OBJECTIVES The purpose of this study was to evaluate tip-to-base intentional laceration of the anterior mitral leaflet to prevent left ventricular outflow tract obstruction (LAMPOON) in patients undergoing transcatheter mitral valve replacement (TMVR) in annuloplasty rings or surgical mitral valves. BACKGROUND LAMPOON is an effective adjunct to TMVR that prevents left ventricular outflow tract obstruction (LVOTO). Laceration is typically performed from the base to the tip of the anterior mitral leaflet. A modified laceration technique from leaflet tip to base may be effective in patients with a prosthesis that protects the aortomitral curtain. METHODS This is a multicenter, 21-patient, consecutive retrospective observational cohort. Patients underwent tip-to-base LAMPOON to prevent LVOTO and leaflet overhang, or therapeutically to lacerate a long anterior mitral leaflet risking or causing LVOTO. Outcomes were compared with findings from patients in the LAMPOON investigational device exemption trial with a prior mitral annuloplasty. RESULTS Twenty-one patients with a annuloplasty or valve prosthesis-protected mitral annulus underwent tip-to-base LAMPOON (19 preventive, 2 rescue). Leaflet laceration was successful in all and successfully prevented or treated LVOTO in all patients. No patients had significant LVOTO upon discharge. There were 2 cases of unintentional aortic valve injury (1 patient underwent emergency transcatheter aortic valve replacement and 1 patient underwent urgent surgical aortic valve replacement). In both cases, the patients had a supra-annular ring annuloplasty, and the retrograde aortic guiding catheter failed to insulate the guidewire lacerating surface from the aortic root. All patients survived to 30 days. Compared with classic retrograde LAMPOON, there was a trend toward shorter procedure time. CONCLUSIONS Tip-to-base laceration is a simple, effective, and safe LAMPOON variant applicable to patients with an appropriately positioned mitral annular ring or bioprosthetic valve. Operators should take care to insulate the lacerating surface from adjacent structures. (J Am Coll Cardiol Intv 2021;14:541-50) (c) 2021 Published by Elsevier on behalf of the American College of Cardiology Foundation.

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OBJECTIVES The authors propose a novel transcatheter transection of the anterior mitral leaflet to prevent iatrogenic left ventricular outflow tract (LVOT) obstruction during transcatheter mitral valve replacement (TMVR).BACKGROUND LVOT obstruction is a life-threatening complication of TMVR caused by septal displacement of the anterior mitral leaflet.METHODS In vivo procedures in swine were guided by biplane x-ray fluoroscopy and intracardiac echocardiography. Retrograde transaortic 6-F guiding catheters straddled the anterior mitral leaflet. A stiff 0.014-inch guidewire with polymer jacket insulation was electrified and advanced from the LVOT, through the A2 leaflet base, into the left atrium. The wire was snared and externalized, forming a loop that was energized and withdrawn to lacerate the anterior mitral leaflet.RESULTS The anterior mitral leaflet was successfully lacerated in 7 live and 1 post-mortem swine under heparinization. Lacerations extended to 89 +/- 19% of leaflet length and were located within 0.5 +/- 0.4 mm of leaflet centerline. The chordae were preserved and retracted the leaflet halves away from the LVOT. LVOT narrowing after benchtop TMVR was significantly reduced with intentional laceration of the anterior mitral leaflet to prevent LVOT obstruction than without (65 +/- 10% vs. 31 +/- 18% of pre-implantation diameter, p < 0.01). The technique caused mean blood pressure to fall (from 54 +/- 6 mm Hg to 30 +/- 4 mm Hg, p < 0.01), but blood pressure remained steady until planned euthanasia. No collateral tissue injury was identified on necropsy.CONCLUSIONS Using simple catheter techniques, the anterior mitral valve leaflet was transected. Cautiously applied in patients, this strategy can prevent anterior mitral leaflet displacement and LVOT obstruction caused by TMVR. (C) 2016 by the American College of Cardiology Foundation.

Frederick Delius was born in Yorkshire, England, on June 29, 1862, the son of German immigrants. He showed early musical talent but his father, a wealthy wool merchant, insisted he pursue a business career. After several failures, including an assignment managing an orange grove near Jacksonville, Florida, his father agreed to support his musical studies in Leipzig, assuming he would then become self-sufficient. Delius spent most of his adult life in France, living with and ultimately marrying Jelka Rosen, a painter of independent means, composing prolifically, and being sexually promiscuous both before and after starting life with Jelka. He contracted syphilis in 1895 and manifestations of neurosyphilis appeared in 1910. Despite periods of relative good health over the ensuing 10 years, he became progressively disabled from 1920 on, ultimately quadriparetic and blind but with preserved cognition and musical inspiration. In his final years, he completed several compositions aided by a young British musician, Eric Fenby, who served as his amanuensis and caregiver, along with Jelka, to his death on June 10, 1934.

