Elsevier

Pediatric Neurology

Volume 41, Issue 3, September 2009, Pages 220-222
Pediatric Neurology

Case Report
Fulminant Cerebellitis: A Fatal, Clinically Isolated Syndrome

https://doi.org/10.1016/j.pediatrneurol.2009.03.018Get rights and content

Acute cerebellitis constitutes a clinically isolated syndrome, and is a frequent condition in childhood, with either viral or autoimmune etiologies. The disease is reported to run a variable course, and is usually benign. Acute cerebellitis with cerebellar swelling, hydrocephalus, and brainstem compression is an exceptional but life-threatening condition. We report a 9-year-old boy in whom death resulted from acute fulminant cerebellitis because of brainstem involvement. Serial computed tomography and magnetic resonance imaging demonstrated rapid progression of the disease. The etiologies, clinical course, and therapeutic interventions regarding this potentially life-threatening condition are briefly reviewed.

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].

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