[CIS-PAGID] infantile recurrent aseptic meningitis

[Seppänen Mikko]

Dear Megan,

Highly interesting! I presume that You checked that his CSF returns to normal in between, and HIV is excluded as well as Reye's? Though in FMF chronic meningitis is more common, in AIS outside cryopyrin-related disorders only FMF springs to my mind.


>From Inf Dis point of view, PMN pleocytosis is seen mostly in relapsing bacterial meningitis, and thus seems unlikely.  A disease like his (and no positive bacterial cultures) does not sound like anything related to states like meningocele, encephalocele, epidermoid cyst, dermoid cyst, sinus pilonidalis, neuroenteral cyst, inner ear malformation (like Mondin's dysplasia) nor neurofibromatosis type 1? Parameningeal infectious focus in inner ear remains an alternative? Nor does it then sound like a known PIDD (asplenia, complement terminal/alternative def, IRAK4/MyD88, where in all one would expect a +ive culture)?


Of other infectious causes (HSV-2, HSV-1, EBV, enteroviral) the pleocytosis would not remain neutrophilic in control CSF analysis, though might be neutrophilic at presentation. In drug-related cases (NSAIDs, carbamazepine, isoniazide, cotrim), the pleocytosis is normally lympho/monocytic, sometimes eosinophilic/PMN. Also in pleocytosis with migraine and with things like spontaneous intracranial hypotension (why would he have that???) the pleocytosis would be mostly monocytic.

Thus one (if no inner ear parameningeal focus)  is most probably left with idiopathic inflammatory (described in lupus = C4/C2/C1qrs defs excluded?, Sjogren, MCTD, Behcet, relapsing polychondritis, from below one notices that recurrent ADEM already excluded), uveal (Vogt-Koyanagi-Harada, neurological APMPPE, both in record young..., sarcoidosis/Blau?) or unknown AIS? Thinking of "true, non-herpetic" idiopathic Mollaret's he would also be record young, and one would like to see s.c. Mollaret's cells in CSF slides?

If his meningitis actually is chronic, then the differential broadens significantly to things like metabolic encephalopathies (eg Fabry's), TTP (platelets normal I presume, complement?),  and to ANCA-associated and large-vessel vasculitides (again a bit too young for those?). In diseases like Kawasaki, the pleocytosis is again usually lympho/monocytic.

There is also the entity "benign chronic aseptic meningitis", which when one reads the original articles is a truly heterogenous group, and one would presume that it is a group of yet undescribed (AIS?) diseases....

You have a new disease in your hands?

Hope this helps?

mikko

__________________________________________________
Mikko Seppänen, MD, PhD
Specialist in Internal Medicine and Infectious Diseases
Senior Consultant, Physician in charge (PIDD)
EM(E)A Expert, PIDDs and Intravenous Immunoglobulin Therapy

Immunodeficiency Unit
Division of Infectious Diseases
Department of Medicine
Helsinki University Central Hospital
Hospital District of Helsinki and Uusimaa
Aurora Hospital, Ward 4-2 and Outpatient Clinic
P.O.Box 348
FI-00029 HUS, Helsinki
FINLAND
phone +358 9 47175923, fax +358 9 47175945
_________________________________________

Lähettäjä: pagid-bounces at list.clinimmsoc.org [mailto:pagid-bounces at list.clinimmsoc.org] Puolesta Cooper, Megan
Lähetetty: 26. maaliskuuta 2012 21:04
Vastaanottaja: pagid at list.clinimmsoc.org
Aihe: [CIS-PAGID] infantile recurrent aseptic meningitis

Hi Everyone -

I have a challenging patient I was asked about.  This is a 3mo (former 36 week EGA) with recurrent aseptic meningitis.  He has now had 4 episodes plus a rule out sepsis evaluation as a newborn.  With these he has fever and irritability, CSF pleocytosis with neutrophil predominance (CSF nucleated cell counts 31-775), normal CSF protein/glucose, and on CBC elevated WBC and platelets.  He also has elevated monocytes on CBC (7-19%).  ESR/CRP were only checked once and were normal.  He has no rash, arthritis, or other symptoms.

Cultures have all been negative, although he seems to respond to antibiotics and had a normal CSF cell count after 1 week of abx with one of the episodes.  An extensive ID workup was negative (including CSF 16S sequencing, see below for everything I could find if you are interested) and MRI of brain and C/T/L spine was normal twice except for fluid in the middle ear - I think ENT got a fluid sample and they are culturing.

He has a normal immune work up so far:
Ig (IgG 275, IgA 12.9, IgM 37)
Flow normal: CD3 3970, CD4 2836, CD8 1008, CD19 1954, CD56/16 378, HLA-DR+ T cells 0
Mitogen proliferation
NOB

Any thoughts?  Cryopyrin sequencing is pending (but no rashes or arthritis).  Without an identified organism I am hesitant to think immune deficiency, perhaps an autoinflammatory disorder, but he doesn't have any other symptoms.

Thanks,

Megan

Megan Cooper, MD, PhD
Instructor, Pediatric Rheumatology and Immunology
Washington University
St. Louis Children's Hospital


ID evaluation negative:
CSF routine, fungal, AFB, LCMV, blastomyces, cryptococcal, parechovirus, enterovirus, HSV; 16S sequencing
Other: PPD; Toxoplasma; RPR; HIV; PCR for VZV, M pneumonia, HSV, enterovirus, EBV, CMV, NP swab, urine

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