[CIS PIDD] [cis-pidd] Chronic meningitis in ar-agammaglobulinemia
João Farela Neves
jpfn13 at gmail.com
Sat May 9 07:02:52 EDT 2015
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
>>>
>>> Fax:+351 213126963
>>>
>>> E-mail 1: joao.farelaneves at chlc.min-saude.pt
>>>
>>> E-mail 2: jpfn13 at gmail.com
No dia 09/05/2015, às 11:35, Richard Wasserman <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> 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
>>
>> No imprimir aquest correu ajudarà a preservar el medi ambient.
>> Si vostè no és el destinatari del missatge, o l'ha rebut per error, si us plau notifiqui-ho al remitent i destrueixi el missatge amb tot el seu contingut. Està prohibida la distribució no autoritzada del contingut d'aquest missatge.
>>
>>
>> No imprimir este correo ayudará a preservar el medio ambiente.
>> Si usted no es el destinatario del mensaje, o lo ha recibido por error, notifíquelo por favor al remitente y destruya el mensaje con todo su contenido. Está prohibida la distribución no autorizada del contenido de este mensaje.
>>
>> 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
>>> ------------------------------------------------------------------------
>>>
4001310079135012
>>> 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
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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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>
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