Acute cerebellitis with tonsillar herniation and hydrocephalus in Epstein-Barr virus infection

Abstract

A 9-year-old girl was admitted with headache, photophobia, diplopia and vomiting 12 days after the onset of infectious mononucleosis (IM). She was afebrile. Neurological examination revealed nuchal rigidity, cerebellar ataxia with bilateral dysmetria, paresis of the VI right cranial nerve and drowsiness. A CT scan was normal, as was the fundus examination. An EEG showed slight diffuse slowing.CSF examination disclosed pleocytosis with 72 white blood cells/mm 3 (80% lymphocytes). CSF-PCR for EBV, herpes viruses, varicella-zoster virus, and cytomegalovirus were negative. Bacterial cultures were negative. Blood cell counts and coagulation studies were normal; AST and ALT were 158 U/l and 363 U/l respectively. Serology showed IgG and IgM antibodies against EBV-capsid antigen. After an initial improvement of the meningeal symptoms, on day 16 left facial palsy, muscle twitching and lethargy were observed. A repeat CT scan showed mild third and lateral ventricular enlargement and moderate reduction of the prepontine cistern. Intravenous (IV) dexamethasone (0.08 mg/kg every 6 h) was started, but 2 days later the girl developed obtundation and irregular breathing. MRI showed marked cerebellar swelling and downward displacement of cerebellar tonsils, enlargement of the lateral and third ventricles and a clear signal increase in diffusion weighted (DW) images in the cerebellar cortex ( Fig. 1-A,B,C). There was no contrast enhancement. Mannitol (0.5 g/kg twice a day) as a 20% IV solution was added, followed by furosemide (0.7 mg/kg IV). The patient improved slowly and therapy was stopped after 12 days, dexamethasone being tapered over 15 more days. Three months later, neurological examination and MRI were normal.Neurological complications occur in 1% to 18% of patients admitted to hospital for IM, being sometimes the first and unique manifestations of EBV disease. The cerebellum is a predilection site for EBV but pathogenesis of cerebellitis is heterogeneous. Direct viral invasion and replication, an immuno-mediated post-infectious mechanism and liberation of an increased number of cytokines in the CNS can be involved in the process, and the distinction using clinical criteria is not always easy. Negative viral PCR assays cannot exclude CNS infections because there may be insufficient genome numbers in CSF and/or variations in virus genome. Both viral nucleic acids and antiviral antibodies have been isolated from the CSF of patients with acute cerebellar ataxia (ACA) and with acute disseminated encephalomyelitis following EBV infection. Auto-antibodies against white matter structures and myelin basic protein have been reported in some patients with ACA, suggesting a demyelinating process.A survey of the literature revealed six other paediatric cases of EBV-related severe cerebellitis (Table 1) [1,2,3,4,6]. Headache, vomiting and alterations in consciousness occurred 2-3 weeks after EBV infection, accompanied by ataxia, nystagmus, dysarthria and cranial nerve palsies. Severe sudden deterioration occurred as acute hydrocephalu...

Acute cerebellar ataxia due to Epstein–Barr virus

“…In these cases, an MR brain scan may show contrast enhancement or increased diffusion weighted signal in the cerebellar hemispheres, with subsequent fourth ventricle obstruction, hydrocephalus and transforaminal herniation 1 5 6. Our patient was alert and had a normal cerebellum on imaging.…”

“…In the review of Erzurum et al , all patients followed up had recovered within 3–13 weeks; corticosteroids had been given in 4 out of 16 cases, without a clear benefit 1. Corticosteroids also did not prevent one patient with severe cerebellar swelling from deteriorating 5. Where cerebellar signs develop weeks after the onset of the EBV infection, and therefore with probable postinfectious cerebellitis, plasma exchange may help 7…”

Epstein-Barr virus-associated cerebellar ataxia

“…Cerebellar swelling leading to obstructive hydrocephalus has been described. MRI scan and particularly DWI sequence (diffusion-weighted imaging) can be helpful to predict early cerebellar cytotoxic oedema and swelling 11 13. Treatment with corticosteroids has been used with doubtful benefit, the evidence behind which is very limited 11 13…”

“…MRI scan and particularly DWI sequence (diffusion-weighted imaging) can be helpful to predict early cerebellar cytotoxic oedema and swelling 11 13. Treatment with corticosteroids has been used with doubtful benefit, the evidence behind which is very limited 11 13Learning points

Neurological complications of Epstein-Barr virus (EBV) are rare, but recognised.

EBV-associated cerebellar ataxia should be considered in the differential diagnosis of acute cerebellar ataxia.

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Epstein-Barr virus encephalitis after kidney transplantation and successful treatment with brivudine

“…Neurological complications caused by EBV after transplantation normally appear within a few weeks or months and are rare [1,2]. They occur in 1–18%, and sometimes they are the first and unique manifestations of EBV infection [3]. The cerebellum is a predilection site for EBV infections [3].…”