Citations

Sixty-four children, aged 0-17 years, undergoing ambulatory electroencephalography (EEG) were prospectively recruited during a 12-month period. The diagnostic yield of ambulatory electroencephalography was determined for each of the following groups: group 1: differentiation of seizures from nonepileptic events; group 2: determination of seizure/interictal discharge frequency; and group 3: classification of seizure type or localization. The ambulatory electroencephalography answered the clinical question in 61% of group 1 (27/44) and 100% of groups 2 (16/16) and 3 (4/4). Of 44 cases in Group 1, clinical events were recorded in 61%; the ambulatory electroencephalography result changed the diagnosis from epileptic to nonepileptic or vice versa in 27%. When clinicians suspected that events were epileptic, ambulatory electroencephalography changed the clinical impression in 50%, whereas when events were suspected to be nonepileptic, ambulatory electroencephalography confirmed that impression in 83%.

Background and Purpose Hemipelvectomy is a life-changing treatment for pelvic malignancies. This study compared functional outcomes and quality of life of patients following internal or external hemipelvectomies. Subjects Ninety-seven patients who underwent tumor-related internal (n=39) or external (n=58) hemipelvectomy surgery between January 1, 1988, and December 31, 2004, participated in the study. Methods Using a descriptive retrospective cohort study design, functional status was evaluated with the Barthel Index at 3 time points. Quality-of-life parameters were evaluated at follow-up using the Linear Analog Self-Assessment tool (LASA). Results Data were collected on all 97 patients at rehabilitation consultation and hospital discharge. Follow-up data were obtained via survey on 44% of the original group at a median of 5.8 years (interquartile range [IRQ]=1.7–10.4) after surgery. Median total Barthel Index scores were similar between the internal and external hemipelvectomy groups at the initial physical medicine and rehabilitation assessment (10 [IQR=10–15] versus 10 [IQR=3.75–15]), at discharge (40 [IQR=30–65] versus 50 [IQR=35–66.25]), and at follow-up (92.5 [IQR=76.25–100] versus 92.5 [IQR=78.75–96.25]). Participants with external hemipelvectomies were less independent in bladder function and experienced greater pain severity at follow-up compared with those with internal hemipelvectomies. Overall quality-of-life parameters were similar between the groups. Discussion and Conclusion Despite significant differences in surgical procedures, immediate and long-term functional outcomes and quality-of-life parameters were similar among participants with internal and external hemipelvectomies.

Background— Outflow tract ventricular tachycardia originating above the semilunar valves has been reported in a small number of studies. Discrete potentials in the great arteries (above the semilunar valves) have been rarely described in patients undergoing electrophysiology evaluation and radiofrequency ablation for ventricular arrhythmias. The mechanisms of these discrete potentials in the great arteries and the utility of such potentials in guiding radiofrequency ablation are unknown. Methods and Results— Twelve patients with outflow tract ventricular arrhythmia originating above the semilunar valves with discrete arterial potentials were studied. The clinical characteristics, properties of the arterial potentials, electrophysiological evaluation and ablation, and short- and long-term outcomes were reviewed. Of the twelve patients, 8 (67%) were women. The patients’ average age was 41�14 years. The average ejection fraction was 0.52�0.16 (range: 0.16 to 0.75). Contact mapping in the great artery demonstrated discrete near-field electrograms that were separate from far-field ventricular electrograms in all patients (8 above the pulmonary valve and in 4 the aortic valve). One or more of the following electrophysiological characteristics, supportive of an arrhythmogenic substrate, were observed in 10 of 12 patients: (1) A fixed or reproducibly variable pattern of discrete potential–ventricular arrhythmia relationship was present at baseline or during pacing; (2) the discrete potential–ventricular electrogram relationship during sinus rhythm was the reverse of that during the ventricular arrhythmia; (3) during sustained ventricular tachycardia, spontaneous variation of the ventricular (V-V) cycle length was preceded by a similar variation of arterial spike potential–spike potential cycle length; and (4) ablation guided by the discrete arterial potential successfully eliminated the clinical arrhythmia. Ablation was successful in these patients. In the remaining 2 patients, the potentials were believed to be bystanders. Over 10�4 months (range: 5 to 32 months) of follow-up, there have been no recurrences of the premature ventricular complex or ventricular arrhythmia. Conclusions— Discrete potentials are present in the great arteries of a select group of patients with outflow tract ventricular tachycardia originating above the semilunar valves. When an arrhythmogenic relationship can be demonstrated, discrete potentials are useful in guiding ablation within the great vessels, despite significant anatomic complexity.

