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Acute cerebellar ataxia is a rare manifestation of Epstein–Barr virus (EBV) infection. We present a case of a 17-year-old male who presented with acute cerebellar ataxia occurring before the onset of tonsillitis and lymphadenopathy. Serological testing confirmed acute EBV infection.
A 17-year-old male college student complained of feeling ‘drunk’ and unsteady when walking. This progressed over a few days so that he could only crawl. By this time he had developed a sore throat and persistent nausea and vomiting. His general practitioner prescribed penicillin, without effect. On day 6, he presented to hospital bed-bound due to severe loss of balance with severe nausea and vomiting.
He was afebrile (37.2°C) but cardiovascular and respiratory examinations were normal. He had bilateral cervical lymphadenopathy and tonsillar enlargement, and there was no hepatosplenomegaly. He was dysarthric with truncal ataxia and dysmetria in all four limbs. He had no nystagmus.
His serum C reactive protein was raised at 24 mg/l (<10). Serum urea and electrolytes, full blood count and liver function tests were normal. Lymphocytosis developed on day 8, with reactive lymphocytes on a blood film. C reactive protein rose steadily and peaked at 88 mg/l on day 10, and then gradually returned to normal. Liver function tests showed hepatitis with serum alanine aminotransferase rising to 268 U/l (5–35) before normalising. His MR scan of brain showed incidental congenital lateral ventricle asymmetry but was otherwise normal; in particular, there was no cerebellar enhancement or swelling. Cerebrospinal fluid (CSF) analysis is shown in table 1.
Serum EBV viral capsid antigen (VCA) immunoglobulin M (IgM) was significantly raised (>160 units/ml), EBV VCA IgG was also raised (119 units/ml) and EBV nuclear antigen IgG was negative (<3 units/ml), in keeping with acute EBV virus infection. Serology for cytomegalovirus, mumps, HIV, hepatitis A, B and C, Legionella and Mycoplasma pneumonia was negative.
His fever was settled within a few days of his admission. His cerebellar ataxia, dysarthria and nausea and vomiting continued and, on day 18, when afebrile, he was given methylprednisolone 1 g intravenously for 3 days. He had several weeks of intractable nausea and vomiting for which he required total parenteral nutrition. He improved slowly, and by 4 weeks after the onset of the illness, his speech had improved and he was able to stand and walk with help. Following discharge, he improved further and now he walks independently.
The differential diagnosis of acute cerebellar ataxia in a young adult includes vascular, inflammatory, toxic, neoplastic, paraneoplastic, genetic and infective causes (table 2). In this case, an adolescent developing tonsillitis shortly after the onset of ataxia raised the likelihood that EBV was the causative agent; this was subsequently confirmed on serological testing.
Acute cerebellar ataxia is a rare presentation of EBV infection and can occur without the systemic manifestations of EBV.1 In a review of acute cerebellar ataxia due to EBV, cerebellar ataxia was the sole manifestation in five out of the 16 patients.1 The age of onset ranged from 4 to 26 years (with the exception of a 52-year-old man) and there was a male predominance (12 of 16 cases). Patients had either a mild CSF lymphocytic pleocytosis (up to 44 cells/mm3) or no white blood cells in the CSF. All patients who were followed up had recovered within 3–13 weeks.1
It is not clear if the neurological manifestations of EBV result from direct tissue invasion or from a postinfectious autoimmune phenomenon. In our case, the neurological symptoms preceded the systemic features of EBV infection by several days, suggesting that they resulted from the acute infection rather than being a postinfectious phenomenon. EBV and its antibodies are often not found in the CSF (as in our case): in one case, however, EBV was detected directly in CSF cells.1,–,4
Although most cases of EBV cerebellitis have a benign outcome, there are several reports of life-threatening cerebellar swelling with acute cerebellitis from EBV. 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. However, MRI may identify patients at risk of developing acute hydrocephalus and allow early intervention to prevent herniation. The number of patients treated with corticosteroids is too small to know whether they help. 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
In summary, we present an adolescent with acute cerebellar ataxia, which preceded the systemic manifestations of EBV infection. Neurological complications of EBV are rare, but also include Guillain–Barré syndrome, meningitis, encephalitis, cranial nerve palsies, optic neuritis and transverse myelitis.8 EBV can cause cerebellitis even without its systemic manifestations. The diagnosis is based on finding EBV VCA IgM antibodies in serum rather than CSF. In most cases, the prognosis is good, with complete recovery; however, clinicians must be aware of the risk of cerebellar swelling and hydrocephalus.
Epstein–Barr virus (EBV) can cause acute cerebellar ataxia in young adults.
Cerebellar ataxia can arise from EBV even where there are no systemic manifestations of infection.
Cerebrospinal fluid can be negative for EBV and its antibodies: the diagnosis is made from EBV viral capsid antigen immunoglobulin M in serum.
The prognosis for full recovery from EBV cerebellitis is generally good, but occasionally there is life-threatening cerebellar swelling and hydrocephalus.
Patient consent Obtained.
Provenance and peer review Not commissioned; externally peer reviewed.
This paper was reviewed by Brendan McLean from The Royal Cornwall Hospital, Turo, Cornwall.