A 30-year-old Filipino female admitted to our hospital with a 1-week history of fever and cough. The patient has diabetes mellitus 1 year ago, but she was not receiving any medication. Other medical histories were unremarkable. On examination, the patient looked well, not dyspneic. Her blood pressure was 110/70 mmHg, pulse rate 90 beats/min, respiratory rate 20 cycles/min and temperature 37°C. Chest examination revealed bronchial breathing with crackles, mainly on the right lung. The rest of the clinical examination otherwise was normal.\nBlood investigations revealed a hemoglobin level of 13.8 g/dL, a total leukocyte count of 12,900/mm3(91.7% neutrophils, 1.7% lymphocytes) and an adequate number of platelets. Erythrocyte sedimentation rate was 53 mm/hour. Blood chemistry, liver profile and coagulation studies were within normal limits. Chest X-ray showed widespread bilateral consolidation with cavity formation, suggesting active tuberculosis infection, whereas sputum was positive for acid fast bacilli (AFB). Human immunodeficiency virus enzyme-linked immunosorbent was negative.\nOn the 3rd day of hospitalization, four-drug antituberculous treatment was started. To control her blood sugar, the patient was kept on sliding scale insulin.\nTwo weeks after starting antituberculous treatment, the patient developed two episodes of loss of consciousness, which were unwitnessed. The exact duration was unknown, but when awakened, the patient complained of severe headache and backache. Neurological examination was unremarkable.\nECG and blood chemistry (including blood sugar), complete blood count (CBC) and liver profile were normal. Lumber puncture was carried out to rule out tuberculous meningitis. Cerebrospinal fluid (CSF) was clear with an opening pressure of 40 mm H2O, and analysis of CSF revealed the following values: WBC count of 5 cells/mm3(100% lymphocytes); glucose level 3.8 mmol/L; and protein level 0.78 g/L. Gram and Zeihl-Neelsen stains were negative. Echocardiography and holter monitoring also were normal, whereas urgent brain computed tomography (CT) showed multiple infarctions without abnormal dural enhancement [Figures and ]. Screening for thrombophilia and vasculitis was carried out. Anti-thrombin III, activated protein C resistance, factor V Leiden, protein C, protein S, clotting time, prothrombin time, activated partial thromboplastin time, fibrinogen, anticardiolipin antibody, lupus anticoagulant screen, homocystine, antinuclear antibody (ANA), C3, C4, C-reactive protein (CRP) and anti-neutrophil cytoplasmic antibodies (ANCAs), all these tests were within normal limits. The electroencephalogram (EEG) showed epileptic discharges. Magnetic resonance imaging (MRI) showed infarction at the head of the left caudate nucleus, the antero-lateral aspect of putamen and intervening internal capsule [], whereas magnetic resonance (MR) angiography showed a picture consistent with moyamoya. Brain CT angiography was performed and it showed the same pictures [].\nThe patient was diagnosed with pulmonary tuberculosis-associated moyamoya syndrome. On the following days, she was discharged on antituberculous, antiepileptic (Leveteracetam) and oral hypoglycemic (Gliclazide) medications. After 6 weeks, CSF culture failed to grow Mycobacterium tuberculosis. One year later, the patient was seen in the clinic; she was well and seizure-free.
A 23-month-old full-term Moroccan baby boy born to a non-consanguineous couple was referred to our hospital with the complaint of sudden onset left-sided hemiparesis evolving for 2 days. There was no history of fever, seizure, head injury, ear discharge, headache, visual disturbances, or vomiting. No family history of early stroke, ischemic heart disease, or autoimmune disease was reported. At presentation, he was conscious and well orientated. There was no dysmorphism, no neurocutaneous markers, and he had normal growth parameters. He was afebrile with a pulse of 110 beats per minute and a respiratory rate of 26 breaths per minute. On neurological examination, there were no signs of meningeal irritation, his gait was hemiplegic, tone was decreased over left side, power was 2/5 over left upper and lower limb using Medical Research Council (MRC) muscle strength scale, and deep tendon reflexes were exaggerated. There were no abnormalities on cardiac auscultation or hepatosplenomegaly. Brain computed tomography (CT) showed a low density area in the right cortical and subcortical frontotemporoparietal regions and right insular cortex representing ischemic lesions in the right middle cerebral artery (MCA) territory (Fig. ). Laboratory investigations, including hemogram, prothrombin, partial thromboplastin, erythrocyte sedimentation rate (ESR), fibrinogen, proteins C and S, antithrombin III activity, and antinuclear antibody tests were all within limits. Renal ultrasonography with Doppler flow study showed no evidence of renal artery stenosis. Electroencephalography revealed no epileptic discharges, only a diffuse slow rhythm over the left hemisphere with slow waves at the temporal regions.\nOur patient was booked for cerebral angiography with contrast, which revealed a proximal obliteration in his MCA with a fine anastomotic moyamoya network and typical “puff of smoke” appearance (Fig. ). Urine for metabolic screening, including homocysteine levels, was normal. A normal hemoglobin electrophoresis ruled out sickle cell disease. An electrocardiogram and echocardiogram revealed no abnormalities. He was finally diagnosed as a case of MMD.\nWe treated our patient conservatively with acetylsalicylic acid 5 mg/kg per day. He showed slight improvement in power over left side (3/5). He was then discharged to follow-up with pediatric neurosurgeon. Cerebral revascularization surgery using encephaloduroarteriosynangiosis (EDAS) was performed. At 8-month follow-up, his hemiparesis was improved and no further ischemic events had occurred.