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Indian Pediatr 2013;50: 418-419 |
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Fulminant Epstein Barr Virus Encephalitis
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Aji George Mathew and Yusuf Parvez
From the Department of Pediatrics, Al-Jahra Hospital,
Kuwait.
Correspondence to: Dr Aji George Mathew,
Registrar Pediatrics, Pediatric Intensive Care Unit,
Al-Jahra Hospital, PO Box 40206,
Email: Kuwait. [email protected]
Received: August 19, 2012;
Initial review: September 24, 2012;
Accepted: October 22, 2012
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Epstein Barr virus (EBV) encephalitis
is rare in children but can have severe neurological complications and
sometimes fatal. It can manifest with varied neurological presentations
like meningoencephalitis, brain stem encephalitis, GBS etc. This can
appear alone or with clinical picture of infectious mononucleosis.
Establishing a diagnosis of EBV encephalitis is difficult and
consequently molecular, serological and imaging techniques should be
used when investigating a child with encephalitis. To highlight this
entity we report two fatal cases of EBV meningoencephalitis presenting
with sole neurological manifestations .
Key words: Encephalitis, Epstein Barr virus.
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EBV virus infection is a common
usually benign systemic viral illness in children. In few
cases it is associated with variety of CNS manifestations
including meningoencephalitis, cerebritis, transverse
myelitis, neuropsychiatric syndrome, GBS and cranial nerve
palsies.This occurs after usually 1-3 weeks of illness;
rarely these can manifest at the onset of illness.
Case 1: A 7-year-old boy was
admitted with history of fever, unsteady gait and drowsiness
of 2 days duration. He was found to have wide-based gait,
nystagmus and increasing drowsiness. Other system
examinations was unremarkable. There were no focal
neurological deficits, no seizures and no signs of raised
intracranial tension or signs of meningeal irritation.
Initial routine investigations and basic metabolic screen
were normal. CT brain also revealed no abnormality. CSF
analysis was done and microscopy and biochemistry was
normal. CSF PCR for EBV virus was positive and EBV serology
was also positive in blood. Immunological profile was
normal. He was treated as meningo-encephalitis with
broad-spectrum antibiotics and acyclovir. One day after
admission, he was found to have deteriorating level of
consciousness with low GCS and required mechanical
ventilation. He was not showing any improvement in his
neurological status, and he received intravenous
immunoglobulin empirically. MRI brain showed abnormal signal
intensity in pontine region suggestive of pontine
encephalitis. Child had persistent fever, his general
condition deteriorated and on 5 th
day of illness, he developed hypotension which was
refractory to fluids, with GI bleeding and DIC with
multiorgan failure and succumbed to the illness.
Case 2: An 8-year-old boy was
admitted with low grade fever and drowsiness of 2 days
duration.There was no history of convulsions or vomiting and
there were no signs of raised intracranial tension. Clinical
diagnosis of encephalitis was made at the time of admission
and other systemic examination was unremarkable. Initial
routine investigations and metabolic screen were normal. CT
brain was also normal. CSF biochemistry and microscopy were
normal. He was started on broad spectrum antibiotics
including antiviral drugs along with other supportive
treatment. He developed generalised tonic clonic seizures
within few hours of admission to the hospital, which was
initially controlled by benzodiazepines, but later
progressed to status epilepticus. He was ventilated and
started on midazolam infusion. Seizures became more frequent
and refractory to maximum dose of benzodiazepines. It was
only controlled by inducing barbiturate coma and by
thiopentone infusion. On second day, he deteriorated and
went into deep coma. CSF virology study revealed EBV DNA PCR
positive, and IgM EBV was positive in blood. MRI brain was
normal. He continued to have brief seizures requiring
multiple anticonvulsants. His general condition didn’t
improve and remained in deep coma, and also developed
diabetes insipidus. Brain perfusion scan showed absent
perfusion. On 5 th
day of admission, he developed bradycardia and hypotension,
and could not be revived despite resuscitative measures.
Discussion
Neurological manifestations are rarely
seen during EBV infection, usually in less than 1% of the
diseased [1,3]. Among these, meningitis and encephalitis
constitute the most common type of neurological
manifestations. The patients may have varied symptoms like
fever, seizure, bizarre behaviour, headache, and
metamorphopsia [3]. The presenting signs, included altered
consciousness, meningeal signs, bulbar signs cerebellar
signs and cranial nerve palsy [2,6]. In our cases the
classical features of infectious mononucleosis were obscure
and the patients presented only with the neurological
manifestations. The first case presented only with unsteady
gait with altered sensorium and cerebellar signs, which
turned out to be pontine encephalitis whereas the other
child presented with refractory seizures which required
barbiturate coma to control seizures and progressing to deep
coma. The neurological manifestations can occur 1 week to 3
weeks after the onset of infectious mononucleosis but may
occur at the outset of the disease. The pathogenesis of
these complications is still under debate. Studies suggest
that some complications are due to direct viral infection,
whereas others are due to autoimmune mechanisms. In contrast
to viruses that invade gray matter directly, acute
disseminated encephalitis and postinfectious
encephalomyelitis associated with Epstein-Barr virus (EBV)
and CMV infections are immune-mediated processes that result
in multifocal demyelination of perivenous white matter [3].
Neurologic complications are the most common cause of death
in infectious mononucleosis, but also can occur in the
absence of clinical or laboratory manifestations of
infectious mononucleosis. Establishing a diagnosis of
Epstein-Barr virus encephalitis can be difficult, and
consequently, a combination of serologic and molecular and
radiologic techniques should be used when investigating a
child with acute encephalitis [7,8].
Contributors: All authors designed,
supervised and analyzed the study, and prepared the
manuscript.
Funding: None; Competing interests:
None stated.
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