A New Complication of Stem Cell Transplantation: Measles Inclusion Body Encephal

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http://pediatrics.aappublications.org/cgi/content/abstract/114/5/e657

A New Complication of Stem Cell Transplantation: Measles Inclusion Body

Encephalitis

Measles inclusion body encephalitis (MIBE) is a disease of the

immunocompromised host and typically occurs within 1 year of acute measles

infection or

vaccination. We report a 13-year-old boy who had chronic granulomatous disease

and

presented 38 days after stem cell transplantation with afebrile focal

seizures that progressed despite multiple anticonvulsants. After an extensive

diagnostic evaluation, brain biopsy was performed, revealing numerous

intranuclear

inclusion bodies consistent with paramyxovirus nucleocapsids. Measles studies

including reverse transcriptase-polymerase chain reaction and viral growth

confirmed measles virus, genotype D3. Immunohistochemistry was positive for

measles

nucleoprotein. Despite intravenous ribavirin therapy, the patient died. MIBE

has not been described in stem cell recipients but is a disease of

immunocompromised hosts and typically occurs within 1 year of measles infection,

exposure, or vaccination. Our case is unusual as neither the patient nor the

stem cell

donor had apparent recent measles exposure or vaccination, and neither had

recent travel to measles-endemic regions. The patient had an erythematous rash

several weeks before the neurologic symptoms; however, skin biopsy was

consistent with graft-versus-host disease, and immunohistochemistry studies for

measles nucleoprotein were negative. As measles genotype D3 has not been seen in

areas where the child lived since his early childhood, the possibility of an

unusually long latency period between initial measles infection and MIBE is

raised. In addition, this case demonstrates the utility of brain biopsy in the

diagnosis of encephalitis of unknown cause in the immunocompromised host.

andra

F. Freeman, MD*, A. sohn, MD, ScM, Stanford T. Shulman, MD*,

J. Bellini, PhD, Preeti Jaggi, MD*, Guillermo de Leon, MD||, Gesina F.

Keating, MD¶, Francine Kim, MD#, M. Pachman, MD*, Kletzel, MD and

Reggie E. Duerst, MD

* Division of Infectious Disease, Department of Pediatrics

Division of Hematology/Oncology/Transplant, Department of Pediatrics

|| Department of Pathology

¶ Division of Neurology

# Division of Diagnostic Imaging

** Division of Immunology, Northwestern University Feinberg School of

Medicine and Children's Memorial Hospital, Chicago, Illinois

Centers for Disease Control and Prevention, Atlanta, Georgia

Key Words: measles • encephalitis • immunocompromised host

Abbreviations: MIBE, measles inclusion body encephalitis • CGD, chronic

granulomatous disease • SCT, stem cell transplantation • CMV,

cytomegalovirus •

ATG, anti-thymocyte globulin • GVHD, graft-versus-host disease • CsA,

cyclosporine • WBC, white blood cell • MRI, magnetic resonance imaging •

PCR,

polymerase chain reaction • HSV, herpes simplex virus • RT-PCR, reverse

transcriptase

PCR

accepted May 25, 2004.