Case of acute cerebellar ataxia associated with primary Epstein–Barr virus infection

Abstract

Key words acute cerebellar ataxia, Epstein-Barr virus, infectious mononucleosis, T cell receptor Vb repertoires.Most primary Epstein-Barr virus (EBV) infections are asymptomatic; however, such infections can cause infectious mononucleosis (IM), which is characterized by a prolonged fever, cervical lymphadenopathy, hepatosplenomegaly, and peripheral lymphocytosis with atypical lymphocytes. IM is sometimes associated with neurological complications, including meningitis, encephalitis, acute demyelinating encephalomyelitis, cranial nerve palsy, cerebellitis, myelitis, and visual seizure with metamorphopsia, that is, "Alice in Wonderland" syndrome. 1,2 Here we describe a case of acute cerebellar ataxia associated with a primary EBV infection and discuss its pathogenesis. Case ReportA 7-year-old boy developed fever and a sore throat and visited a local hospital, where antibiotics (cefditoren pivoxil) were administered. Three days later he had skin eruptions on his hips and thigh. The eruption thereafter expanded over his face and back, but subsided within a few days. Eight days later, he presented with ataxia and a gait disturbance. On the next day, he also had dysphasia and was admitted to Toyama University Hospital. His medical history included bronchial asthma at 3 years of age, Kawasaki disease at 4 years of age and allergic rhinitis at 7 years of age.A physical examination at the time of his admission revealed the patient to be febrile, but his consciousness was alert. A general examination showed bilateral tonsillitis, but no lymphadenopathy or hepatosplenomegaly. A neurological examination revealed the deep tendon reflexes to be diminished and the Babinski reflex was negative. He showed a normal finger-nose test and no tremors, but ataxia in his trunk and a gait disturbance were observed. In addition, a speech disturbance was observed.Laboratory studies showed mild leukocytosis (9470/mL) with 46.5% neutrophils, 2% eosinophils, 4.5% monocytes, 37% lymphocytes, and 9% atypical lymphocytes. Other studies including electrolytes and liver function were normal. No Streptococcus pyogenes or adenovirus were detected with a throat swab culture. Cerebrospinal fluid (CSF) analysis revealed 1/3 leukocytes/mL, a protein of 20.1 mg/dL and a glucose of 61 mg/dL. A serological test for EBV showed viral capsid antigen (VCA)-immunoglobulin (Ig) G of 1 : 1280, VCA-IgM of 1 : 160, early antigen (EA)-IgG < 1 : 10, EA-IgM < 1 : 10, EBV nuclear antigen <1 : 10, thus indicating a primary EBV infection. A polymerase chain reaction (PCR) analysis for EBV was positive (521 copies/mL) in the serum, but negative in the CSF. A lymphocyte subset analysis showed an increased number of human leukocyte antigen-DR-positive (activated) CD3 + T cells (55%) and an inverted CD4/CD8 ratio (9.6%/54% = 0.18), thus suggesting a primary EBV infection.Computed tomography (CT) and magnetic resonance imaging (MRI) of the brain revealed no abnormalities. Brain singlephoton emission computed tomography (SPECT) showed a mild hypoperfusion in the bilateral cere...

Early acute cerebellar ataxia after meningococcal B vaccine: a case report of a 7-month-old infant and a review of the literature

“…1994 [28} 40 14 (35%) 26 (65%) 0 to 18 N.A 6.6 Yes 1 24 Not specified Harai T. et al . 2010I [ 29 ] 1 1 (100%) 0 (0%) 7 7 7 No / / / Hata A. et al . 2008 [ 30 ] 2 1 (50%) 1 (50%) 1.3 1.3 1.3 No / / / Hennes E. et al .…”

Case Report of Subacute Cerebellar Ataxia of Adolescence With Long-Term Sequelae

“…34 The Miller-Fisher variant has been reported in patients 2 to 86 years old with onset of ataxia, ophthalmoplegia, areflexia, and presence of IgG anti-GQ1b antibodies. 19 ,34…”

“…21 It is out of the scope of the current article to enumerate all agents of postinfectious acute cerebellar ataxia, but consideration in diagnosis may be lent to measles, mumps, herpes simplex virus, coxsackie A and B, hepatitis A, influenza A and B, parvovirus B19, echoviruses, poliovirus, Mycoplasma pneumoniae, and Legionella pneumophilia. [18][19][20][21]24 Additional etiologies that may present with acute cerebellopathies include tick paralysis, 25 cerebellar tuberculomas, 26 and brain stem encephalitis caused by Epstein-Barr virus, Listeria monocytogenes, or enterovirus 71 infection. 19 Acute disseminated encephalomyelitis is another autoimmune phenomenon typically preceded by infection that affects the entire brain and not only the cerebellum.…”

Postinfectious Acute Cerebellar Ataxia in Childhood

“…It is characterized by the sudden onset of ataxia following a viral infection, usually varicella [2,3]. However, other infectious agents including Epstein Barr virus (EBV), mumps, Legionella pneumophila, hepatitis A, influenza, herpes simplex, enterovirus, parvovirus B19, rubeola, and mycoplasma pneumoniae are also associated with acute cerebellar ataxia [1,4]. Acute cerebellar ataxia usually results from postinfectious cerebellar demyelination; it less commonly occurs as a result of direct infection of the cerebellum.…”