A 61-year-old female with past medical history of multiple myeloma diagnosed July 2017 (stem cell transplant through Mayo Clinic Dec 2017 and chemotherapy instituted April 2018), type 2 non-insulin dependent DM (diagnosed Dec 2017 with last A1C of 11.2%- March 2019), unprovoked DVT in 2018 (Eliquis), and hypertension, who presented to the ER March 2019 with 2 day history of progressive weakness and decreased appetite. On day of admission, she was unwilling to get out of bed due to weakness, and her significant other noted right facial droop and right upper lid lag. In the emergency department, the patient reported blurred vision and headache for 3 weeks. She also reported no recent fever, vomiting, head injury, syncope, upper respiratory infection, abdominal pain, or urinary symptoms. With questioning, there had been no recent travel. The patient had suffered intermittent diarrhea that started after she was prescribed metformin. The patient was seen by her primary oncologist the day before, and serum testing was found to be baseline. She was compliant with her medications including metformin, Revlimid (lenalidomide), acyclovir, and Eliquis.
There was decreased attenuation throughout the white matter of the right cerebral hemisphere. This was asymmetric compared to the left. There was mild mass effect on the right lateral ventricle. Findings were worrisome for diffuse vasogenic edema. Differential would include sequela of prior therapy, underlying mass, less likely, given the asymmetry, and demyelinating process
Focal area with decreased attenuation in the right basal ganglia. Findings may represent a small lacunar infarction.
Mild diffuse cerebral volume loss.
CT scan head per radiologist:
MRI interpretation by radiologist (Figure 1): Extensive patchy right frontoparietal and central midbrain perivascular (likely perivenular) enhancement. There is associated asymmetric white matter edema and midbrain edema. Differential considerations include vasculitis, intravascular lymphoma, amyloid angiopathy, granulomatous angiitis, and less likely demyelinating process. A small irregular area of restricted effusion in the right parietal lobe is nonspecific. Intravascular thrombus and venous infarction cannot be excluded.
The patient remained stable in the ER and was admitted to medical bed with close neurologic monitoring. Revlimid, metformin, and Eliquis were held. We continued prophylactic acyclovir.
Neurosurgery as well as Oncology was consulted with continued daily oversight by Neurology.
Day 1: patient stable with similar exam findings.
  CSF analysis including flow cytometry: no malignant cells and cytology, and no monoclonal process.
  CSF fluid analysis: 83 lymphs, 11 monocytes, and 6 neutrophils.
  CSF cell count (2 bottles): RBCs 161/146 muL; nucleated cells 32/20 muL; neutrophils 2/6%, lymphs 80/83%, and monocytes 17/11%.
  Glucose 62 mg/dL and protein 69 mg/dL. Blood glucose was 95 at the time of the LP.
  CSF: complement factor elevated at 233, complement C3 elevated at 200, and complement C4 elevated at 48.
  CSF immunoglobulin testing unremarkable.
  CSF DNA testing: ACE, cryptococcus, E. coli, Epstein-Barr, herpes simplex, Listeria monocytogenes, Neisseria meningitidis, Streptococcus pneumococcus, GBS, CMV, Lyme, West Nile, neuron-specific enolase, VDRL, human herpes, varicella-zoster, human parechovirus, H. influenza: all negative.
Day 1: lumbar puncture results.
  JC virus and prion testing pending and not resulted until 1 week later but ultimately negative.
  Specific autoimmune and connective tissue disorder testing negative.
  Hepatitis C&B, HIV, and syphilis testing negative.
Initial CSF culture negative for growth after 6 days
  CT chest abdomen and pelvis on day 1: slight interval enlargement of left adrenal mass (present on previous imaging); otherwise no evidence of acute/new disease process.
  CTA head and neck on day 1: redemonstrated low attenuation diffuse right cerebral edema with possible small site of hemorrhage in the right frontal lobe. No vascular abnormality noted.
