Cerebral Venous Sinus Thrombosis in a Young Female Misdiagnosed as Migraine Ending in a Permanent Vegetative State

A Case Report and Review of the Literature

Sana Alshurafa; Wadiah Alfilfil; Ayah Alshurafa; Khadijah Alhashim

Disclosures

J Med Case Reports. 2018;12(323) 

In This Article

Case Presentation

A 35-year-old Arabic female, who was not known to have any medical illnesses, presented to the emergency department (ED) of a secondary hospital for the third time with the same complaint: a throbbing headache for the past 2 days that did not respond to analgesics (Table 1). The migraine-like headache was in the left frontal area, moderate in severity, and sometimes severe enough to interrupt her sleep. It was continuous with no relieving or exaggerating factors. Her headache was accompanied by two episodes of vomiting. She denied having a migraine in the past or any similar kind of headache. There was no history of fever, photophobia, or change in behavior or personality. No history of abnormal movement or loss of consciousness was reported. Furthermore, she had no history of weakness or loss of sensation, or gate or posture abnormalities. She denied diplopia, vision loss, or dysphagia. There was no previous history of venous thromboembolism (VTE) or any hematological diseases in her family. There was a positive family history of stroke at a young age. Other systematic reviews were unremarkable.

On physical examination, the patient walked to the observation area in the ED. She looked a bit tired, hypoactive, and in mild pain distress. She was hemodynamically stable, and her vital signs were temperature 37 °C, blood pressure 117/68 mm Hg, and pulse rate 71 beats per minute. Her respiratory rate was 18 per minute, and oxygen saturation was 99% in room air. General examinations revealed no neck rigidity, the cranial nerves were intact, and there was no proptosis. Her chest was clear, and her abdomen was soft and not tender. Neurologically, she was completely conscious, alert, and oriented to time, place, and person. Her Glasgow Coma Scale (GCS) was 15/15 with no focal neurological deficit, and the power in all four limbs was 5/5, with normal reflexes, and equally reactive pupils. The Brudziniski and Kerning signs were negative. The Babinski reflex went down. Fundus examination was not performed.

She had been seen twice before in the ED by two junior general practitioners who diagnosed her condition as migraine. They attributed her migraine to an anemic state and oral contraceptive pills, which were taken recently to regulate her menstrual cycle. They therefore discharged her after prescribing oral analgesics and diclofenac injection. During her third visit, an emergency consultant raised the suspicion of CVT based on the following red flags: recurrent visits to ED, no previous migraine in the past, headache interfered with sleep, and recent use of oral contraceptives. Plain computed tomography (CT) of the brain was performed initially and showed hyperdense signs in the left transverse sinus and straight sinus (Figure 1). Early in the morning, a brain venogram was conducted which confirmed left transverse sinus thrombosis, inferior sagittal sinus, straight sinus, and deep venous thrombosis in the confluence sinuses (Figs. 2, 3 and 4).

Figure 1.

A cross sectional view of a computed tomography brain scan without contrast shows hyperdensity in the thrombosed sinus

Figure 2.

The cord sign shown in the computed tomography scan without contrast

Figure 3.

The filling defect in the computed tomography scan with contrast

Figure 4.

A sagittal view of the computed tomography brain with contrast showing the filling defect of the superior sagittal sinus that reassembles a triangle shape, called empty triangle or empty delta sign

During the therapeutic interventions, the patient was referred to the neurologist on call who initiated a therapeutic dose of low-molecular weight heparin (LMWH; enoxaparin) 1 mg/kg subcutaneously. The patient was admitted to a regular ward in good condition. Twelve hours following the ED visit, in the evening, her level of consciousness deteriorated. She became agitated, confused, disoriented, and talked only a few words inappropriately. Her GCS dropped to 12/15 (eye opening 4, verbal response 4, motor response 4) and both her pupils were reactive equally to light measuring 4 mm.

She was evaluated by a critical care physician on call who intubated her electively and moved her to the intensive care unit (ICU) for monitoring. He advised urgent CT brain scan and to initiate phenytoin 900 mg intravenously. Two hours later, the plain CT showed a newly developed third ventricular hemorrhage and deep venous thrombosis in the vein of Galen and left transverse venous sinus thrombosis (Figure 5).

Figure 5.

Newly developed hyperdensity seen along the third ventricle suggestive of a hemorrhagic component. The old thrombosis in the deep veins and left transverse sinuses are still present

Her neurologist saw her 12 hours later in the morning round who attributed the deterioration in her condition to the increase in intracranial pressure. The neurologist advised to discontinue phenytoin and continue enoxaparin in the same therapeutic dose (60 mg subcutaneously). Acetazolamide (500 mg intravenously) followed by 250 mg twice daily was started. Moreover, a neurosurgeon, who was consulted, evaluated her 10 hours later and agreed to proceed with an extraventricular drain (EVD) to relieve the early hydrocephalus. However, despite EVD insertion, a CT scan of her brain showed an increase in brain edema with an acute increase in the intraventricular pressure and transtentorial herniation resulting in hydrocephalus and left thalamic ischemia (Figs. 6 and 7). A multidisciplinary team from neurology, neurosurgery, and ICU decided to perform bi-frontal craniotomy as brain decompression surgery (Figure 8). One day following the craniotomy, she developed left pupil dilatation, and an urgent CT brain scan showed a large extracranial (epidural) hemorrhage in the left frontoparietal area, with generalized brain edema and periventricular ischemia (Figs. 9 and 10). Her condition required urgent evacuation, and enoxaparin was switched to low therapeutic range heparin at a dose of 40 mg subcutaneously daily. A few days later, a follow-up brain CT scan revealed newly developed ischemic areas in the left parietal and occipital lobes and a significant reduction in the hemorrhage. Furthermore, during her ICU stay, she developed surgically induced meningitis and multiple chest infections. The patient ended up in the ICU on tracheostomy with mechanical ventilation, in a permanent vegetative state and in need of long-term care.

Figure 6.

Right-sided external ventricular drain with the tip in the right ventricle. An increase in brain edema, acute increase in interventricular pressure, and downward transtentorial herniation causing hydrocephalous, with no evidence of thrombosis in the sinuses and veins

Figure 7.

Right-sided external ventricular drain with the tip in the right ventricle. An increase in brain edema, acute increase in interventricular pressure, and downward transtentorial herniation causing hydrocephalous, with no evidence of thrombosis in the sinuses and veins

Figure 8.

Post-decompression craniotomy of the bilateral frontoparital area

Figure 9.

Large extra-axial hemorrhage with mid-line shift

Figure 10.

Large extra-axial hemorrhage with mid-line shift

processing....