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Indian Pediatr 2013;50: 890 |
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Fulminant Epstein Barr Virus Encephalitis–Can
it be Due to X-linked Lymphoproliferative Disease
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JB Ghosh
Consultant Pediatrician, Ex-Professor and HOD, IPGME
and R, Kolkata, Ushashi Housing Society, 245 Vivekananda Road,
Kolkata 700 006, West Bengal.
Email: jbghosh@yahoo.com
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I read with great interest the recently published article "Fulminant
Epstein Barr Virus Encephalitis" [1]. Infectious mononucleosis
(IM) is usually a common benign illness caused by Epstein Barr Virus
(EBV). I fully agree that neurological manifestations in IM are
extremely rare. It may be the presenting feature or occur as a result of
complication usually appearing after 1-3 weeks of illness. The authors
described two such cases with neurological manifestations as presenting
feature in absence of classical signs of IM like sore throat, skin rash,
lymphadenopathy, hepatomegaly, splenomegaly. I feel the authors should
have considered the background illness i.e. X-linked
lymphoproliferative (XLP) disease referred to as Duncan disease, a
combined immunodeficiency disease that manifest after exposure to EBV.
Boys with XLP mutation remain clinically well before exposure to EBV.
Exposure to EBV results in fulminant and fatal IM in 60% of cases [2].
The fulminant course ultimately leading to death within 8 weeks after
onset of IM in 95% of cases [3]. The fulminant nature of the course of
EBV infection in both the cases should draw attention to the possibility
of XLP disease. This may also be further supported by the fact that both
the cases were boys. There is marked impairment in production of
antibodies to the EBV nuclear antigen (EBNA),where as titres of
antibodies to viral capsid antigen (VCA) have ranged from absent to
markedly elevated [4]. Authors have mentioned that in first case EBV
serology was positive in blood and in second case IgM EBV was positive
in blood. It is not clearly understood what type of serology was
undertaken. Although patients with XLP disease have normal number of T
lymphocyte CD 8 + T cell
predominate with inversion of normal CD 4 – CD 8 ratio [3].CD 4+,
CD 8+ lymphocyte count and
its ratio study also could have been done in the index cases. Genetic
study for detection of SAP (for SLAM i.e. signaling lymphocyte
activation molecule.- associated protein) now known as SH2D1A may be
done if facilities permit [4].
References
1. Mathew AG, Parvez Y. Fulminant Epstein Barr virus
encephalitis. Indian Pediatr. 2013;50:418-9.
2. Conley ME, Notarangelo L, Etzinoni E. Diagnostic
criteria for primary immunodeficiencies. Clin Immunol. 1987;93:190.
3. Schuster V, Kreth HW: X-linked lymphoproliferative
disease. In Ochs HD, Smith CIE, Puck JM (eds): Primary
Immunodeficiency Diseases. New York, Oxford University Press,1999.
p.222-32.
4. Buckley RH. Primary Defects of Antibody Production.
In: Stanton BF, Schor NF, St. Geme 111 JW, Behrman RE, in Nelson
Textbook of Pediatrics.19th ed, Philadelphia: WB Saunders
Co;2012;118;722-27.
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