Testing for the 14-3-3 protein is only modestly sensitive to sporadic CJD, and we caution against ruling out a diagnosis of the disease on the basis of a negative 14-3-3 result.

This case cautions against relying solely on T2- and diffusion-weighted pulvinar hyperintensity and clinical features to differentiate between vCJD and sCJD, and further supports established diagnostic criteria for vCJD.

A 42-year-old woman presented with myopathy and without a family history of neuromuscular disorder. Muscle biopsy showed ragged red fibers and reduced activities of mitochondrial respiratory chain enzyme complexes I, III, and IV. Analysis of mitochondrial DNA revealed a heteroplasmic T10010C mutation in the transfer RNA glycine gene.

Despite complex presentation of adult hypertension and a concomitant obesity epidemic, little is known about overweight in relation to blood pressure among Caribbean children. We examined blood pressure in relation to body size in a cross-sectional study of 573 Barbadian children aged 9–10 years (2010-2011).The United States normative blood pressure percentiles were used to identify children with high (≥ 95th percentile) or high normal blood pressure (90th – 95th percentile). The World Health Organization body mass index cut-off points were used to assess weight status.Major findingsThirty percent of children were overweight/obese. Percentage fat mass differed between girls (20.4%) and boys (17.72%) (p< 0.05). Mean systolic blood pressure among girls was 106.11 (95% CI 105.05, 107.17) mmHg and 105.23 (104.09, 106.38) for boys. The percentages with high or high-normal mean systolic blood pressurewere14.38% (10.47, 18.29) for girls and 8.08% (4.74, 11.41) for boys. Height and body mass index were independent correlates of systolic and diastolic blood pressure. Mean systolic blood pressure was related to lean mass but not fat mass, while diastolic blood pressure was associated with fat mass index and overweight.Principal conclusionOne third of 9-10 year old children in Barbados were overweight/obese and 12% had elevated mean systolic blood pressure. BP was related to body size. These findings signal potential adverse trends in weight gain and BP trends for children growing up in the context of a country that has recently undergone rapid economic transition.

Myxoinflammatory fibroblastic sarcoma (MIFS), originally described as a low-grade malignant soft-tissue tumor in adults, has recently been reported in children and in non-acral sites. This report describes the clinicopathologic features of a series of 5 MIFS in children and adolescents (3 males, 2 females), ranging in age from 5 to 17 years (mean, 13 years). These tumors presented as small, superficial, slowly growing soft-tissues masses of the scalp, neck, middle finger, forearm, and thigh. Histologically, the tumors were composed of spindled and plump polygonal cells with prominent nuclear pleomorphism, nuclear pseudoinclusions; large eosinophilic nucleoli; myxoid foci intermingled with spindled foci; and an accompanying inflammatory infiltrate of lymphocytes, plasma cells, and variable neutrophils. Immunohistochemical analysis revealed variable reactivity for CD34 and smooth muscle actin in the tumor cells. Genetic analysis in 3 cases showed no rearrangements of TGFBR3 or MGEA5. Follow up in 4 cases revealed no recurrence or metastasis. These 5 cases of childhood and adolescent MIFS demonstrate an expanded age range and topographic distribution and a favorable outcome. The differential diagnosis and importance of recognizing this rare neoplasm in young patients are discussed.