Case ReportFulminant Cerebellitis: A Fatal, Clinically Isolated Syndrome
Introduction
Acute postinfectious cerebellitis is usually a self-limiting condition in children presenting with sudden-onset ataxia, and is characterized by complete recovery in the majority of cases. However, a few recent reports of acute cerebellitis with a critical or potentially critical course have changed the conception of this disease from an acute cerebellar ataxia with a benign course to a more serious disease entity with a heterogeneous pathogenesis [1]. A diagnosis of acute cerebellitis is difficult because clinical presentations often vary, resulting in diagnostic delay. Some patients may present with only mild cerebellar signs, whereas others may present with brainstem involvement.
We report on the clinical presentation and neuroimaging features of one patient with acute cerebellitis that was associated with brainstem involvement and hydrocephalus. Although cases of diffuse cerebellitis with or without brainstem involvement were previously documented, cases of cerebellitis resulting in death are rare. To our knowledge, only one example of cerebellitis causing death was previously reported [2].
Section snippets
Case Report
A 9-year-old boy was referred to our hospital with complaints of severe occipital headache, vomiting, and gait disorder in the form of swaying to either side for 10 days. These complaints were associated with intermittent fever (temperature rising to 102°F). However, there had been no history of trauma or any viral exanthem in the preceding few weeks. There was also no history of altered sensorium, nystagmus, seizures, dysarthria, or difficulty in feeding and swallowing. On admission, a
Discussion
Cerebellitis is an inflammatory syndrome resulting in acute cerebellar dysfunction, which can occur as a primary infectious, postinfectious, or postvaccination disorder. It may also occur without evidence of an antecedent or concurrent factor [3]. According to the proposed consensus definition by the International Multiple Sclerosis Study group for central nervous system inflammatory demyelinating disorders of childhood in 2007, cerebellitis is a type of clinically isolated syndrome [4].
References (10)
- et al.
Imaging of acute cerebellitis in children. Report of 4 cases
J Neuroradiol
(2006) - et al.
Acute cerebellitis
Cerebellum
(2002) - et al.
Sudden death from fulminant acute cerebellitis
Pediatr Neurosurg
(2001) - et al.
A child with cerebellar ataxia
Br J Radiol
(2008) - et al.
Consensus definitions proposed for pediatric multiple sclerosis and related disorders
Neurology
(2007)
Cited by (35)
Acute Fulminant Cerebellitis in Children With COVID-19: A Rare But a Treatable Complication
2021, Pediatric NeurologyCerebellitis as a neurosurgical disease in pediatrics
2021, Journal of Clinical NeuroscienceCitation Excerpt :With faster diagnosis and treatment, patients may have the best chance at acceptable cognitive and functional outcomes if surgery to relieve intracranial pressure via temporary CSF diversion or decompression is done in a timely manner, if and when indicated. While most patients recover completely [10], though persistent cerebellar dysfunction and mortality, due to brainstem compression, have been reported [2,7,10–13]. Severity of presentation may predict poor outcome [10,11]; perhaps contributing to the lower rate of complete recovery observed in our cohort, as all but two patients presented with moderate or severe disease.
Autoimmune cerebellitis in Crohn's disease
2019, Gastroenterologia y HepatologiaAcute cerebellitis in paediatric patients: Our experience
2019, NeurologiaFulminant acute cerebellitis: An under-diagnosed condition?
2019, Anales de PediatriaParainfectious and Postinfectious Neurologic Syndromes
2017, Principles and Practice of Pediatric Infectious Diseases