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Medical Science Monitor Basic Research


Pediatric Fulminant Cerebellitis Is Still a Fatal Disease that We Know Little About! Two Case Reports and a Literature Review

Unusual clinical course, Challenging differential diagnosis, Rare disease

Hakem Alomani, Muhammad Arshad, Mahmoud Elzonfly, Ali Ahmad Aldakhil, Abdullah H. Alharbi, Abdulrahman Alasqah, Bandar Rashed Alfheed, Hesham Aldhalan

Saudi Arabia Department of Pediatrics, Maternity and Children’s Hospital, Buriadah, Saudi Arabia

Am J Case Rep 2021; 22:e928370

DOI: 10.12659/AJCR.928370

Available online: 2020-12-01

Published: 2021-01-17


BACKGROUND: Acute cerebellitis is a rare inflammatory disease with a variable clinical course that ranges from self-limiting illness with a benign outcome to a fulminant process (known as fulminant cerebellitis) associated with high risk of morbidity and mortality. It poses a unique challenge to the pediatric community due to its infrequent occurrence and variable presentation, which ultimately contribute to variation in treatment and variation in clinical outcome. This condition has received little attention, with scattered case reports and reviews in the literature and no clear consensus or guidelines for pediatricians to standardize the approach and management.
CASE REPORT: We report the cases of 2 previously healthy children diagnosed with fulminant cerebellitis. The clinical picture started with severe headache, irritability, and acute deterioration of consciousness level with the absence of cerebellar signs. There was radiological evidence of obstructive hydrocephalus and cerebellar tonsillar herniation in the context of the inflamed cerebellum. The 2 patients received timely standard medical and surgical management for acute cerebellitis in the form of neuroprotective measures, osmotherapy, broad-spectrum antibiotics, and CSF diversion. One patient received aggressive immunomodulatory therapy and had a better outcome compared with the other patient who received no immunomodulatory therapy and had a poor outcome.
CONCLUSIONS: A high index suspicion and early aggressive intervention are the best tools for achieving optimal outcomes in fulminant cerebellitis in children, given the lack of classic cerebellar signs. In addition to CSF diversion and broad-spectrum antibiotics and antivirals, some patients may benefit from early immunomodulator therapy and posterior fossa decompressive craniotomy.

Keywords: Cerebellar Ataxia, Cerebellar Diseases, Encephalocele