[CIS PIDD] [cis-pidd] HHV6 meningoencephalitis

[Church, Joseph]

Consider giving VZV vaccine first. You have an effective therapy (acyclovir) that should cover this attenuated virus if the should develop chicken pox.  If she tolerates VZV then you might consider MMR.



Joe Church

Children's Hospital Los Angeles

________________________________

From: Maite de la Morena [Maite.delaMorena at UTSouthwestern.edu]
Sent: Saturday, June 20, 2015 10:19 AM
To: CIS-PIDD
Subject: RE: [cis-pidd] HHV6 meningoencephalitis

Thank you Tom for your comments.
HHV6 was identified in blood  and CSF during acute event, I am waiting for all of the inpatient records.

While rare, STAT2 and MCM4 came to mind due to their variable phenotypic  expression (note: in my patient there is no evidence of consanguinity and no evidence of adrenal insufficiency documented)
Based on normal Igs, IgG to HHV6 and normal specific responses to vaccines I may guessing that adaptive immunity is probably normal
Unfortunately I don’t have good evidence that she has been exposed to other herpes viruses, which is why it make me nervous to proceed with live vaccines. Had she had evidence of CMV, EBV or HSV without consequences, I would feel more comfortable trusting T cell numbers and in vitro function. Instead, if the defect is early in innate response, the consequences of such defect may manifest before effective ab production

Sequencing these genes are not available clinically, I was hoping someone would be working on them
Thanks
Maite

From: Boyce, Thomas G., M.D. [mailto:Boyce.Thomas at mayo.edu]
Sent: Saturday, June 20, 2015 9:10 AM
To: CIS-PIDD
Subject: Re: [cis-pidd] HHV6 meningoencephalitis

Dear Maite,

It is very difficult to know if HHV-6 is the cause of encephalitis as asymptomatic reactivation is common. Primary infection occurs before age 2 in the vast majority of children. I would consider testing for the various causes of autoimmune encephalopathy and other progressive neurological conditions.

Personally, I would not hesitate to give live vaccines to a child with normal T cell counts and function.

Tom Boyce
Peds ID
Mayo Clinic


On Jun 19, 2015, at 4:55 PM, Maite de la Morena <Maite.delaMorena at UTSouthwestern.edu<mailto:Maite.delaMorena at UTSouthwestern.edu>> wrote:
Dear all
I have seen a 4 yof who developed HHV6 meningoencephalitis at age 3 and now has seizures, now severe developmental delay.
Had been achieving milestones appropriately until then

Negative serologies for CMV, EBV, HSV but + IgG for HHV6 (Negative IgM),
serum Igs and specific response to vaccines normal, Normal flow and normal mitogens and antigens .
Normal perforin, NK function and CTLs and TLR signaling ( ARUP )

She has not received MMR or varicella. I am hesitant to give these.
Would appreciate your comments
Thank you
Maite

Maite de la Morena, MD
Professor of Pediatrics  and Internal Medicine
Division of Allergy and Immunology
University of Texas Southwestern Medical Center in Dallas
5323 Harry HInes Blvd
Dallas, Texas 75390-9063
Phone 214 456-5161
Fax: 214 456-8317
Email: maite.delamorena at utsouthwestern.edu<mailto:maite.delamorena at utsouthwestern.edu>


________________________________

UT Southwestern


Medical Center



The future of medicine, today.


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