[PAGID] X-linked CGD with meningitis

[Kate Sullivan]

I doubt non-infectious granulomas in the brain or meninges.  Maybe I  
am biased because i have been surprised a few times but i would wager  
viral (he can get common things too) or fungal.  I have also had two  
mycobacterial brain infections in CGD patients although it is not  
supposed to be that common.

Kate
On Dec 1, 2009, at 1:11 PM, Christine Seroogy wrote:


> Dear Colleagues,

>

> I would appreciate your input on a 6 y/o boy with X-linked CGD who  

> presented with  headache (no meningismus signs on presentation),  

> intermittent fever and generally not feeling well for 3 days.

>

> He has been managed with IFN-g 3x/wk, itraconazole and bactrim  

> prophylaxis.

>

> He was diagnosed at age 2 when he presented with aspergillus pna— 

> this was successfully treated with one year of antifungals.  He has  

> had problems with granulomatous disease in his GI/GU tract over the  

> past 2 years.

>

> He lives on a farm in WI, however the family tries to remain  

> vigilant about exposures.

>

> He was admitted for further eval; during second hospital day he  

> developed severe headache and meningismus signs:

>

> Head CT normal

> Chest/abdomen CT: RUL pneumonia

> CSF leukocytosis: 2238 WBCs/ul; 25 RBCs/ul 93% PMNs Protein 54  

> (15-45) glucose normal.

> ESR 42 CRP 6

> Peripheral blood WBC with mild leukocytosis

>

> Blood culture (prior to antibiotics) grew S. viridans in 20 hours  

> (felt to be contaminate)

> CSF culture negative, however tap occurred 24 hours after initiation  

> of antibiotics.

>

> He has been on chronic low-dose steroids because of his  

> granulomatous disease and was given stress-dosing on admission which  

> was temporally related to relief of headache.

>

> He also had improvement of signs and symptoms after LP.

>

> He has had worsening of his headache in the last 24 hours despite  

> broad-spectrum antibiotics (and decrease in steroid dose to baseline— 

> 5 mg QOD).  He is scheduled for head MRI with contrast today.

>

> I am wondering if anyone has seen  a similar presentation?  We are  

> concerned about granulomatous disease in the CNS and currently do  

> not have an identified pathogen.  The next step will be lung tissue  

> since the processes are likely unified.

>

> Thank you for any comments or input.

>

> Regards,

>

> Chris

>

>

> -- 

> Christine M. Seroogy MD, FAAP, FAAAAI

> Assistant Professor

> University of Wisconsin

> Department of Pediatrics

> Division of Allergy, Immunology & Rheumatology

> 1111 Highland Avenue

> 4139 WIMR

> Madison, WI  53705-2275

> phone: 608-263-2652

> fax: 608-265-9721

>

>


Kathleen Sullivan MD PhD
Professor of Pediatrics
Chief, Division of Allergy Immunology
The Children's Hospital of Philadelphia
(p) 215-590-1697
(f) 267-426-0363




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