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

Sat May 9 06:35:47 EDT 2015

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>
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  /  34660psp at comb.cat Web: www.upiip.com ORCID ID:
> http://orcid.org/0000-0002-0346-5570 Scopus Author ID:
> http://www.scopus.com/authid/detail.url?authorId=55923378300 ResearchGate:
> http://www.researchgate.net/profile/Pere_Soler-Palacin LinkedIn:
> http://es.linkedin.com/pub/pere-soler-palac%C3%ADn/73/918/b16
>
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>
> ------------------------------
> *De: *"Richard Wasserman" <drrichwasserman at gmail.com>
> *Per: *"CIS-PIDD" <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> 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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-- 
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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