[P1-207] Thrombotic thrombocytopenic purpura in a 13-year-old girl presenting with Thunderclap headache and Reversible expressive aphasia: A case report
Thrombotic thrombocytopenic purpura (TTP) is a rare disorder characterized by extensive thrombosis in small blood vessels. Clinical characteristics include fever, Coombs-negative microangiopathic hemolytic anemia (MAHA), thrombocytopenic purpura, renal insufficiency and neurological symptoms. We reported a 13-year- old Thai girl who had thunderclap holocephalic headache associated with multiple vomiting. Soon after, she became expressive aphasic with right hemiparesis. Physical examination revealed high grade fever, moderate palor with petechiae and purpuras on chest wall, back and extremities. Computed tomography (CT) scans at 5 and 14 hours after onset of the headache were normal. Laboratory results showed anemia (Hb 8.2g/dl), thrombocytopenia (Plt 15,000/mm3) with MAHA blood picture. Direct and indirect Coomb's tests were negative. ADAMTS 13 (A disintegrin and metalloproteinase with a thrombospondin type I motif member protein 13) activity was less than 3% with inhibitor. Renal laboratory showed BUN 19 mg/dL and Creatinine 1.2 mg/dL. On second day of admission, aphasia and right hemiparesis were fully recovered. Magnetic resonance imaging (MRI) at 40 hours after onset showed no restricted diffusion on diffusion weighted imaging (DWI) and Apparent diffusion coefficient (ADC). On magnetic resonance angiography (MRA), asymmetric flow signal of middle cerebral arteries (MCA) was appreciated with the left MCA being larger than the right. The patient underwent plasmapheresis. Volume overload and cardiac arrest ensues. Resuscitative measures were unsuccessful. MRA that showed larger left middle cerebral artery may imply the reversibility. Cerebral perfusion studies and serial transcranial Doppler may give better insight.