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

[Osman C Dokmeci]

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? 
> 
> 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 <mailto:joao.farelaneves at chlc.min-saude.pt>
> 
> E-mail 2: jpfn13 at gmail.com <mailto:jpfn13 at gmail.com>
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