We report a case of 2-year-old male patient, resident of Punjab, Pakistan, presenting with high grade fever along with seizures and left sided weakness of face, arm, and leg. An MRI was recommended along with a CSF culture; the MRI did not show any signs of stroke; it was deduced to be an episode of a transient ischemic attack (TIA) or an infection. The CSF culture also was negative; the patient was treated for meningoencephalitis and recovered in 10 days. Six months after this initial presentation, he suffered another stroke-like episode, this time affecting the middle cerebral artery/right temporal artery regions with hemiparesis in the right upper and lower extremities.
An MRI was recommended, which was positive for the ischemic changes in the regions supplied by the abovementioned arterial regions. Blood biochemistries for Protein C and S deficiencies were negative.
This continued when he suffered 3 more stroke-like episodes within the following three months, which affected his speech and vision along with right sided facial and limb weakness. He underwent all his lab investigations such as Hepatitis B titers, VZV titers, and poliovirus which were negative for viral illnesses. Lupus anticoagulant was negative, refuting presence of lupus disorders, and Hb electrophoresis was done showing normal electrophoresis negating presence of sickle cell disease. His coagulation profile was with normal PT/APTT and INR values; no abnormality was seen on echocardiogram. It is pertinent to mention that the patients' mother had suffered tuberculosis during her pregnancy with the patient; the TB was negative however in the patient upon testing. He was screened for Protein C and S deficiencies, which were negative. The patient was finally diagnosed as the case of bilateral moyamoya disease based on CT angiogram, which showed severe bilateral stenosis and occlusion of supraclinoid segments of bilateral internal carotid artery (ICA), middle cerebral artery (MCA), and proximal A1 and bilateral Anterior Cerebral Arteries (ACAs), with an extensive collateral formation and enhanced vascularity in both cerebral hemispheres (please refer to Figure 1). Patient was recommended for surgery and was started on Carbamazepine and aspirin.
He was listed for bilateral EDAS, first on the right side of the brain to preserve the better functioning hemisphere and four months later for the left side of the brain. He has shown considerable improvement with no new stroke reported after the EDAS. CT angiography was repeated after 6 months from his second surgery and showed improved patency of collateral vessels and better anastomoses (please refer to Figure 2). He is currently undergoing physiotherapy and has shown continued progress in his functional recovery. With only slight residual weakness in fine motor movements of the right hand and some drooling and slurring of speech, the patient is progressively getting better. He is now able to move his limbs and can speak few words.
Patient has been seizure-free for more than a year and is now being maintained on Carbamazepine.