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AJ  Fay  T  McMahon  C  Im  C  Bair-Marshall  KJ  Niesner  H  Li  A  Nelson  SM  Voglmaier  Y-H  Fu  LJ  Ptáček 《Neurogenetics》2021,22(3):171-185

Paroxysmal kinesigenic dyskinesia is an episodic movement disorder caused by dominant mutations in the proline-rich transmembrane protein PRRT2, with onset in childhood and typically with improvement or resolution by middle age. Mutations in the same gene may also cause benign infantile seizures, which begin in the first year of life and typically remit by the age of 2 years. Many details of PRRT2 function at the synapse, and the effects of mutations on neuronal excitability in the pathophysiology of epilepsy and dyskinesia, have emerged through the work of several groups over the last decade. However, the age dependence of the phenotypes has not been explored in detail in transgenic models. Here, we report our findings in heterozygous and homozygous Prrt2 knockout mice that recapitulate the age dependence of dyskinesia seen in the human disease. We show that Prrt2 deletion reduces the levels of synaptic proteins in a dose-dependent manner that is most pronounced at postnatal day 5 (P5), attenuates at P60, and disappears by P180. In a test for foot slippage while crossing a balance beam, transient loss of coordination was most pronounced at P60 and less prominent at age extremes. Slower traverse time was noted in homozygous knockout mice only, consistent with the ataxia seen in rare individuals with biallelic loss of function mutations in Prrt2. We thus identify three age-dependent phenotypic windows in the mouse model, which recapitulate the pattern seen in humans with PRRT2-related diseases.

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BackgroundAlthough pelvic osteotomy (PO) is an important surgical procedure that can alleviate symptoms and potentially slow progression of osteoarthritis in patients with development dysplasia of the hip, some patients eventually require conversion to total hip arthroplasty (THA). This study aimed to determine the outcome of conversion THA in patients with prior PO.MethodsForty nine patients with a history of prior PO who underwent conversion THA at a single institution were matched at a 1:3 ratio based on the date of surgery, age, gender, and body mass index with 147 developmental dysplasia of the hip patients who underwent primary THA without prior PO. A retrospective chart review was performed to compare outcomes at a minimum follow-up of 2 years.ResultsPatients with prior PO required more supplemental screw fixation for the acetabular component (59.2% vs 38.1%, P = .016), more autologous bone grafting (24.5% vs 11.6%, P = .048), had a longer mean operative time (106.0 vs 79.8 minutes, P < .001), and greater estimated blood loss (350.0 vs 206.8 mL, P = .015). Patients with prior PO had smaller cup version angle (26.0° vs 29.0°, P = .012) and greater discrepancy in the limb length (10.3 vs 7.26 mm, P = .041). Eight hips (16.3%) with prior PO and 6 (4.1%) without osteotomy required reoperation (P = .008). There was no difference in outcome scores at the latest follow-up.Conclusion: THA after prior PO is technically demanding, leading to longer operative times, greater blood loss, and variation in implant placement. Although functional outcomes are similar, THA after a prior PO is more likely to require reoperation.ConclusionTHA after prior PO is technically demanding, leading to longer operative times, greater blood loss, and variation in implant placement. Although functional outcomes are similar, THA after a prior PO is more likely to require reoperation.  相似文献   
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Osteogenesis imperfecta (OI) type V is an ultrarare heritable bone disorder caused by the heterozygous c.-14C > T mutation in IFITM5. The oro-dental and craniofacial phenotype has not been described in detail, which we therefore undertook to evaluate in a multicenter study (Brittle Bone Disease Consortium). Fourteen individuals with OI type V (age 3–50 years; 10 females, 4 males) underwent dental and craniofacial assessment. None of the individuals had dentinogenesis imperfecta. Six of the 9 study participants (66%) for whom panoramic radiographs were obtained had at least one missing tooth (range 1–9). Class II molar occlusion was present in 8 (57%) of the 14 study participants. The facial profile was retrusive and lower face height was decreased in 8 (57%) individuals. Cephalometry, performed in three study participants, revealed a severely retrusive maxilla and mandible, and moderately to severly retroclined incisors in a 14-year old girl, a protrusive maxilla and a retrusive mandible in a 14-year old boy. Cone beam computed tomograpy scans were obtained from two study participants and demonstrated intervertebral disc calcification at the C2-C3 level in one individual. Our study observed that OI type V is associated with missing permanent teeth, especially permanent premolar, but not with dentinogenesis imperfecta. The pattern of craniofacial abnormalities in OI type V thus differs from that in other severe OI types, such as OI type III and IV, and could be described as a bimaxillary retrusive malocclusion with reduced lower face height and multiple missing teeth.  相似文献   
