Cerebral Venous Sinus Thrombosis With Severe Thrombocytopenia

Case

A 35-year-old woman with a medical history of migraine headaches and chronic thrombocytopenia presented to the emergency department (ED) with severe headache 7 days after vaccination with the J&J COVID-19 vaccine. The patient was not pregnant, had no history of hypercoagulability, and no use of hormone replacement therapy; however, her body mass index was 36.6. She described her headache as being more severe and retro-orbital compared with her typical migraine. Physical examination was remarkable only for mild neck stiffness. Laboratory evaluation revealed a platelet count of 114K/ul (106 K/ul in 2015, although January 2021 was up to 164 K/uL; Table 1). Noncontrast head CT was normal (Figure 1A), and a CT venogram head did not reveal any evidence of cerebral venous thrombosis (Figure 1B). She was discharged to home with improvement in symptoms after migraine treatment. She returned to a separate ED 20 hours later with persistent headache. No repeat imaging or laboratory studies were completed. The patient continued to have a normal neurologic examination. She was again discharged after symptomatic relief.

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Figure 1 Noncontrast CT Head and CT Venogram Sagittal View, a Comparison Between First and Third ED Visit

(A) Noncontrast head CT performed on 1st ED visit; no abnormal density noted within the sinuses. (B) CT venogram postcontrast performed on the initial ED visit, note appropriate opacification of sinuses. (C) Noncontrast head CT was performed on the third ED visit; the arrow points toward abnormal density within superior sagittal and transverse sinus. (D) CT venogram postcontrast performed on the third ED visit; a lack of contrast opacification of sinuses and persistent density as demonstrated on noncontrast CT head consistent with cerebral venous sinus thrombosis.

Fourty-eight hours after initial ED visit and 16 hours after discharge from the second ED visit, the patient experienced an acute loss of consciousness with an onset of generalized tonic-clonic seizure activity. On arrival to the ED, she was unresponsive with no purposeful movements. Neurologic examination revealed a Glasgow coma score of 3 with corneal, gag, and cough reflexes absent. Pupils were 6 mm and fixed. Noncontrast head CT revealed right temporal intraparenchymal hemorrhage and extensive subarachnoid hemorrhage (Figure 2A). There was also evidence of elevated intracranial pressure (sulci effacement) and resultant transtentorial herniation with obliteration of the basilar cisterns. A hyperdensity involving the superior sagittal sinus, right transverse sinus, and right sigmoid sinus suggested cerebral venous thrombosis (Figures 1C and 2B). Subsequent CT angiogram and venogram of the head revealed findings consistent with superior sagittal, transverse, and sigmoid sinus thrombosis (Figure 1D).

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Figure 2 Coronal and Axial View of Noncontrast Head CT Performed on the Third Visit

(A) Axial view of noncontrast head CT with findings consistent with intraparenchymal and subarachnoid hemorrhage. (B) Axial view of noncontrast head CT showing prominent density in the right sigmoid sinus (indicated by arrow sign) compared with the left sigmoid sinus, indicating right sigmoid sinus thrombosis. (C) shows coronal view of noncontrast head CT; the arrow indicates delta sign suggestive of dural sinus thrombosis.

Laboratory evaluation revealed a platelet count of 10 K/uL, fibrinogen of 128 mg/dL, and D-dimer of 13476 ng/dL. Heparin-induced thrombocytopenia antibody assay was very elevated at 2.240 OD (normal <0.4). The patient had no exposure to heparin products. Evaluation by neurology and neurosurgery did not recommend any neurosurgical intervention secondary to futility. The patient had an ongoing absence of cortical activity with the absence of all cortical and brainstem reflexes. Nuclear medicine perfusion imaging documented the absence of cerebral perfusion.