Long-Term Outcome of Children with Acute Cerebellitis

E. Hennes; S. Zotter; L. Dorninger; H. Hartmann; M. Häusler; P. Huppke; J. Jacobs; V. Kraus; C. Makowski; K. Schlachter; H. Ulmer; A. van Baalen; J. Koch; T. Gotwald; K. Rostasy

doi:10.1055/s-0032-1324732

Neuropediatrics, Table of Contents

Neuropediatrics 2012; 43(05): 240-248
DOI: 10.1055/s-0032-1324732

Original Article

Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.

Long-Term Outcome of Children with Acute Cerebellitis

E. Hennes

¹Division of Pediatric Neurology, Department of Pediatrics I, Medical University Innsbruck, Innsbruck, Austria

,

S. Zotter

¹Division of Pediatric Neurology, Department of Pediatrics I, Medical University Innsbruck, Innsbruck, Austria

,

L. Dorninger

²Department of Pediatrics, Steyr Regional Hospital, Steyr, Austria

,

H. Hartmann

³Department of Pediatrics, Hannover Medical School, Hannover, Germany

,

M. Häusler

⁴Department of Pediatric Neurology, University Hospital Aachen, Aachen, Germany

,

P. Huppke

⁵Department of Pediatrics, University Hospital Göttingen, Göttingen, Germany

,

J. Jacobs

⁶Department of Pediatrics, University Hospital Freiburg, Freiburg, Germany

,

V. Kraus

⁷Department of Pediatric Neurology, Children Hospital, Technische Universität, Munich, Germany

,

C. Makowski

⁷Department of Pediatric Neurology, Children Hospital, Technische Universität, Munich, Germany

,

K. Schlachter

⁸Department of Pediatrics, Bregenz Regional Hospital, Bregenz, Austria

,

H. Ulmer

⁹Department of Medical Statistics, Informatics and Health Economics, University Hospital Innsbruck, Innsbruck, Austria

,

A. van Baalen

¹⁰Department of Neuropediatrics, University Hospital Kiel, Kiel, Germany

,

J. Koch

¹¹Department of Neuropediatrics, University Hospital Salzburg, Salzburg, Austria

,

T. Gotwald

¹²Departement of Radiology, Hospital Kettenbrücke, Innsbruck, Austria

,

K. Rostasy

¹Division of Pediatric Neurology, Department of Pediatrics I, Medical University Innsbruck, Innsbruck, Austria

› Author Affiliations

Abstract

Background Acute cerebellitis (AC) is characterized by cerebellar symptoms and magnetic resonance imaging (MRI) changes primarily confined to the cerebellum.

Objective To analyze the neurological and cognitive long-term outcome of children with AC.

Methods Children with AC diagnosed by typical clinical features and MRI findings were included in this retrospective study. Medical charts were reviewed and neurological deficits were assessed by neurological examination or by the expanded disability status scale telephone interview. Cognitive outcome was evaluated with a parental questionnaire (Kognitive Probleme bei Kindern und Jugendlichen).

Results A total of 11 children (6 boys, 5 girls; age range: 3 years to 14 years and 10 months) were included. Of them, six children had a severe disease manifestation including mental status changes and neurological symptoms. Of the rest, two children had a moderate and three children had a mild form of AC. MRI of the cerebellum was obtained in the acute phase revealing signal alterations with different patterns. The average follow-up period was 4 years and 4 months. A complete recovery was observed in five children. Neurological sequelae were reported in five children ranging from ataxia to mild tremor. Cognitive deficits were found in six patients. The affected areas of cognition did include spatial visualization ability, language skills, and concentration.

Conclusion Neurological and cognitive sequelae are common in children with AC and underline the role of the cerebellum in cognition.

