Objective:

NA

Background:

Craniocerebral aspergillosis linked to targeted chemoradiation in patients with hematological malignancies is a rare and devastating complication. Atypical presentations like isolated headache and cerebrovascular events are underreported. Here, we highlight an atypical case of a patient treated with multiple immune mediated therapies and targeted radiation for Chronic Lymphocytic Leukemia (CLL) who developed new onset side-locked headache in the setting of an expanding skull-based lesion followed by several neurovascular complications. 

Design/Methods:

NA

Results:

A 64-year-old male with CLL, on ibrutinib, Obinutuzumab, and an experimental Cereblon modulator, presented with new onset persistent right-sided headache and facial pain. Initial imaging revealed an enhancing skull-based lesion in the right middle cranial fossa, thought to be CLL progression, and he was treated with targeted radiation. The patient's headache continued to worsen over next 3 months and he developed multiple neurological deficits including right-sided hearing loss, right-sided dysmetria, and truncal ataxia. Subsequent imaging revealed an acute right cerebellar stroke, right transverse/sigmoid sinus thrombosis, and mild narrowing of the right cavernous ICA due to lesion expansion. Repeat biopsy of the skull-based lesion now demonstrated Aspergillosis. Patient was started on Voriconazole and anticoagulation. Despite antifungal therapy, the patient's neurological status deteriorated with recurrent ischemic strokes and new-onset seizures. Ultimately, the patient transitioned to home hospice and expired 4 months after diagnosis.

Conclusions:

CNS aspergillosis can expand rapidly at the skull base causing severe cerebrovascular complications including ischemic stroke, venous thrombosis, and angioinvasion resulting in significant morbidity and mortality. This case highlights that it is crucial to maintain a high index of suspicion for CNS aspergillosis in patients on targeted immune therapies with new onset headache or new focal neurologic signs.