[PAGID] X-linked CGD with meningitis

[Christine Seroogy]

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



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