Neurointensivist Saint Vincent's Medical Center Frank H. Netter School of Medicine Stamford, Connecticut, United States
Introduction: A 62-year-old obese Albanian woman with a history of Diabetes and Ozempic use, was admitted with 3 days of worsening headaches described as being "hit by a bus". She was alert and non-focal on admission. CT scans revealed L cerebellar venous infarction with hemorrhage and extensive Cerebral Sinus Venous Thrombosis(CSVT) in superior sagittal, straight, R transverse and sigmoid sinuses
Description: Due to heavy clot burden, she was started on iv Heparin despite newly noted thrombocytopenia of 45000/ml. She was intubated for obtundation the next day and underwent EVD placement, venous thrombectomy as well as suboccipital craniectomy. Her platelet count continued to drop therefore she was transitioned to Argatroban. Workup was positive for Platelet-activating factor 4 antibodies, but Serotonin Release Assay (SRA) was negative. She had a protracted ICU course with malignant cerebellar edema and refractory ICP crisis needing escalating sedatives and hypertonics. Hypercoagulable testing revealed heterozygosity of Prothrombin gene 20210A mutation. Her platelet count resolved to normal by day 7 of admission. By week 3, she had improvement in posterior fossa edema. Her EVD was removed, and she underwent tracheostomy and PEG placement. She had slow improvement in mentation and could communicate with her eyes and tongue movement, but had significant quadriparesis, which was thought to be from critical illness myopathy. By the time she was discharged to rehab, she was successfully decannulated and able to communicate and move her extremities.
Discussion: CSVT is a rare complication of HIT (Heparin-Induced Thrombocytopenia). It is infrequently reported with SRA (Serotonin Release Assay) negative HIT. This case posed a challenging clinical scenario with significant thrombosis and thrombocytopenia needing urgent anticoagulation. It is highly likely that her underlying heterozygous Prothrombin gene mutation combined with Ozempic use contributed to her severe CSVT. This case reminds us to consider SRA negative HIT in the rare cases of CSVT presenting with thrombocytopenia.
