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

[João Farela Neves]

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

E-mail 2: jpfn13 at gmail.com

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