Case of the Week
Section Editors: Matylda Machnowska1 and Anvita Pauranik2
1University of Toronto, Toronto, Ontario, Canada
2BC Children's Hospital, University of British Columbia, Vancouver, British Columbia, Canada
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June 4, 2015
Acute Cerebellitis
- Acute cerebellitis is a rare inflammatory syndrome characterized by cerebellar dysfunction. It usually occurs as a primary infectious, postinfectious or postvaccination disorder, and mostly presents in early childhood.
- Clinical Presentation: Variable: trunk and limbs ataxia, fever, abnormal eye movements, dysarthria, headache, nausea, vomiting and decreased level of consciousness
- Clinical Course: From self-limited to fatal, depending on the amount of cerebellar swelling
- Key Diagnostic Features:
- Typical: Bilateral hemispheric cerebellar swelling with cortical and white matter T2 hyperintensities; leptomenigeal enhancement may be present.
- Less frequent: Obstructive hydrocephalus, unilateral involvement, normal T2 signal
- Atypical: parenchymal cortical enhancement
- CSF: Elevated protein and leukocytes, with lymphocytic predominance, normal glucose
- Blood/CSF: Antibodies anti- HSV, EBV, VZV, mumps, rubella, Lyme disease
- Typical MRI findings strongly support the diagnosis in patients with suitable clinical presentation and CSF findings.
- DDx:
- ADEM
- Lhermitte-Duclos disease
- Vasculitis
- Drug intoxication (eg, heroin inhalation, or “chasing the dragon”)
- Treatment: Steroids, Aciclovir, decompressive craniectomy if necessary
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