A 56-year-old woman with a history of atrial fibrillation, hypertension, congestive heart failure, and mechanical mitral valve repair following rheumatic heart disease on warfarin was transferred to our hospital after being found unresponsive. On arrival, her blood pressure was 141/66. She was awake and able to move all four extremities to command; however, she had no verbal output, and her Glasgow Coma Scale (GCS) was 10 (E = eye opening 3, V = verbal response 1, and M = motor response 6).
A head CT (Figure 1A) demonstrated a small ICH in the left periventricular white matter with significant intraventricular blood. CT angiogram of the head (Figure 1B) was suggestive of Moyamoya-like vasculopathy involving the left internal carotid artery (ICA) summit. Laboratory testing showed an INR of 3.6. She received intravenous vitamin K and prothrombin complex concentrate for INR reversal.
Following intubation for airway protection due to declining mental status, an EVD was placed; however, drainage ceased within 1 h of placement due to obstruction of the catheter with blood. On repeat clinical exam off sedation, the patient was not following commands with a GCS of 4T. She had fixed, non-reactive pupils at 3 mm; absent corneal, oculocephalic, cough, and gag reflexes; no spontaneous breaths; and slight flexion to painful stimuli in the upper extremities. Repeat head CT showed worsened IVH (Figure 1C) with hydrocephalus and apparent compression of the midbrain (Figure 1D).
The patient's husband was immediately informed that these developments were concerning for a devastating functional outcome, if she were to survive. Consequently, no further attempt was made to adjust the EVD for improved drainage or place a second EVD. Her code status was changed to "do not resuscitate" in the evening of admission. Her husband requested time to consider full comfort measures as goals of care, while continued routine ICU care would be pursued. Given the non-functioning EVD, obtained ICP readings were deemed inaccurate and thus not further acted upon through EVD manipulation or osmotherapy.
However, the patient's clinical exam spontaneously improved over the following 48 h, while supportive ICU care was continued. She began overbreathing the ventilator and regained reactive pupils, cough, and gag reflexes, with absent, but eventual return of corneal and oculocephalic reflexes. On motor exam, she extended in the right arm and leg, but moved the left arm spontaneously and withdrew in both left arm and leg.
Shortly after these observations, her EVD then started to drain spontaneously, without intervention, again approximately 48 h after initial placement. Based on this clinical improvement, the patient's husband requested that her code status be changed back to "full resuscitation." Repeat head CT on postbleed day 4 (Figure 1E) showed improved hydrocephalus, although still with ventricles "casted" from IVH. A more aggressive care approach was pursued at that time, and a left-sided EVD was placed.
A diagnostic cerebral angiogram confirmed a Moyamoya syndrome characterized by (1) chronic occlusion of the left supraclinoid ICA above the origin of the anterior choroidal artery, (2) a meshwork of small vessels replacing the ICA summit, proximal ACA, and proximal MCA, and (3) robust collaterals supplying the L MCA vascular territory via a widely patent anterior communicating artery and leptomeningeal collaterals derived from the distal L PCA (Figures 1F,G). No definite, angiographically demonstrable culprit lesion was demonstrated. However, in light of these findings, empiric administration of intraventricular tissue plasminogen activator was deferred, given an unknown further risk of intracranial hemorrhage in the setting of the patient's unique cerebrovascular anatomy. The patient was extubated safely 1 week after admission.
At the time of discharge to inpatient rehabilitation, 3 weeks posthemorrhage, the patient was able to follow commands intermittently and could move the right arm in the plane, the right leg with flicker movement, and the left arm and leg antigravity.
Figure 1H shows a surveillance head CT at 3 weeks without residual intraventricular blood.
At the time of outpatient follow-up at 12 weeks, the patient was alert, communicated well, was able to eat by herself, and required two-person assist for walking. Neurologic examination showed her to be partly oriented, with full ability to follow commands and no evidence of aphasia. On cranial nerve testing, she could count fingers in central visual fields only. Gaze was dysconjugate with a right third nerve palsy and bilateral restricted upgaze. The remaining cranial nerves were intact. Motor exam showed near full strength in the upper extremities and strength of 4 of 5 in the lower extremities. Sensory and cerebellar functions were intact. Her mobility had further improved, and at 9 months, she is able to ambulate with a rolling walker and requires one-person assist at times.