We report the case of a 28-year-old woman who presented simultaneously with superior sagittal sinus
thrombosis and
thyroid crisis, and was subsequently found to have
protein C deficiency. February 3, 2003, she admitted complaining of
abdominal pain. The diagnosis of
appendicitis was made, and she was operated on under lumbar anaesthesia. Day 7, she developed acute
headache and distal weakness of the left lower limb. On examination she was alert, with a temperature of 38 degrees C, a
sinus tachycardia of 124/min and blood pressure 164/84 mmHg. Neurological examination revealed neck stiffness and left
hemiparesis, predominantly in her lower limb. Gadlinium-enhanced brain MRI revealed extensive superior sagittal sinus
thrombosis. CT scan demonstrated
infarction in the right frontal cortex, and
subarachnoid hemorrhage adjacent to the right cerebellar tentorium. The patient was treated with a
free radical scavenger edarabon, and
glycerin. No
anticoagulant therapy was instituted. Over the next 24 hours, her condition worsened. She became
comatose, as well as developing a
generalized tonic-clonic seizure. Day 12, laboratory examinations revealed an undetectable TSH-level CTSH (
thyroid stimulating hormone) <0.005 mcIU/ml), with a level of free
thyroxin 7.77 ng/dl (0.9-1.7), free triiodothyronin 29.6 pg/ml (2.3-4.3), and positive anti-
TSH receptor antibodies determined subsequently.
Coagulation factor VIII activity was 155% (normal range 60-150).
Protein C deficiency (
antigen 59%, activity 49%) was also present, suggesting a congenital type I heterozygous deficiency. A diagnosis of
thyroid crisis on the basis of
Graves' disease was made. The patient remained
comatose and died on Day 16, with
renal failure. The patient had
protein C deficiency, a well-established risk factor for cerebral
venous thrombosis (CVT). However, additional risk factors are required in most cases to precipitate CVT. In our case, this trigger was most likely
thyroid crisis, suggesting that
thyrotoxicosis, probably through
hypercoagulability, may be a predisposing factor for the development of CVT.