Keywords

children - acute cerebellitis - long-term outcome - MRI

Full Text

References

References
1 Adachi M, Kawanami T, Ohshima H, Hosoya T. Cerebellar atrophy attributed to cerebellitis in two patients. Magn Reson Med Sci 2005; 4 (2) 103-107
2 Kamate M, Chetal V, Hattiholi V. Fulminant cerebellitis: a fatal, clinically isolated syndrome. Pediatr Neurol 2009; 41 (3) 220-222
3 Shiihara T, Kato M, Konno A, Takahashi Y, Hayasaka K. Acute cerebellar ataxia and consecutive cerebellitis produced by glutamate receptor delta2 autoantibody. Brain Dev 2007; 29 (4) 254-256
4 Vincent A, Dale RC. Inflammatory and autoimmune disorders of the nervous system in children. 1st ed. London, England: Mac Keith Press; 2010: 190-206
5 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
6 De Bruecker Y, Claus F, Demaerel P , et al. MRI findings in acute cerebellitis. Eur Radiol 2004; 14 (8) 1478-1483
7 Sawaishi Y, Takada G. Acute cerebellitis. Cerebellum 2002; 1 (3) 223-228
8 Gleissner U, Lendt M, Mayer S, Elger CE, Helmstaedter C. [A new questionnaire for recognizing cognitive problems in children and adolescents]. Nervenarzt 2006; 77 (4) 449-465
9 Levy EI, Harris AE, Omalu BI, Hamilton RL, Branstetter IV BF, Pollack IF. Sudden death from fulminant acute cerebellitis. Pediatr Neurosurg 2001; 35 (1) 24-28
10 Connolly AM, Dodson WE, Prensky AL, Rust RS. Course and outcome of acute cerebellar ataxia. Ann Neurol 1994; 35 (6) 673-679
11 Yiş U, Kurul SH, Cakmakçi H, Dirik E. Acute cerebellitis with cerebellar swelling successfully treated with standard dexamethasone treatment. Cerebellum 2008; 7 (3) 430-432
12 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) 970-974
13 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) 500-502
14 Kurtzke JF. Rating neurologic impairment in multiple sclerosis: an expanded disability status scale (EDSS). Neurology 1983; 33 (11) 1444-1452
15 Lechner-Scott J, Kappos L, Hofman M , et al. Can the Expanded Disability Status Scale be assessed by telephone?. Mult Scler 2003; 9 (2) 154-159
16 Borghi E, Pagani E, Mancuso R , et al. Detection of herpesvirus-6A in a case of subacute cerebellitis and myoclonic dystonia. J Med Virol 2005; 75 (3) 427-429
17 Papavasiliou AS, Kotsalis C, Trakadas S. Transient cerebellar mutism in the course of acute cerebellitis. Pediatr Neurol 2004; 30 (1) 71-74
18 Stoodley CJ. The cerebellum and cognition: evidence from functional imaging studies. Cerebellum 2012; 11 (2) 352-365
19 Levisohn L, Cronin-Golomb A, Schmahmann JD. Neuropsychological consequences of cerebellar tumour resection in children: cerebellar cognitive affective syndrome in a paediatric population. Brain 2000; 123 (Pt 5) 1041-1050
20 Schmahmann JD. Disorders of the cerebellum: ataxia, dysmetria of thought, and the cerebellar cognitive affective syndrome. J Neuropsychiatry Clin Neurosci 2004; 16 (3) 367-378
21 Schmahmann JD, Caplan D. Cognition, emotion and the cerebellum. Brain 2006; 129 (Pt 2) 290-292
22 Krienen FM, Buckner RL. Segregated fronto-cerebellar circuits revealed by intrinsic functional connectivity. Cereb Cortex 2009; 19 (10) 2485-2497
23 Stoodley CJ, Valera EM, Schmahmann JD. Functional topography of the cerebellum for motor and cognitive tasks: an fMRI study. Neuroimage 2012; 59 (2) 1560-1570
24 Rostásy K, Wilken B, Baumann M , et al. High dose pulsatile dexamethasone therapy in children with opsoclonus-myoclonus syndrome. Neuropediatrics 2006; 37 (5) 291-295