Value of sphenoidal electrodes in the EEG diagnosis of herpes encephalitis

EEG features in acute necrotizing encephalitis show periodic activity, localized in the temporal area with a slow background activity. Nevertheless, this periodic activity is transient and may lack. Sphenoidal electrodes study of the internal face of the temporal lobe could record such activity in herpes encephalitis. Sphenoidal electrodes were inserted in 4 patients known to have herpes encephalitis. In 2 cases, periodic discharges were observed both on the surface and sphenoidal electrodes, while in 2 cases, periodic discharge was observed under only sphenoidal electrodes. Faced with uncertain diagnosis of herpes encephalitis, it may be useful to insert sphenoidal electrodes to record deep periodic discharges, not seen on surface electrodes, in order to confirm the diagnosis.     

Malignant mullerian mixed tumor arising from ovarian serous carcinoma: a clinicopathologic and molecular study of two cases.

The clinical, histologic, and molecular pathologic features of two cases of malignant mullerian mixed tumor (MMMT) arising from ovarian papillary serous carcinoma are presented. Identical p53 mutations were detected in the primary ovarian carcinoma and the subsequent MMMT in each case.     

Aktueller Stand der Versorgung vorderer Beckenringfrakturen

The incidence of pelvic injury is increasing. In addition to high-speed trauma among younger patients, low-speed injuries among mainly older people (osteoporotic age-related fractures) play an important role. Pelvic ring stability is the most important consideration in the indication for conservative or surgical therapy. Unstable pelvic ring injuries are combined with severe concomitant injuries in >80% of cases and their primary treatment is usual in the context of multiple trauma management. In the case of anterior pelvic ring injuries (undisplaced/minimally displaced anterior pelvic ring fractures, pelvic rim breaks, type A avulsion fractures), fractures are usually stable and can be treated conservatively. Unstable pelvic ring fractures are generally treated surgically, enabling early functional aftercare in addition to anatomical reconstruction. Established osteosynthesis procedures for the anterior pelvic ring include external fixation, plate osteosynthesis and pubic rami screw. It is too early to say whether, and to what extent, new fixed-angle plate systems can improve the clinical results of surgically treated anterior pelvic ring injuries.     

Stroke volume measurements with first-pass dynamic positron emission tomography: Comparison with cardiovascular magnetic resonance

Background  The assessment of forward stroke volume (SV) using dynamic, first-pass cardiac positron emission tomography (PET) was shown to be feasible in a limited number of studies with small numbers of subjects. The aim of this study was to compare first-pass derived SV with cardiovascular magnetic resonance imaging (CMR)-obtained values in a larger population of subjects. Methods and Results  Fifty-nine subjects with varying degrees of cardiac function were studied. Stroke volume was assessed using oxygen-15-labeled water (H₂ ¹⁵O) dynamic first-pass PET for both the right ventricle (RV) and left ventricle (LV), and compared with the findings of aorta velocity-encoded phase-contrast CMR. The PET-estimated SV was higher for the RV than for the LV (133±34 vs 116±31 mL, P<.01, ±SD), and both were higher compared with values obtained by CMR (81±20 mL, both P<.01,±SD). Although significant, the correlations between PET and CMR were moderate for both the RV (r=0.37, P<.01) and the LV (r=0.40, P<.01,±SD). Bland-Altman analysis revealed a progressive overestimation with increasing SV measured in either ventricle. Conclusions  First-pass dynamic H₂ ¹⁵O PET for the assessment of forward SV is feasible, although values are progressively overestimated with increasing SV, particularly when the RV is used, and correlations with aorta velocity-encoded phase-contrast CMR are moderate. These findings are probably protocol-dependent and warrant further study before the use of first-pass dynamic H₂ ¹⁵O PET in clinical or research settings can be advocated.     

Graft intolerance syndrome requiring graft nephrectomy after late kidney graft failure: can it be predicted? A retrospective cohort study

Graft nephrectomy is recommended in case of early graft failure. When the graft fails more than 3–6 months after transplantation, it is current practice to follow a wait‐and‐see policy. A common indication for graft removal is the graft intolerance syndrome. We aimed to create a risk prediction model for the occurrence of graft intolerance resulting in graft nephrectomy. We collected data of kidney transplantations performed in our center between 1980 and 2010 that failed at least 6 months after transplantation. We evaluated the association between baseline characteristics and the occurrence of graft nephrectomy because of graft intolerance using a competing risk regression model. Prognostic factors were included in a multivariate prediction model. In‐ and exclusion criteria were met in 288 cases. In 48 patients, the graft was removed because of graft intolerance. Donor age, the number of rejections, and shorter graft survival were predictive factors for graft nephrectomy because of the graft intolerance syndrome. These factors were included in a prediction rule. Using donor age, graft survival, and the number of rejections, clinicians can predict the need for graft nephrectomy with a reasonable accuracy.     

GM3 synthase deficiency in non-Amish patients

PurposeBiallelic loss-of-function variants in ST3GAL5 cause GM3 synthase deficiency (GM3SD) responsible for Amish infantile epilepsy syndrome. All Amish patients carry the homozygous p.(Arg288Ter) variant arising from a founder effect. To date only 10 patients from 4 non-Amish families have been reported. Thus, the phenotypical spectrum of GM3SD due to other variants and other genetic backgrounds is still poorly known.MethodsWe collected clinical and molecular data from 16 non-Amish patients with pathogenic ST3GAL5 variants resulting in GM3SD.ResultsWe identified 12 families originating from Reunion Island, Ivory Coast, Italy, and Algeria and carrying 6 ST3GAL5 variants, 5 of which were novel. Genealogical investigations and/or haplotype analyses showed that 3 of these variants were founder alleles. Glycosphingolipids quantification in patients’ plasma confirmed the pathogenicity of 4 novel variants. All patients (N = 16), aged 2 to 12 years, had severe to profound intellectual disability, 14 of 16 had a hyperkinetic movement disorder, 11 of 16 had epilepsy and 9 of 16 had microcephaly. Other main features were progressive skin pigmentation anomalies, optic atrophy or pale papillae, and hearing loss.ConclusionThe phenotype of non-Amish patients with GM3SD is similar to the Amish infantile epilepsy syndrome, which suggests that GM3SD is associated with a narrow and severe clinical spectrum.