A 37-year-old female patient who received epidural anesthesia during childbirth presented to the neurosurgical service eleven months postpartum for worsening headaches with nausea, vomiting, vision changes, and weight loss. Here, a medical history was significant for peripheral neuropathies, unspecified mood disorder, herpes labialis, hepatitis C (HCV), intravenous substance abuse (including heroin and cocaine, now on buprenorphine), and noncompliance. Her medications before admission included gabapentin 300 mg daily, buprenorphine 2 mg daily, and topiramate 25 mg daily. She was never treated for hepatitis.
Delivery of her child the following month was uncomplicated, but she did require a second epidural injection during labor. After birth of her child, she had persistent headaches that were suspected to be secondary to a cerebrospinal fluid leak. She underwent epidural blood patch to treat the suspected leak, but her symptoms did not resolve. She began having shooting pain in her back that radiated into her legs. She described the headache as generalized, dull, nonradiating, and constant with a gradual onset that "worsen with laying down or standing up" and endorsed accompanying photophobia.
She had papilledema with diplopia, mild bilateral cranial nerve VI palsy, and decreased visual acuity. At this point, she was seen by ophthalmology and was admitted to the hospital. A lumbar puncture (LP) showed mildly elevated nucleated cell count of 179 with 50% PMNs, 42% lymphocytes, and 6% monocytes. Glucose was low at 36 mg/dL (serum glucose was 92 mg/dL) and protein was elevated at 287 mg/dL, consistent with a meningitis process and suggestive of bacterial meningitis. However, her CSF cultures were negative for histoplasma, tuberculosis, AFB, Cryptococcus, cytospin, fungus, and viruses. Additional testing showed HIV negativity, hepatitis A and C positivity and negative serology for listeria, Brucella, VDRL, syphilis, and lyme.
Her general chemistry panel on admission and throughout her hospital stay was normal. Liver function tests, CRP, IgG, and thrombocyte count were unremarkable. She was negative for antinuclear antibody, anti-LA/anti-RO, and antineutrophil cytoplasmic antibodies. Her CRP was normal with a mildly elevated ESR at the time of presentation in April, consistent with a largely resolved inflammatory process.
MRI studies showed diffuse leptomeningeal enhancement of the distal spinal cord, conus medullaris, and nerve roots of the cauda equina extending beyond the neural foramina bilaterally in T1 contrasted images [Figures 1 and 2]. The nerve roots were displaced laterally in the distal thecal sac. The dura appeared thickened and avidly enhanced from L5 to S2 and there was a fluid-fluid level appreciated on the T2-weighted sequences suggesting possibly proteinaceous debris, blood, or pus [Figure 3]. The DWI image showed marked restriction from L4 to L5 through the S2 levels [Figure 4].
Infectious disease was consulted and suggested chronic neutrophilic meningitis in the setting of an underlying autoimmune/inflammatory condition given the nature of her pain and her complicated medical history. She was discharged after 1 week on methylprednisolone (1 g/day for 5 days) that resulted in a near resolution of her back pain, headaches, and vision changes except for when she missed doses. She had no fevers, chills, or night sweats but reported a 10 lb weight loss with poor appetite. Her steroids were tapered to 50 mg, daily and when the dose was tapered, she had symptom return.
Infectious disease continued following and a lack of resolution of her pain prompted additional screening. Repeat MRI at a month post-partum showed no significant changes from the previous scan and continued leptomeningeal enhancement of the distal spinal cord and cauda equina nerve roots consistent with arachnoiditis. Her second LP showed a worsening inflammatory picture with 2682 nucleated cells (94% PMNs), low glucose at 26, elevated protein at 297, and 13 bands. The patient's WBC was elevated at 23.2 from 16.4 three months prior although she remained afebrile. Her back pain rating at this time was 6-7/10.
Given her persistent symptoms and lack of diagnosis, she was scheduled for an L5-S1 laminectomy for an intradural tissue biopsy. During surgery, cultures were taken at every level of the exposure. After the laminectomy was complete, the epidural space appeared normal, but the dura was thickened and difficult to open inferiorly. The dura incision was carried cranially and once the subarachnoid space was entered, a copious amount of white, soft purulence material was released. The nerve roots were adherent to surrounding dura and to each other. There were no obvious signs of a capsule; however, the infection was likely loculated based on its appearance and indolent course. The intradural compartment was irrigated until the CSF ran clear. There were no intraoperative complications and she was started on postoperative antibiotics, including 500 mg metronidazole q8 h, 1 g vancomycin q12 h, IV fluconazole 400 mg, daily, and 2 g cefepime q12 h. After the procedure, the patient remained afebrile, without aberration in vitals, and was ambulatory.
The histology showed rare nonseptate pseudohyphae and blastospores without a broad-based bud, indicative of a Candida species [Figure 5]. The submitted tissue was necrotic and demonstrated local inflammation on pathologic examination [Figure 6]. Microbiology cultures from the abscess grew colonies of C. dubliniensis. No other organisms were isolated or seen under various other staining modalities and multiple cultures taken from the abscess all grew only C. dubliniensis and no other organisms. This was a pan-sensitive microbe with low minimum inhibitory concentrations to caspofungin, fluconazole, and voriconazole of 0/25, 1.0, and 0.012 mcg/mL, respectively.
Cefepime, metronidazole, vancomycin, and IV fluconazole were discontinued and the patient was started on 400 mg fluconazole PO daily (rather than outpatient peripherally inserted central catheter due to IV drug abuse) for a planned minimum of 4 weeks. After surgery, she endorsed only mild low back pain at the incision site and with position changes.
At 3-week postoperative follow-up, her back pain was worsening and the operative site was infected with induration of the surrounding skin but remained afebrile. She reported a significant amount of purulent drainage produced from her incision, which resulted in improvement of her symptoms. She was started on cephalexin. She is currently scheduled for follow-up with infectious disease and for a repeat MRI of her lumbar spine.
Of note, throughout the patient's entire hospital course and at her follow-up appointments, she remained without neurological deficit. Her mental status examination, cranial nerves, strength, sensory perception, coordination, and reflexes were all within normal limits and without significant change at any time point.