurology. 2005;65:1114–6.CrossRefPubMed
23.
go back to reference Adams C, Diadori P, Schoenroth L, Fritzler M. Autoantibodies in childhood post varicella acute cerebellar ataxia. Can J Neurol Sci. 2000;27:316–20.CrossRefPubMed Adams C, Diadori P, Schoenroth L, Fritzler M. Autoantibodies in childhood post varicella acute cerebellar ataxia. Can J Neurol Sci. 2000;27:316–20.CrossRefPubMed
24.
go back to reference Fritzler MJ, Zhang M, Stinton LM, Rattner JB. Spectrum of centrosome autoantibodies in childhood varicella and post-varicella acute cerebellar ataxia. BMC Pediatr. 2003;3:11.CrossRefPubMedPubMedCentral Fritzler MJ, Zhang M, Stinton LM, Rattner JB. Spectrum of centrosome autoantibodies in childhood varicella and post-varicella acute cerebellar ataxia. BMC Pediatr. 2003;3:11.CrossRefPubMedPubMedCentral
25.
go back to reference Camacho-Badilla K, Méndez I, Soriano-Fallas A, Ulloa-Gutiérrez R, Avila-Aguero ML. Postvaricella cerebellar ataxia in children in Costa Rica. An Pediatr (Barc). 2008;68(1):49–53.CrossRef Camacho-Badilla K, Méndez I, Soriano-Fallas A, Ulloa-Gutiérrez R, Avila-Aguero ML. Postvaricella cerebellar ataxia in children in Costa Rica. An Pediatr (Barc). 2008;68(1):49–53.CrossRef
27.
go back to reference Klassen TP, Hartling L, Wiebe N, Belseck EM. Acyclovir for treating varicella in otherwise healthy children and adolescents. The Cochrane Library, Issue 4, 2005. 13. Klassen TP, Hartling L, Wiebe N, Belseck EM. Acyclovir for treating varicella in otherwise healthy children and adolescents. The Cochrane Library, Issue 4, 2005. 13.
28.
go back to reference Marchetto S, de Benedictis FM, de Martino M, Versace A, Chiappini E, Bertaine C, et al. Epidemiology of hospital admissions for chickenpox in children: an Italian multicenter study in the pre-vaccine era. Acta Paediatr. 2007;96(10):1490–3.CrossRefPubMed Marchetto S, de Benedictis FM, de Martino M, Versace A, Chiappini E, Bertaine C, et al. Epidemiology of hospital admissions for chickenpox in children: an Italian multicenter study in the pre-vaccine era. Acta Paediatr. 2007;96(10):1490–3.CrossRefPubMed
29.
go back to reference Salas AA, Nava A. Acute cerebellar ataxia in childhood: initial approach in the emergency department. Emerg Med J. 2010;27(12):956–7.CrossRefPubMed Salas AA, Nava A. Acute cerebellar ataxia in childhood: initial approach in the emergency department. Emerg Med J. 2010;27(12):956–7.CrossRefPubMed
30.
go back to reference Chamizo FJ, Gilarranz R, Hernández M, Ramos D, Pena MJ. Central nervous system infections caused by varicella-zoster virus. J Neurovirol. 2016;22(4):529–32.CrossRefPubMed Chamizo FJ, Gilarranz R, Hernández M, Ramos D, Pena MJ. Central nervous system infections caused by varicella-zoster virus. J Neurovirol. 2016;22(4):529–32.CrossRefPubMed
31.
go back to reference Grahn A, Studahl M. Varicella-zoster virus infections of the central nervous system – Prognosis, diagnostics and treatment. J Infect. 2015;71(3):281–93.CrossRefPubMed Grahn A, Studahl M. Varicella-zoster virus infections of the central nervous system – Prognosis, diagnostics and treatment. J Infect. 2015;71(3):281–93.CrossRefPubMed
32.
go back to reference Paul R, Singhania P, Hashmi M, Bandyopadhyay R, Banerjee AK. Post chicken pox neurological sequelae: Three distinct presentations. J Neurosci Rural Pract. 2010;1(2):92–6.CrossRefPubMedPubMedCentral Paul R, Singhania P, Hashmi M, Bandyopadhyay R, Banerjee AK. Post chicken pox neurological sequelae: Three distinct presentations. J Neurosci Rural Pract. 2010;1(2):92–6.CrossRefPubMedPubMedCentral
33.
go back to reference Sivaswamy L. Approach to acute ataxia in childhood: diagnosis and evaluation. Pediatr Ann. 2014 Apr;43(4):153–9.CrossRefPubMed Sivaswamy L. Approach to acute ataxia in childhood: diagnosis and evaluation. Pediatr Ann. 2014 Apr;43(4):153–9.CrossRefPubMed
34.
go back to reference Gilden D. Varicella zoster virus and central nervous system syndromes. Herpes. 2004;11(Suppl. 2):89–94. Gilden D. Varicella zoster virus and central nervous system syndromes. Herpes. 2004;11(Suppl. 2):89–94.
35.
go back to reference Connolly AM, Dodson WE, Prensky AL, Rust RS. Course and outcome of acute cerebellar ataxia. Ann Neurol. 1994;35(6):673–9.CrossRefPubMed Connolly AM, Dodson WE, Prensky AL, Rust RS. Course and outcome of acute cerebellar ataxia. Ann Neurol. 1994;35(6):673–9.CrossRefPubMed
Metadata
Title
Acute cerebellitis in children: an eleven year retrospective multicentric study in Italy
Authors
Laura Lancella
Susanna Esposito
Maria Luisa Galli
Elena Bozzola
Valeria Labalestra
Elena Boccuzzi
Andrzej Krzysztofiak
Laura Cursi
Guido Castelli Gattinara
Nadia Mirante
Danilo Buonsenso
Claudia Tagliabue
Luca Castellazzi
Carlotta Montagnani
Chiara Tersigni
Piero Valentini
Michele Capozza
Davide Pata
Maria Di Gangi
Piera Dones
Silvia Garazzino
Luca Baroero
Alberto Verrotti
Maria Luisa Melzi
Michele Sacco
Michele Germano
Filippo Greco
Elena Uga
Giovanni Crichiutti
Alberto Villani
Publication date
01-12-2017
Publisher
BioMed Central
Published in
Italian Journal of Pediatrics / Issue 1/2017
Electronic ISSN: 1824-7288
DOI
https://doi.org/10.1186/s13052-017-0370-z

Other articles of this Issue 1/2017

Italian Journal of Pediatrics 1/2017 Go to the issue