Neonate with chronic meningitis and hydrocephalus (2003)

A 6-day-old Hispanic girl was admitted for fever and irritability and underwent evaluation including examination of blood, urine and cerebrospinal fluid (CSF) with cultures to rule out serious bacterial infection. Her birth history was unremarkable. She was born by normal spontaneous vaginal delivery after an uncomplicated pregnancy. There was no history of maternal infection, chorioamnionitis or use of any medication. Her birth weight was 3.5 kg. On physical examination she was crying, but consolable. Mucous membranes were slightly dry. The rest of the examination was normal, including the neurologic examination. The white blood cell count (WBC) was 17 800/mm3 with 55% neutrophils, 23% lymphocytes, 2% band forms, 15% monocytes, 1% metamyelocytes and 4% atypical lymphocytes. The hemoglobin was 12.5 g/dl and platelets 234 000/mm3. The spinal fluid showed CSF pleocytosis (Table 1). Cultures of blood, urine and CSF showed no growth. During the hospitalization episodes of supraventricular tachycardia were noted. She defervesced, antibiotics were discontinued after 3 days and she was discharged with the diagnosis of aseptic meningitis. She was sent home with an apnea monitor and instructions to follow up with the primary care physician and cardiologist.TABLE 1: Cerebrospinal fluid findingsFever recurred at home shortly after discharge, and she was readmitted at 3 weeks of age with increasing fever and irritability. She had no signs of upper respiratory tract infection or gastrointestinal symptoms and was feeding well. There were no sick contacts, nor were there pets in the household. A full evaluation was done once again, including blood, urine and CSF cultures. Physical examination at the time of the second admission was notable for weight of 3.8 kg. Her temperature was 38.8°C, pulse 179 beats/min, respiratory rate 27 breaths/min and blood pressure 94/52 mm Hg. She was sleeping but could be aroused and was consolable. The anterior fontanel was full/mildly bulging. Lungs were clear. Cardiac examination revealed a I/VI systolic ejection murmur. Neurologic examination showed normal suck, grasp, Moro reflex and patellar reflexes. There was no hepatosplenomegaly, lymphadenopathy or rash. Laboratory studies revealed a WBC of 17 300 cells/mm3 with 36% neutrophils, 28% band forms, 25% lymphocytes, 9% monocytes and 2% eosinophils. The hemoglobin was 9.5 g/dl, and platelets were 393 000/mm3. C-reactive protein was elevated at 25.8 mg/dl. CSF findings (Table 1) revealed an increasing pleocytosis, falling glucose concentrations and rising protein concentrations. No organisms were seen on the Gram-stained smear. A chest radiograph was unremarkable. She was treated with ampicillin and cefotaxime. She continued to have low grade fevers, mild fussiness and documented episodes of supraventricular tachycardia. A computed tomography scan of the head showed ventriculomegaly, subdural hygromas and a left parietooccipital hypodense area with mild contrast enhancement consistent with a focal area of cerebritis and/or vasculitis. Ampicillin was replaced by vancomycin empirically, and cefotaxime was continued. She was treated with propranolol for probable Wolff-Parkinson-White syndrome. Bacterial cultures of the blood and CSF remained negative, and herpes simplex virus PCR was negative from CSF. On Hospital Day 3 a repeat lumbar puncture revealed continued CSF abnormalities. Because of lack of improvement, cefotaxime was changed empirically to meropenem, and the dosage of vancomycin was increased from 60 mg every 6 h to 100 mg every 8 h. On Hospital Day 4, the temperature rose to 39°C. Purified protein derivative skin tests showed no induration in the patient and any household contacts. Magnetic resonance imaging of the head showed diffuse arachnoid enhancement, small convexity hygromas vs. empyemas, cerebral edema vs. cerebritis/encephalomalacia and increased ventriculomegaly. An ophthalmology consultant found no evidence of retinitis. The patient developed opisthotonic posturing. Neurosurgery was consulted. Bilateral subdural taps were performed yielding slightly hazy hygroma fluid. There was no frank purulence. Multiple bacterial, fungal, viral and acid-fast bacillus cultures of the CSF and hygroma fluid were negative. In addition to antibiotics, the patient was given Decadron and phenobarbital for seizure prophylaxis. On Hospital Day 7 empiric acyclovir was started. A rapid plasma reagin circle card test for syphilis was negative in serum and CSF. Repeat PCR for herpes simplex, as well as PCR for Mycoplasma pneumoniae, Mycobacterium tuberculosis and enteroviruses from the CSF were all negative. On Hospital Day 10 magnetic resonance imaging of the brain and spine showed increased hydrocephalus with intense meningeal enhancement along the entire spinal cord. On Hospital Day 11 CSF was again examined (Table 1). Fluconazole was started empirically pending Coccidioides immitis serology, which later proved negative. Acid-fast bacillus stains on gastric aspirates were negative. Serologies for Toxoplasma gondii, rubella, cytomegalovirus, herpes simplex viruses 1 and 2 and Cysticercus were sent from the serum. On Hospital Day 13 CSF cultures from Hospital Day 11 was reported as positive after 48 h of incubation.