Eps15 (EGFR pathway substrate clone 15) is well known for its role in clathrin-coated vesicle formation at the plasma membrane through interactions with other clathrin adaptor proteins such as AP-2. Interestingly, we observed that in addition to its plasma membrane localization, Eps15 is also present at the trans-Golgi network (TGN). Therefore, we predicted that Eps15 might associate with clathrin adaptor proteins at the TGN and thereby mediate the formation of Golgi-derived vesicles. Indeed, we have found that Eps15 and the TGN clathrin adaptor AP-1 coimmunoprecipitate from rat liver Golgi fractions. Furthermore, we have identified a 14-amino acid motif near the AP-2–binding domain of Eps15 that is required for binding to AP-1, but not AP-2. Disruption of the Eps15–AP-1 interaction via siRNA knockdown of AP-1 or expression of mutant Eps15 protein, which lacks a 14-amino acid motif representing the AP-1 binding site of Eps15, significantly reduced the exit of secretory proteins from the TGN. Together, these findings indicate that Eps15 plays an important role in clathrin-coated vesicle formation not only at the plasma membrane but also at the TGN during the secretory process.

ObjectiveTo evaluate the image quality of coronal reformatted CT images compared with direct coronal CT images.Study DesignCross‐sectional.Subjects and MethodsPatients who underwent paranasal sinus CT scans using both direct coronal images (DC) and axially acquired images with coronal reconstructions (CR) were consecutively enrolled. Two reconstruction groups were evaluated: group CR1 with 1‐mm axial collimation and group CR2 with 0.625‐mm axial collimation. Scans were presented in a randomized, blinded fashion to two sinus surgeons and a neuroradiologist.ResultsWhen compared with CR1, DC images had higher image quality for each of five key anatomic landmarks (P <. 0001). DC images were more likely to be adequate preoperative imaging (P <. 0001). When compared with CR2, improved DC image quality was noted in the majority of key anatomic landmarks. No difference was noted in the “adequacy rating” between the DC and CR2 (0.625 mm) protocols.ConclusionThe 0.625‐mm reformatted images were judged to be adequate preoperative imaging. The additional information provided by multiple‐image planes make reformatted 0.625‐mm axial images an acceptable approach.

One hundred two patients with Sturge-Weber disease who were seen at the Mayo Clinic between 1942 and 1986 were studied retrospectively to determine the difference in prognosis between unihemispheric (88 patients) and bihemispheric (14 patients) involvement. Seizures occurred in 63 with unihemispheric involvement and 13 with bihemispheric; the mean age at onset of seizures was 24 months in the former and 6 months in the latter. Of the total group, 19% were severely or moderately mentally retarded, 27% were mentally retarded but educable, and 45% had average intelligence. In the bihemispheric involvement group, 46% were severely or moderately mentally retarded, 38% were retarded but educable, and only 8% had average intelligence. Bilateral involvement of the brain by Sturge-Weber disease is associated with earlier onset of seizures and worse prognosis for mental development compared with unilateral involvement. ( J Child Neurol 1988;3:181-184).

The electrophysiologic evaluation of peripheral nerves may provide critically important information, both with respect to diagnosis and prognosis, in the child with a suspected neuromuscular disorder. However, special attention to various technical considerations is necessary to avoid misleading results. Utilizing these techniques, both hereditary and acquired neuropathies may be identified and characterized. The latter has become especially important in view of recent advances in the treatment of acquired demyelinating neuropathies. (J Child Neurol 1986;1:19-26)