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

Mon May 11 15:47:19 EDT 2015

We had two children with Omaya reservoirs placed.  Removed 10 mL CSF and placed 10 mL 10% IVIg daily, back in the 1980s, virus was not classified at that time.  Peripheral IVIg dose was increased to 1 g/kg every two weeks.

If I remember correctly, we used the Omaya for about three months, with daily use (except maybe skipping a weekend day)…but left in place to see how symptoms went for another 6 months or so.  Then removed.  One patient developed hydrocephalus and required a shunt…this was not apparent while we were using the Omaya reservoir, perhaps due to some removal of CSF and that the IVIg was otherwise well-absorbed(?).

During the several years I had subsequently seen them…no obvious neurologic problems.

Again, these were young children (under 10 years of age).

Terry Harville MD PhD D(ABMLI) D(ABHI)
*Medical Director, Special Immunology Laboratory
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Immunodeficiencies, and Organ Transplantation Immunology
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– specializing in diagnostic evaluations of immunodeficiencies
*Diplomate of the American Board of Histocompatibility and Immunogenetics
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disease associations, and platelet transfusion support

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From: João Farela Neves [mailto:jpfn13 at gmail.com]
Sent: Saturday, May 9, 2015 06:03 AM
To: CIS-PIDD
Subject: Re: [cis-pidd] Chronic meningitis in ar-agammaglobulinemia

Thanks for your replies

Her trough levels are >17, already... (At least since February)

She has severe hydrocephaly and she is very symptomatic... We need to insert a csf draining device.

We planned ommaya to be placed next week but your experiences are not brilliant... Anyone else with different experiences?

Do you think it would be best to simply insert an external drainage while we try to identify the bug?

In the meanwhile We have identified tropheryma whipplei in the stools. PAS colorations and PCR in the duodenum are pending. Has anyone seen whipple's cns involvement in agammaglobulinemia?

Thanks for you precious help
Regards
João F Neves

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<tel:%2B351%20213126600>

Fax:+351 213126963<tel:%2B351%20213126963>

E-mail 1: joao.farelaneves at chlc.min-saude.pt<mailto:joao.farelaneves at chlc.min-saude.pt>

E-mail 2: jpfn13 at gmail.com<mailto:jpfn13 at gmail.com>

No dia 09/05/2015, às 11:35, Richard Wasserman <drrichwasserman at gmail.com<mailto:drrichwasserman at gmail.com>> escreveu:
My older patient did well with IGIV when his IgG levels were >1500 mg/dL
Richard Wasserman

On Sat, May 9, 2015 at 4:25 AM, Pere Soler Palacin <psoler at vhebron.net<mailto:psoler at vhebron.net>> wrote:
Dear all, few years ago we had a similar XLA patient with enteroviral meningitis presenting as progressive dementia. We tried high dose IVIG (Ig levels always above 1000 mg/dl) without significant clinical response. Unluckily, when an Ommaya was placed CT showed massive bleed and the patient developed severe neurological sequeale. He's still alive but in a persistent vegetative state.
Pere.

Pere Soler Palacín, MD, PhD.
Pediatric Infectious Diseases and Immunodeficiencies Unit. Hospital Universitari Vall d'Hebron
Assistant Professor. Universitat Autònoma de Barcelona (UAB)
Pg. de la Vall d'Hebron, 119-129
08035 Barcelona. Spain.
Tel. 0034934893140  /  Fax 0034934893039

psoler at vhebron.net<mailto:psoler at vhebron.net>  /  34660psp at comb.cat<mailto:34660psp at comb.cat>
Web: www.upiip.com<http://www.upiip.com/>
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________________________________
De: "Richard Wasserman" <drrichwasserman at gmail.com<mailto:drrichwasserman at gmail.com>>
Per: "CIS-PIDD" <cis-pidd at lyris.dundee.net<mailto:cis-pidd at lyris.dundee.net>>
Enviats: Divendres, 8 de Maig 2015 16:24:59
Assumpte: Re: [cis-pidd] Chronic meningitis in ar-agammaglobulinemia

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<mailto: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
------------------------------------------------------------------------

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saude.pt<mailto:joao.farelaneves at chlc.min-saude.pt>

E-mail 2: jpfn13 at gmail.com<mailto:jpfn13 at gmail.com>

No dia 04/05/2015, às 20:24, Osman C Dokmeci <cdokmeci at gmail.com<mailto: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<mailto: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<tel:%2B351%20213126600>

Fax:+351 213126963<tel:%2B351%20213126963>

E-mail 1: joao.farelaneves at chlc.min-saude.pt<mailto:joao.farelaneves at chlc.min-saude.pt>

E-mail 2: jpfn13 at gmail.com<mailto:jpfn13 at gmail.com>

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