Not Your "Tick"-pical Headache
Abstract
Introduction: Subarachnoid hemorrhage is a medical emergency in which blood develops between the arachnoid and pia mater requiring prompt head imaging and potential neurosurgical intervention. It can be caused by trauma or aneurysm rupture and is associated with significant mortality and morbidity [1]. We present a case of a patient developing non-traumatic subarachnoid hemorrhage in the setting of a tick-borne illness.
Case Information: A 60-year-old female with past medical history significant for immunosuppression secondary to polycystic kidney disease status post living donor kidney transplant in 2020 and without history of blood disorders presented with flu-like symptoms and required admission for new pancytopenia. Lab findings included elevated ferritin and transaminases with concern for hemophagocytic lymphohistiocytosis (HLH) as well as positive parvovirus, ehrlichiosis, CMV, EBV, and BK. Initially, the patient was mentating appropriately with negative CT head imaging. Despite steroids, transfusions, IVIg, antibiotics, and antivirals, she had persistent fevers with progressive pancytopenia and subsequent encephalopathy requiring intubation. Follow-up CT head showed new bifrontal subarachnoid hemorrhages (SAH) and repeat imaging in the following days demonstrated progression of the SAH's and a new intraparenchymal hemorrhage. MRI and CTA head did not demonstrate any predisposing structural etiologies such as stenosis, occlusions or aneurysms. Lumbar puncture was delayed due to severe thrombocytopenia but ultimately showed minimal pleocytosis without infection. The patient initially had a period of clinical improvement with Ehrlichia resolution and some viremic suppression. However, she then decompensated again in the setting of new GNR bacteremia and aspergillosis with inability to wean immunosuppression and eventual transition to comfort care.
Discussion/Clinical Findings: This was a challenging case given difficulty managing the underlying hyper-inflammatory disorder with subsequent co-infection from multiple pathogens. Ehrlichia, a small gram negative bacteria initially specific to canines, was found to have human transmission as recently as 1986. Common non-specific symptoms include fever, fatigue, leukopenia, thrombocytopenia, transaminitis, and rarely encephalopathy, but it has also been linked to bone marrow hypoplasia causing pancytopenia and HLH [2]. Lumbar puncture typically demonstrates lymphocytosis with elevated protein but this was not the case in our patient potentially due to doxycycline initiation prior to sampling. Cytomegalovirus (CMV) PCR was also positive later in the course but the hemorrhages were stable at that point. CMV is associated with intraventricular hemorrhages mainly in premature infants with thrombocytopenia, but was unlikely related to this patient's bleeds given the timeline [3]. We hypothesize that the Ehrlichia induced thrombocytopenia and adjunctive HLH predisposed our patient to the SAH. The patient showed clinical improvement after restarting doxycycline but continued to require persistent immunosuppression in an attempt to mitigate the persistent maladaptive immune response. This led to an augmentation of her already profound immunosuppression with unremitting pancytopenia predisposing her to the emergence of multiple other infectious processes including CMV, GNR bacteremia and aspergillosis.
Conclusion: We present this case to highlight a rare presentation of Ehrlichia induced subarachnoid and intraparenchymal hemorrhages in the setting of pancytopenia.