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While we often engage in conversational reminiscing with loved ones, the effects of these conversations on our memory performance remain poorly understood. On the one hand, Wegner's transactive memory theory predicts that intimate groups experience benefits from remembering together. On the other hand, research on collaborative recall has shown costs of shared remembering in groups of strangers—at least in terms of number of items recalled—and even in intimate groups there is heterogeneity in outcomes. In the current research, we studied the effects of particular communicative features in determining the outcomes of collaborative recall in intimate groups. We tested 39 older, long‐married couples. They completed a non‐personal recall task (name all the countries in Europe) and a personal recall task (name all your mutual friends), both separately and together. When they collaborated, we recorded their conversation. We coded for specific “communication variables” and obtained measures of “conversational style.” Overall, we found two clusters of communication variables positively associated with collaborative success: (a) cuing each other, responding to cues, and repeating each other; and (b) making positive statements about memory performance and persisting with the task. A negative cluster of behaviors—correcting each other, having uneven expertise, and strategy disagreements—was associated with less interactive, more “monologue” style of collaboration, but not with overall recall performance. We discuss our results in terms of the importance of different conversational processes in driving the heterogeneous outcomes of group remembering in intimate groups, suggesting that a focus on recall output alone limits our understanding of conversational remembering.  相似文献   
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Aims. Small encephaloceles of the anterior temporal pole have been increasingly recognised as an underlying epileptogenic substrate in patients with medically refractory epilepsy. The current report aims to expand on the current knowledge by emphasising that seizure semiology in such patients can vary significantly. Methods. Patients were selected from an epilepsy surgery database between 2012 and 2017. Results. Of the 143 patients who underwent epilepsy surgery, six patients had a temporal encephalocele. Four of these patients had stereo-EEG implantation. Of the four patients studied, each had a seizure semiology discordant with an ictal focus in the temporal lobe. Intracranial EEG assessment demonstrated, irrespective of this semiology, seizures originated from the anterior temporal pole. Seizures were observed to rapidly propagate to the orbitofrontal cortex, insula, temporo-occipital junction, and posterior language regions. Engagement of the mesial temporal structures could occur early or late, however, a good surgical outcome was achieved following a focused lesionectomy in either situation. Conclusion. The major finding was that seizures arising from anterior temporal encephaloceles can have an extra-temporal semiology. The varied clinical semiology and the rapid propagation to seemingly distant cortical regions could be explained by the connectivity of the anterior temporal lobe.  相似文献   
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Minimal residual disease (MRD) during early chemotherapy is a powerful predictor of relapse in acute lymphoblastic leukaemia (ALL) and is used in children to determine eligibility for allogeneic haematopoietic stem cell transplantation (HSCT) in first (CR1) or later complete remission (CR2/CR3). Variables affecting HSCT outcome were analysed in 81 children from the ANZCHOG ALL8 trial. The major cause of treatment failure was relapse, with a cumulative incidence of relapse at 5 years (CIR) of 32% and treatment‐related mortality of 8%. Leukaemia‐free survival (LFS) and overall survival (OS) were similar for HSCT in CR1 (LFS 62%, OS 83%, n = 41) or CR2/CR3 (LFS 60%, OS 72%, n = 40). Patients achieving bone marrow MRD negativity pre‐HSCT had better outcomes (LFS 83%, OS 92%) than those with persistent MRD pre‐HSCT (LFS 41%, OS 64%, P < 0·0001) or post‐HSCT (LFS 35%, OS 55%, P < 0·0001). Patients with B‐other ALL had more relapses (CIR 50%, LFS 41%) than T‐ALL and the main precursor‐B subtypes including BCR‐ABL1, KMT2A (MLL), ETV6RUNX1 (TELAML1) and hyperdiploidy >50. A Cox multivariate regression model for LFS retained both B‐other ALL subtype (hazard ratio 4·1, P = 0·0062) and MRD persistence post‐HSCT (hazard ratio 3·9, P = 0·0070) as independent adverse prognostic variables. Persistent MRD could be used to direct post‐HSCT therapy.  相似文献   
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