Blood cultures obtained on day 1 to cover for rare bacterial pathogens were negative after 6 days
Day 2: Neurologic symptoms worsened with facial droop and right eye ptosis, and occlusion of vision. The patient reported increased weakness with limited use of the right arm for ADLs. The patient denied worsening headache, fever, or vomiting. Serum testing on this day revealed negative leukocytosis, however, ESR 37 and CRP 5.11.
Informal consultation completed with radiologist regarding a revisit on the differential diagnosis.
CSF cultures were still negative and given her negative CSF PCR testing for pathogens, and dexamethasone was initiated for cerebral edema and presumed cerebral vasculitis.
On the evening of day 2, the patient reported improvement of clinical symptoms with less ptosis and more strength.
Day 3: the patient with continual clinical improvement and requested discharge home in lieu of brain biopsy; however, this request was denied.
CT imaging unchanged and the patient afebrile.
The patient was scheduled for brain biopsy on Day 4 but was cancelled and rescheduled for Day 7 due to pending prion testing and lack of neurosurgical equipment backup while awaiting prion results. A further investigation regarding etiology of cerebritis was not further evaluated, given clinical improvement and stable vitals.
Day 6 in the AM: the patient's condition deteriorates with vomiting and escalating hypertension and tachycardia. Facial drooping on the right returned. The patient was afebrile in the AM on morning rounds with WBC 3.8, normal electrolytes, and unchanged renal function with glucose 230 and calcium 8.5. The patient was immediately transferred to ICU, and repeat imaging was ordered: CT head revealed no significant interval change in the cerebral white matter hypodensity with a small dense focus within the right frontal lobe similar to prior CT exam.
MRI head on day 6: Little change in abnormal brain MRI with diffuse parenchymal and perivascular enhancement in the right frontal lobe, right occipital lobe, floor of the 4th ventricle, and interpeduncular cistern. Minimal decrease in vasogenic edema. Small amounts of petechial blood products. Continue mild effacement of the right lateral ventricle and minimal right to left shift of midline. Differential: multifocal leukoencephalopathy, lymphoma, encephalitis, vasculitis, or myelomaotsis
The patient was transferred to a Level 1 center early afternoon on day 6, but developed a low grade fever before departure. Before transfer, she was fatigued but responsive. On arrival to the Level 1 center (2 hr away), she was unresponsive with 103F fever. Blood cultures obtained upon arrival and the patient started on prophylactic cefipime, vancomycin, azithromycin, acyclovir, and voriconazole.
  Respiratory PCR panel including influenza negative
  Toxoplasma antibodies negative
  Coccidioides testing negative
  HIV, TB, and mycoplasma testing negative
  Procalcitonin: 0.04
  CT head: continued vasogenic edema right frontalparietal distribution.
  CTA head and neck perfusion: unremarkable
Day 6 at Level 1 center:
Day 7: another lumbar puncture completed; however, brain biopsy was held in lieu of ID workup.
CSF results from day 7: 172 nucleated cell, 45% neutrophils, 17% lymphocytes, and 38% macrophages.
Glucose 49 and protein 124; lactate 7.7 and LD 54. Blastomyces, toxoplasma, and varicella negative. Histoplasma and CMV negative.
CSF culture negative for growth at 5 days at the Level 1 center.
Day 7: blood culture positive for Gram-positive rods. Added amphotericin to treatment.
Day 8: ampicillin added to antimicrobial treatment.
Day 11: blood cultures positive for Listeria.
Day 13: gentamicin added to treatment.
Day 15: MRI imaging showed advancement of disease and comfort care instituted.
The patient expired on day 17.
Brain autopsy: revealed abscesses of the white matter in the frontal, temporal, and occipital lobes as well as the midbrain, pons, and cerebellum with multiple macroscopic leukocytes with perivascular inflammation consisting of lymphocytes and plasma cells consistent with listeriosis.
Diagnosis: Listeria cerebritis with bacteremia.
Neurosurgeon agreeable to brain biopsy if CSF analysis inconclusive since the concern was that her imaging finding suggestive of vasculitis or malignancy.