PURPOSE:To provide a rapid method to reduce the radiofrequency (RF) E-field coupling and consequent heating in long conductors in an interventional MRI (iMRI) setup.METHODS:A driving function for device heating (W) was defined as the integration of the E-field along the direction of the wire and calculated through a quasistatic approximation. Based on this function, the phases of four independently controlled transmit channels were dynamically changed in a 1.5 T MRI scanner. During the different excitation configurations, the RF induced heating in a nitinol wire immersed in a saline phantom was measured by fiber-optic temperature sensing. Additionally, a minimization of W as a function of phase and amplitude values of the different channels and constrained by the homogeneity of the RF excitation field (B1) over a region of interest was proposed and its results tested on the benchtop. To analyze the validity of the proposed method, using a model of the array and phantom setup tested in the scanner, RF fields and SAR maps were calculated through finite-difference time-domain (FDTD) simulations. In addition to phantom experiments, RF induced heating of an active guidewire inserted in a swine was also evaluated.RESULTS:In the phantom experiment, heating at the tip of the device was reduced by 92% when replacing the body coil by an optimized parallel transmit excitation with same nominal flip angle. In the benchtop, up to 90% heating reduction was measured when implementing the constrained minimization algorithm with the additional degree of freedom given by independent amplitude control. The computation of the optimum phase and amplitude values was executed in just 12 s using a standard CPU. The results of the FDTD simulations showed similar trend of the local SAR at the tip of the wire and measured temperature as well as to a quadratic function of W, confirming the validity of the quasistatic approach for the presented problem at 64 MHz. Imaging and heating reduction of the guidewire were successfully performed in vivo with the proposed hardware and phase control.CONCLUSIONS:Phantom and in vivo data demonstrated that additional degrees of freedom in a parallel transmission system can be used to control RF induced heating in long conductors. A novel constrained optimization approach to reduce device heating was also presented that can be run in just few seconds and therefore could be added to an iMRI protocol to improve RF safety.

Voltage-gated potassium channel (VGKC) complex antibody-mediated encephalitis is a recently recognised entity which has been reported to mimic the clinical presentation of Creutzfeldt-Jakob disease (CJD). Testing for the presence of this neuronal surface autoantibody in patients presenting with subacute encephalopathy is therefore crucial as it may both revoke the bleak diagnosis of prion disease and allow institution of potentially life-saving immunotherapy. Tempering this optimistic view is the rare instance when a positive VGKC complex antibody titre occurs in a definite case of prion disease. We present a pathologically and genetically confirmed case of CJD with elevated serum VGKC complex antibody titres. This case highlights the importance of interpreting the result of a positive VGKC complex antibody with caution and in the context of the overall clinical manifestation.

Alice Brandfonbrener died peacefully at home on May 31, 2014, after a protracted illness. For all of us involved in performing arts medicine, she was the inspiration and guiding spirit. She will be missed. [Alice Brandfonbrener was one of the founding members of the field of performing arts medicine, perhaps the most critical founding member. In 1983, she organized the first "Conference on the Medical Problems of Musicians" in Aspen, Colorado, and co-directed these for the next 20 years. These symposia led to the formation of the Performing Arts Medicine Association, of which Dr. Brandfonbrener was the first president, and in 1986, establishment of the first journal in the field--this journal, Medical Problems of Performing Artists--for which she served as Editor for 20 years. At her performing arts clinic in Chicago, she saw and treated thousands of musicians, from young students to world-renowned professionals, and mentored many students and professionals who would extend research and practice in performing arts medicine.].

Introduction: Isolated sural mononeuropathy is rare and frequently constitutes a diagnostic challenge. Methods: This investigation was a retrospective study of sural neuropathy at a single electrodiagnostic center. Results: Our study included 36 patients with sural neuropathy, the largest sample so far reported. Non-surgical, non-traumatic etiologies account for 50% of the cases, including 7 patients with inflammatory or vasculitic conditions. Routine sural conduction study was positive in 34 of 36 patients, whereas a distal recording method was used to verify the diagnosis of sural mononeuropathy in 2 patients. Most (58%) patients did not require specific treatment, but persistent sensory symptoms were seen in a minority of cases. Sural nerve biopsy in 1 patient helped diagnostic and treatment planning. Conclusions: Sural mononeuropathy has distinct etiologic, clinical, and electrophysiological features. Recognition can be beneficial in treating patients with sensory symptoms involving the distal lower extremity. Muscle Nerve49:443-445, 2014

OBJECTIVE: Individuals with Tourette syndrome (TS) often display co-morbid symptoms of attention-deficit hyperactivity disorder (ADHD) and obsessive-compulsive disorder (OCD). The aim of the study was to examine the factors that predict the long-term functional outcome of patients with TS, especially with co-morbid behavioral problems.

PURPOSE MR-guided cardiovascular interventions require imaging sequences that are fast, interactive and low SAR, when using conductive devices. Spiral trajectories use fewer RF pulses with longer readouts [1], making them particularly efficient with respect to SAR and SNR. However, imperfections in magnetic field gradient waveforms are problematic for non-Cartesian readouts and can lead to image distortions [2]. In addition, blurring caused by off-resonance effects during long readouts needs to be corrected during reconstruction [3,4]. In the context of interventional procedures, these corrections must be made in real-time, and it is therefore not practical to measure true k-space trajectories and field maps in each arbitrary image orientation. In this abstract, we present a real-time spiral imaging platform for MR-guided interventions with k-space trajectory correction from a one-time measured gradient system impulse response function (GIRF)[5] and interactive off-resonance reconstruction.

