Acute cerebellitis in children

Liora Kornreich, Vered Shkalim-Zemer, Yoel Levinsky, Wafa Abdallah, Esther Ganelin-Cohen, Rachel Straussberg.  Acute Cerebellitis in Children: A Many-Faceted Disease.   J Child Neurol  online.

Abstract

Acute cerebellitis is a rare inflammatory condition. It may have a benign, self-limiting course or present as a fulminant disease resulting in severe cerebellar damage or even sudden death. We present the clinical, laboratory, and radiologic data in 9 children diagnosed with acute cerebellitis, who were identified by database search in our pediatric medical center from January 2000 to November 2014. The main presenting symptom was headache, and the main presenting sign was ataxia. Bilateral diffuse hemispheric involvement was the most common imaging finding at presentation. Mycoplasma pneumoniae was the most common infectious pathogen found. Treatment included steroids in all cases, antibiotics in 4, and intravenous immunoglobulins in 6. Six patients had a full recovery, and 3 had residual neurologic complications. Magnetic resonance imaging (MRI) is the modality of choice for diagnosis. The course of acute cerebellitis varies from a commonly benign and self-limiting disease to an occasionally fulminant disease, resulting in severe cerebellar damage or sudden death.

From the article:

Acute cerebellitis is a rare disease that poses both diagnostic and therapeutic challenges. The clinical manifestations at presentation are variable and nonspecific, including nausea, vomiting, headache, fever, seizures, and altered mental status.  In the present cohort of 9 children diagnosed with cerebellitis at a single pediatric medical center over a 15-year period, headache was the most common symptom at presentation and ataxia was the most common sign (6 patients each). Meticulous physical examination is extremely important, as it may yield more specific findings such as ataxia, nystagmus, dysarthria, dysmetria, and tremor.  Laboratory findings are usually not contributory . There are no specific indices of the diagnosis in blood chemistry, and markers of infection may be absent. Cerebrospinal fluid test may yield normal-range results (as in patient 1) or reveal pleocytosis and may assist with diagnosis, by revealing a causative infectious pathogen.  In some cases, lumbar puncture is not conducted because of a suspected increase in intracranial pressure…

Imaging of the brain is essential for the diagnosis of cerebellitis. MRI is the modality of choice, as brain computed tomographic scan may be normal in the early stage of the disease.  Several patterns of imaging findings have been described in acute cerebellitis. Imaging patterns vary widely.  In only 3 of our patients did we find the most frequently reported imaging picture of bilateral symmetric hyperintensity on T2-weighted and fluid-attenuated inversion recovery images  whereas 4 had unilateral involvement, reported less often, including 2 with an additional focus in the vermis or in the other hemisphere. One patient demonstrated isolated involvement of the vermis , and 1 had small defined bilateral foci …

There are no consensus guidelines for the management of acute cerebellitis. Most cases of acute cerebellitis are self-limited and do not require treatment. Antimicrobial therapy should always be considered, because ataxia can be a presenting sign of both viral encephalitis and bacterial meningitis. The use of steroids is controversial. Göhlich-Ratmann et al reviewed the outcome of 7 patients with acute cerebellitis: all 3 treated with high-dose recovered completely, whereas the 4 who did not either died or suffered from sequelae. There are a few case reports of patients with parainfectious cerebellitis in whom steroid treatment may have improved long-term outcome and shortened disease duration. The authors suggested that the corticosteroids act by inhibiting the autoimmune cascade and stabilizing the blood-brain barrier.  All our patients were empirically treated with steroids. Seven also received antibiotics (azithromycin or doxycycline). Four patients were treated with intravenous immunoglobulin. The administration of intravenous immunoglobulin in our cases was prompted by reports of a successful outcome of immune-modulating therapy in patients with cerebellar ataxia.

Childhood acute cerebellitis generally has a good outcome. Most patients (50% to 86%) show full clinical recovery.  Reported long-term cognitive sequelae include poor spatial visualization and decreased language skills and concentration.34⇓-36 Mild but persistent cerebellar symptoms, such as dysmetria, intentional tremor, and ataxia, have been observed in 10% to 50% of patients.  In the present study, 3 of the 9 children (nos. 1 to 3) were neurologically impaired on follow-up, both verbally and functionally.