Department of Pediatrics, Maternity and Children's Hospital, Buriadah, Saudi Arabia.
Department of Pediatrics, Maternity and Children Hospital, Buriadah, Saudi Arabia.
Am J Case Rep. 2021 Jan 17;22:e928370. doi: 10.12659/AJCR.928370.
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.
急性小脑炎是一种罕见的炎症性疾病，其临床病程变化较大，从自限性疾病（预后良好）到暴发性病程（称为暴发性小脑炎）不等，后者与高发病率和死亡率相关。由于其发病率低且临床表现多样，儿科医生在治疗和临床结局方面存在差异，这给儿科医生带来了独特的挑战。这种疾病很少受到关注，文献中只有散在的病例报告和综述，没有明确的共识或指南来规范儿科医生的治疗方法。
我们报告了 2 例先前健康的儿童被诊断为暴发性小脑炎的病例。临床表现为严重头痛、烦躁不安和意识水平急性恶化，而无小脑体征。在炎症性小脑的背景下，存在影像学证据提示阻塞性脑积水和小脑扁桃体疝。这 2 例患者均接受了及时的标准医学和手术治疗，包括神经保护措施、渗透压治疗、广谱抗生素和脑脊液分流。其中 1 例患者接受了强化免疫调节治疗，与未接受免疫调节治疗的另 1 例患者相比，预后较好。
鉴于缺乏典型的小脑体征，对暴发性小脑炎保持高度怀疑并尽早积极干预是获得最佳结局的最佳方法。除了脑脊液分流和广谱抗生素和抗病毒药物外，一些患者可能受益于早期免疫调节剂治疗和后颅窝减压手术。
