Buttmccormick2521

Temporal-parietal-occipital carrefour epilepsy is part of the genetic epilepsy with febrile seizures plus spectrum. The electroclinical features in this family support the involvement of a genetically impaired neural network. High prevalence of post-ictal migraine suggests the role of posterior brain areas in the clinical expression of this gene defect.Perioral myoclonia with absences (POMA) is not recognized as a unique electro-clinical syndrome and studies suggest its inclusion under the genetic generalized epilepsy (GGE) spectrum. The aim of this study was to explore the prevalence and electro-clinical homogeneity of this disorder in an epilepsy monitoring unit. Between 2013 and 2019, among drug-resistant epilepsy patients who were referred for video-telemetry, those diagnosed with POMA based on the presence of documented absences with prominently observed peri-oral muscular contractions accompanied by generalized EEG features were included. Among 62 patients who were diagnosed with absence epilepsy, five finally met the criteria for POMA (8.1%) with late childhood or adolescent onset of epilepsy. Four (80%) had a referral diagnosis of focal epilepsy based on historical focal features with exacerbation of seizures on oxcarbazepine. All five patients demonstrated brief absences with orbicularis oris muscle contractions accompanied by subtle focal phenomenrhythms and valproate unresponsiveness are not consistent with the diagnosis of this unique absence epilepsy. [Published with video sequences].Epilepsia partialis continua (EPC) is a form of focal motor status epilepticus, associated with multiple etiologies. Etiology-specific treatments, such as hemispherotomy for Rasmussen encephalitis, lesionectomy for focal cortical dysplasia, and metabolic correction for non-ketotic hyperglycemia, have proven to be efficacious in treating EPC, but, in general, EPC is difficult to treat and often drug-resistant, and there is little evidence to guide therapy. We report the successful treatment of EPC with perampanel in two pediatric patients. The first patient was a 12-year-old boy with neuronal ceroid lipofuscinosis (NCL) who started to have EPC around the age of 10 years, characterized by left hemifacial myoclonic twitches and hemi-body jerks that were almost continuous throughout the day and disappeared during sleep. He had failed several antiepileptic drugs (AEDs). The EPC stopped within three days of initiating perampanel. The second patient was a six-year-old boy with POLG-related mitochondrial disease who presented to the emergency room with continuous jerky movements of the right arm and face after a trivial head injury. After failing several AEDs, including a midazolam drip, the EPC was controlled with perampanel. Both patients showed dramatic improvement and continue to show sustained efficacy after around five months of follow-up. selleck products Based on our observations, perampanel, which has a unique mechanism of action, appears to be a promising therapeutic option for treating EPC. [Published with video sequence].Sensory-guided limb control relies on communication across sensorimotor loops. For active touch with the hand, the longest loop is the transcortical continuation of ascending pathways, particularly the lemnisco-cortical and corticocortical pathways carrying tactile signals via the cuneate nucleus, ventral posterior lateral (VPL) thalamus, and primary somatosensory (S1) and motor (M1) cortices to reach corticospinal neurons and influence descending activity. We characterized excitatory connectivity along this pathway in the mouse. In the lemnisco-cortical leg, disynaptic cuneate→VPL→S1 connections excited mainly layer (L) 4 neurons. In the corticocortical leg, S1→M1 connections from L2/3 and L5A neurons mainly excited downstream L2/3 neurons, which excite corticospinal neurons. The findings provide a detailed new wiring diagram for the hand/forelimb-related transcortical circuit, delineating a basic but complex set of cell-type-specific feedforward excitatory connections that selectively and extensively engage diverse intratelencephalic projection neurons, thereby polysynaptically linking subcortical somatosensory input to cortical motor output to spinal cord.Current treatments of visceral leishmaniasis face limitations due to drug side effects and/or high cost, along with the emergence of parasite resistance. Novel and low-cost antileishmanial agents are therefore required. We report herein the antileishmanial activity of β-acetyl-digitoxin (b-AD), a cardenolide isolated from Digitalis lanata leaves, assayed in vitro and in vivo against Leishmania infantum. Results showed direct action of b-AD against parasites, as well as efficacy for the treatment of Leishmania-infected macrophages. In vivo experiments using b-AD-containing Pluronic® F127 polymeric micelles (b-AD/Mic) to treat L. infantum-infected mice showed that this composition reduced the parasite load in distinct organs in more significant levels. It also induced the development of anti-parasite Th1-type immunity, attested by high levels of IFN-γ, IL-12, TNF-α, GM-CSF, nitrite and specific IgG2a antibodies, in addition to low IL-4 and IL-10 contents, along with higher IFN-γ-producing CD4+ and CD8+ T-cell frequency. Furthermore, low toxicity was found in the organs of the treated animals. Comparing the therapeutic effect between the treatments, b-AD/Mic was the most effective in protecting animals against infection, when compared to the other groups including miltefosine used as a drug control. Data found 15 days after treatment were similar to those obtained one day post-therapy. In conclusion, the results obtained suggest that b-AD/Mic is a promising antileishmanial agent and deserves further studies to investigate its potential to treat visceral leishmaniasis.Recent studies suggest advantages to intramedullary nailing (IMN) of extra-articular proximal tibia fractures compared to plating. To our knowledge, no studies have evaluated IMN treatment of proximal tibia fractures with simple articular extension. We sought to compare rate of reoperation, malalignment, and patient-reported outcomes in patients with intra-articular versus extra-articular proximal tibia fractures treated via IMN. This retrospective cohort study compared patients that underwent IMN of extra-articular proximal tibia fractures (AO/OTA 41A2 and A3; n = 33) to simple intra-articular fractures (AO/OTA 41C1 and C2; n = 20) with minimum 12-month follow-up. With the numbers available, no significant differences were detected between the extra- and intra-articular groups for unplanned reoperation (9/33 vs. 2/20, p = 0.18), infection (4/33 vs. 1/20, p = 0.64), nonunion (4/33 vs. 2/20, p > 0.99), or malunion (5/30 vs. 3/19, p > 0.99). IMN of simple intra-articular proximal tibial fractures is a reasonable treatment strategy that may be desirable in certain clinical situations.