[CIS PIDD] [cis-pidd] Chronic meningitis in ar-agammaglobulinemia

Fri May 8 10:24:59 EDT 2015

I have experience with two XLA patients with Echo 11 CNS infection.

The first presented in early childhood to Diane Wara in San Francisco. He
was initially treated with IGIV but did not improve and an Ommaya resevoir
was placed. He received IgG infusions several times a week and improved. He
then developed non-communicating hydrocephalus and symptoms of spinal cord
disease. Another resevoir was place in the lumbar region and he was treated
through both ports and improved. The family then moved to Dallas and I
began caring for him. Over time, we were able to decrease and finally
discontinute intraventricular and intrathecal IgG. He did not have a
recurrance of Echo 11 positivity. His peripheral IgG levels were always
>1000mg/dL. In his teenage years he developed crippling paresis and
contractures and died in his late 20's.

The second came to me at age 35 with chronic liver disease and wasting. He
had been initially treated with IGIM and then FFP. When he grew Echo 11
from stool and LP was performed. There was no evidence of meningitis but
the CSF grew Echo 11. Based on patient one, I had an Ommaya placed and
began intraventricular IgG. A few hours after the first dose he developed
neurologic changes and a CT showed a small bleed. Intraventricular IgG was
stopped and he received high dose IGIV every two weeks. Shortly thereafter
he received a liver transplant. Several years later he was doing well on
home IGIV and decreased his dose. He developed a seizure and CSF was
positive for Echo 11. IGIV was incresed and Echo 11 became undetectable.
There were no further Echo 11 problems until his death 15 years later from
chronic rejection.

Based on these experiences, I would push the IV dose hard before placing a
resevoir for intraventricular/intrathecal IgG therapy. Good luck.
Richard Wasserman
Dallas

On Wed, May 6, 2015 at 5:45 AM, Joao Neves <jpfn13 at gmail.com> wrote:

> Thank you for the reply.
>
> Toscana will be searched in the biopsy and CSF. He is not on chronic
> SM-TMP nor NSAID.
>
> Any other thoughts?
>
> João FN
> ------------------------------------------------------------------------
>
> João Farela Neves, MD
>
> Infectious Diseases Unit
>
> Primary Immunodeficiencies Unit
>
> Clinical Immunology Working Party
>
> Hospital Dona Estefania, Pediatric University Hospital
>
> Rua Jacinta Marto, 1169-045
>
> Lisbon, Portugal
>
> Tel: +351 213126600
>
> Fax:+351 213126963
>
> E-mail 1: joao.farelaneves at chlc.min-saude.pt
>
> E-mail 2: jpfn13 at gmail.com
>
> No dia 04/05/2015, às 20:24, Osman C Dokmeci <cdokmeci at gmail.com>
> escreveu:
>
> Hi Dr. Neves,
>
> Did you consider for Toscana Virus meningitis, or drug related causes
> (TMP-SMX) or NSAIDs?
> Sometimes it escapes one's mind to look for these.
>
> Osman C. Dokmeci, M.D.
>
>
>
> On May 4, 2015, at 2:37 PM, João Farela Neves <jpfn13 at gmail.com> wrote:
>
> Hello all
>
> We've been asked to help a 16 YO female patient with agammaglobulinemia
> (ar, mu chain def). She is suffering from chronic meningitis and
> myelo-radiculitis.
> "Standard" microbiologic procedures have failed to identify the causative
> organism in CSF/stools/blood (Including culture, PCR for virus and bacteria
> + 16S PCR)
>
> Apparently she had Enteroviral meningitis in 2009 (fever+headaches+ CSF
> with pleocytosis and EV PCR +). Her doctors increased her IgG trough levels
> (>14) and her symptoms subsided. Since June 2014 her clinical condition has
> been deteriorating. In brief, her MRI reveals leptomeningitis,
> decompensated hydrocephalus because of decreased CSF absorption, and
> myelitis. She has severe headaches, difficulty walking (pyramidal signs and
> hypertonia) and has developed neurogenic bladder. She has persistent
> pleocytosis (lymph) and Enterovirus PCR is negative (5x). All other PCR and
> cultures are negative.
>
> We have seen her last week and are planning brain biopsy to try to
> identify the micro-organism.
>
> We are seeking your help because:
> 1-We need to send samples (brain biopsy + CSF) to a lab that is able to
> perform NGS for microbiologic identification. Can anyone help us with this?
>
> 2- We need to treat her hydrocephaly. We are favouring a Ommaya reservoir
> placement. Do you agree? Would you attempt intra-techal IgG administration
> through Ommaya’s reservoir? If you do, what would the posology be?
>
> 3- If we don’t succeed in the identification of the bug, would you attempt
> empirical treatment with alpha-IFN? With or without ribavirin?
>
> 4- Other thoughts?
>
> Thanks in advance
> Regards
> João FN
> ------------------------------------------------------------------------
>
> João Farela Neves, MD
>
> Infectious Diseases Unit
>
> Primary Immunodeficiencies Unit
>
> Clinical Immunology Working Party
>
> Hospital Dona Estefania, Pediatric University Hospital
>
> Rua Jacinta Marto, 1169-045
>
> Lisbon, Portugal
>
> Tel: +351 213126600
>
> Fax:+351 213126963
>
> E-mail 1: joao.farelaneves at chlc.min-saude.pt
>
> E-mail 2: jpfn13 at gmail.com
>
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-- 
Richard L. Wasserman, MD, PhD
Allergy Partners of North Texas
7777 Forest Lane, Suite B-332
Dallas, Texas 75230
Office (972) 566-7788
Fax (972) 566-8837
Cell (214) 697-7211

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