Cranial ultrasonography in diagnosis of infantile cerebral cavernous malformation.

The case of an infantile cerebral cavernous malformation detected by transfontanelle cranial ultrasonography. Infantile cerebral cavernous malformations tend to cause more major bleeding compared to those in older groups, so early detection and treatment are crucial. Cranial ultrasonography can contribute to the early diagnosis of infantile cerebral cavernous malformations.

Serum tau protein as a marker of disease activity in enterohemorrhagic Escherichia coli O111-induced hemolytic uremic syndrome.

Tau protein levels in cerebrospinal fluid (CSF) and serum are elevated in patients with various central nervous system diseases. We investigated whether serum tau protein levels are useful for predicting and assessing disease activity of acute encephalopathy (AE) in enterohemorrhagic Escherichia coli (EHEC) O111-induced hemolytic uremic syndrome (HUS; EHEC encephalopathy). Serum samples were obtained from 14 patients with EHEC O111/HUS, 20 patients with non-EHEC-related AE, and 20 age- and sex-matched healthy controls. CSF samples were obtained from 2 patients with EHEC encephalopathy and 20 patients with non-EHEC-related AE. Tau protein levels and levels of several proinflammatory cytokines were quantified by enzyme-linked immunosorbent assays. Results were compared with the clinical features of EHEC encephalopathy, including magnetic resonance image (MRI) findings. Serum tau levels in patients with EHEC encephalopathy were significantly elevated compared with those in patients with EHEC O111/HUS without encephalopathy, patients with non-EHEC-related AE, and healthy controls. The ratio of CSF tau levels to serum tau levels was >1.0 in all patients with non-EHEC-related AE but <1.0 in 2 patients with EHEC encephalopathy. Serum tau protein levels increased rapidly and markedly in patients with severe EHEC 0111/HUS and encephalopathy when HUS occurred, but were not elevated in mild patients, even in the HUS phase. Furthermore, changes in serum tau protein levels in patients with EHEC encephalopathy were consistent with abnormalities on brain MRI and were positively correlated with proinflammatory cytokine levels. Our results indicate that serum tau protein might be useful to predict and assess disease activity of EHEC encephalopathy.

[Case of systemic-onset juvenile idiopathic arthritis complicated with sepsis --usefulness of cytokine profile for the differentiation between macrophage activation syndrome and sepsis].

The differential diagnosis of macrophage activation syndrome (MAS) and sepsis must be considered in the clinical course of systemic-onset juvenile idiopathic arthritis (s-JIA) with sudden onset of high-grade fever and abnormal laboratory findings, including leukocytopenia, thrombocytopenia, and coagulopathy. In this report, we describe the case of a 17-month-old girl diagnosed with s-JIA complicated with sepsis. Her serum interleukin (IL)-18 level was significantly elevated throughout the clinical course. Furthermore, compared to other MAS patients, she showed a significantly elevated serum IL-6 level and procalcitonin in sepsis. Therefore, our results suggest that a patient's cytokine profile may be a useful indicator of disease activity and may thus help in the differential diagnosis of sepsis and MAS in s-JIA.