Thrombotic Thrombocytopenic Purpura with Two Different Kinds of Brain Lesions

Abstract

Correspondence Neuroradiology ClinicalThrombotic thrombocytopenic purpura (TTP), also called Moschkowitz syndrome, is an uncommon disease (annual incidence four to eleven cases per million people) initially characterised by the following pentad of clinical features: hemolytic anaemia, thrombocytopenia, neurologic and renal abnormalities, and fever. With less stringent criteria, only hemolytic anemia and thrombocytopenia without an apparent alternative cause are required for diagnosis [1].TTP is associated with a severe deficiency of a von-Willebrand-factor -cleaving protease, termed ADAMTS-13 -, that cleaves the large von-Willebrandmultimers that are synthesized and secreted by endothelial cells. When ADAMTS-13 is not present, the resulting abnormally large von Willebrand factor multimers have a greater ability to react with platelets and cause the disseminated platelet thrombi characteristic of TTP [1]. If TTP is infect-associated, it is assumed that bacterial toxins bind to endothelial surfaces and initiate the production of cytokines and large von-Willebrandmultimers.Here, we describe a 45-year-old woman with two kinds of MR-findings: firstly of assumed pontine and extrapontine "myelinolysis" and secondly of multiple gray matter infarcts. Case ReportWe report on a 45-year-old severely agitated and confused woman complaining of increasing headache, that started after an infection of the upper air ways six weeks before. On admission, she did not have focal neurologic deficits. Her blood pressure was elevated to 280/140 mmHg; with the exception of ASS, no other drugs were taken. Cranial unenhanced CT showed hypodense lesions of the lateral thalamus and adjacent white matter on both sides (Figure 1a). On MRI, with FLAIR and T2-weighted fast spin echo sequences, hyperintense lesions of the pons sparing the outer rim were detected (Figure 1b). ADC values calculated from axial and coronal diffusion-weighted SE EPI sequences revealed increased values for both sides (115.5 x 10 -3 mm 2 /s right and 112.3 x 10 -3 mm 2 /s left, respectively), suggesting vasogenic edema. Intracerebral bleedings as well as infarctions were not found.Laboratory investigations revealed a Coombs-negative hemolytic anemia (hemoglobin 6.7 g/dl, thrombocytopenia 44,000/µl, fragmented red blood cells (3%), sodium 136 mmol/l, elevated levels of lactate dehydrogenase with 972 U/l) and a renal dysfunction (creatinine 6 mg/dl). Fundoscopic examination showed a hypertensive fundus. ADAMTS-13 (an acronym for disintegrin and metalloprotease with thrombospondin-1-like domains) antibodies were negative.Based on the clinical history and supported by laboratory findings, TTP was diagnosed and treated by repeated plasmapheresis together with the medication of fresh frozen plasma. The patient's confusional state disappeared slowly, but high creatinin levels did not change so that the patient needed hemodialysis at regular intervals furthermore. On follow-up MRI 22 days later, brain stem and basal ganglia lesions suggestive of pontine and extrapontine vasogeni...