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INTRODUCTION:Creutzfeldt-Jakob disease (CJD) is a rapidly progressive dementia with a median survival of 2-14 months. The diagnosis can only be made accurately by biopsy/autopsy. However, this is not always feasible or desirable. Thus, diagnostic criteria have been proposed by UCSF, European MRI-CJD Consortium, and WHO. We will compare these criteria. PATIENTS AND METHODS:Retrospective study of 31 patients (average age of 69.2 years) between 2003 to 2010 by ICD9 codes 046.1, 046.11, and 046.19. RESULTS:All patients presented with rapidly progressive dementia (mean duration of 4.25 months). Pyramidal and extrapyramidal findings, myoclonus, cerebellar changes, akinetic mutism, and visual disturbances were observed in 6.5-48.4%. Five had periodic pattern on EEG. CSF biomarker 14-3-3 was positive in 11. Tau was positive in 6. Neuron specific enolase was positive in 9. By consensus (kappa = 0.62), MRI was "typical" of CJD in 23 with cortical ribboning (n = 16), basal ganglia hyperintensity (n = 15), or combination (n = 8). By WHO criteria, which does not include neuroimaging, CJD was diagnosed in 10, but 14 if any CSF biomarker was used (p = NS). The UCSF criteria, which does not include CSF biomarkers, diagnosed 18 cases, and the European MRI-CJD Consortium, which includes neuroimaging and CSF biomarkers but with less neurological signs, diagnosed 23 cases (p < 0.05 and p < 0.001, respectively). CJD-mimics included urosepsis, neurosarcoidosis, idiopathic left temporal lobe epilepsy, alcohol intoxication, central nervous system vasculitis, viral encephalitis, and non-Hodgkin's lymphoma. CONCLUSION:This study illustrates the variability in diagnosing CJD and emphasizes the diagnostic utility of neuroimaging. It also highlights false-positives that occur with neuroimaging.

Fear of a tremulous or unsteady bow is widespread among string instrumentalists. Faulty technique and performance anxiety have generally been blamed. The cases of 4 high-level violinists and 1 violist, 3 women and 2 men, with uncontrollable bow tremor are presented. Age at onset was from 16 to 75 years, and symptom duration 8 months to 20 years at the time of neurological evaluation. The degree of tremor varied with type of bow stroke and even the portion of the bow contacting the string. Only 1 patient had a 'slight postural tremor of the opposite limb. In 3 of 5 the tremor was tasks-pecific; the other 2 had mild and nontroubling tremor with other activities. The tremor appeared to worsen over time but then seemed to stabilize. The characteristics of this tremor appear to be distinguishable from the features of both essential tremor and focal dystonia; comparison is made with representative string players afflicted by these other disorders. Analogy of this tremor is made with primary writing tremor, a well-defined task-specific movement disorder also sharing at least some features with both essential tremor and writers' cramp, a focal dystonia. Hence, it was decided to call this primary bowing tremor. Clinical features, family history, diagnostic studies, and responsiveness to treatment of primary writing tremor are discussed to emphasize the similarity to primary bowing tremor. This appears to represent a previously unreported form of task-specific movement disorder of string instrumentalists. Med Probl Perform Art 2012; 27(4):219-223.

Duchenne muscular dystrophy (DMD) cardiomyopathy patients currently have no therapeutic options. We evaluated catheter-based transendocardial delivery of a recombinant adeno-associated virus (rAAV) expressing a small nuclear U7 RNA (U7smOPT) complementary to specific cis-acting splicing signals. Eliminating specific exons restores the open reading frame resulting in translation of truncated dystrophin protein. To test this approach in a clinically relevant DMD model, golden retriever muscular dystrophy (GRMD) dogs received serotype 6 rAAV-U7smOPT via the intracoronary or transendocardial route. Transendocardial injections were administered with an injection-tipped catheter and fluoroscopic guidance using X-ray fused with magnetic resonance imaging (XFM) roadmaps. Three months after treatment, tissues were analyzed for DNA, RNA, dystrophin protein, and histology. Whereas intracoronary delivery did not result in effective transduction, transendocardial injections, XFM guidance, enabled 30±10 non-overlapping injections per animal. Vector DNA was detectable in all samples tested and ranged from <1 to >3000 vector genome copies per cell. RNA analysis, western blot analysis, and immunohistology demonstrated extensive expression of skipped RNA and dystrophin protein in the treated myocardium. Left ventricular function remained unchanged over a 3-month follow-up. These results demonstrated that effective transendocardial delivery of rAAV-U7smOPT was achieved using XFM. This approach restores an open reading frame for dystrophin in affected dogs and has potential clinical utility.