Clinical Case Presentation #3 - Maria G. Lopez-Marti, MD - Duke ...

A 2 month old baby with newonsetseizures…Maria Guadalupe Lopez-MartiFellows’ case presentation

Presentation• 5wk old healthy male• New onset of right-sided focal seizures• Transferred to Duke PICU for anintracranial mass on head CT at an outsidehospital

HPI• FT baby, no complications• 10 days prior had fever for 4 days and wasdiagnosed with AOM by PCP• On amoxicillin day 10/10• Afebrile for 1 day, but then new fever of102.3F→reconsult PCP• No URI symptoms now• No sick contacts

PCP office• Febrile=103F• Focal seizure of RUE , with rhythmicfisting movements which self resolved• Sent to ER, where he had a 2 nd seizure• Head CT showed a 2.1x1.7cm hypodensemass in L frontal lobe producing shift to Rfrontal lobe with mass effect downward

CT: rim-enhancing low-density fluid collectionmeasuring 29 x 19 mm in the left frontal lobeMRI: rim-enhancing 3.0 cm left frontal lobemass

Differential diagnosis?

Hospital course• Subdural empyema and brain abscess• Pediatric NSU→ OR for drainage• OR Culture grew MRSA• Treated with vancomycin and rifampin• Discharged home on IV antibiotics tocomplete 2 months

Brainabscess

Brain abscess in infants• 1 st 3 months of life: Gram-negatives arekey players• Usually secondary to meningitis• Citrobacter koseri , Serratia marcescens,Proteus mirabilis and Enterobacter• MRSA not your usual pathogen here…

PID clinic follow-up 1 monthlater• Clinically doing very well…• Would you do further testing at this point?

Immunology work-up• CBC w/diff• Ig profile• Cellular competence• Lymphocyte enumeration• Respiratory burst• CH50= less than 10

Complement deficiency• Frequency of inherited complement deficienciesin the general population= 0.03%• Association with SLE, atypical HUS, Neisseriameningitidis, S. pneumoniae• Rare form: CR3 deficiency (Membrane ProteinRegulating C′ Activation) assoc with S. aureus• Evaluation by Peds AI for defect characterizationin progress

Take home points• High index of suspicion needed to detectprimary immunodeficiencies• “Common things are common, but…”• Unusually aggressive presentations/rarelocations/rare organisms/recurrentinfections should prompt further work-up