A 48-year-old African American woman with a past medical history of anxiety presented to the emergency room with an acute change in mental status. The patient's husband reported that she had been in her usual state of health with the exception of a headache the day before presentation. On the morning of admission, the patient went grocery shopping and on her return appeared confused and disoriented. The patient's husband also reported that her gait was unsteady and that she had an episode of urinary incontinence.
The patient was not taking any medications or supplements and consumed 6 beers daily. Family history included breast cancer in her mother and prostate cancer in her father. On physical examination, the patient was afebrile and hemodynamically stable with a Glasgow Coma Scale score of 11. During neurologic examination, the patient was combative, spoke inappropriately, and was indifferent to her surroundings. However, she had equally round and reactive pupils and full range of motion in all 4 extremities.
Laboratory findings were normal. Results of a urinary drug screen and noncontrast computed tomography of the brain performed on the day of admission were unremarkable. A lumbar puncture was performed; cerebral spinal fluid testing was negative for white blood cells and revealed a protein level of 75 mg/dL and glucose level of 86 mg/dL. The patient was given empiric intravenous thiamine hydrochloride and acyclovir sodium for Wernicke encephalopathy and herpes encephalitis. On hospital day 2, magnetic resonance imaging of the brain revealed new hydrocephalus, and an electroencephalogram showed changes consistent with diffuse encephalopathy of nonspecific origin. Results of contrast-enhanced computed tomography of the chest, abdomen, and pelvis were unremarkable. On hospital day 4, cerebrospinal fluid testing was negative for bacterial, fungal, and acid fast cultures and cryptococcal antigens, and the patient was given methylprednisolone (125 mg intravenously). On day 5, cerebrospinal fluid testing for herpes simplex virus returned negative results and acyclovir was discontinued. On day 6, the patient was showing little improvement, so magnetic resonance angiography/venography was performed. This imaging revealed thromboses of the paired deep internal cerebral veins, the Galen vein, and the straight sinus (
Figure).
After the diagnosis of a cerebral vein thrombosis, warfarin therapy (7.5 mg daily) was initiated. Before diagnosis, a comprehensive panel was drawn for coagulopathies and vasculitides. These findings were negative for anticardiolipin antibody, lupus anticoagulant, factor V Leiden, factor II gene mutation, β2 glycoprotein 1 antibody, antinuclear antibody, cryoglobulinemia, antineutrophil cytoplasmic antibody–associated vasculitis, and human immunodeficiency virus. The patient's protein C and protein S activity, protein electrophoresis, homocysteine levels, and thyroid stimulating hormone levels were normal. The only remarkable laboratory value was an elevated factor VIII assay at a level of 399% (reference range, 75% to 160%).
The patient remained in the hospital for 34 days. Her mental status improved substantially, and, at the time of discharge, she was oriented with an appropriate affect and able to answer questions properly. She was instructed to remain on warfarin (7.5 mg daily) indefinitely. The patient was followed up 2 weeks after discharge, at which time her husband reported that her mental status had returned to baseline (ie, mental status prior to the encephalopathic event).