Gloria M. Galloway

Sustained alpha‐sarcoglycan gene expression after gene transfer in limb‐girdle muscular dystrophy, type 2D

The aim of this study was to attain long‐lasting alpha‐sarcoglycan gene expression in limb‐girdle muscular dystrophy, type 2D (LGMD2D) subjects mediated by adeno‐associated virus (AAV) gene transfer under control of a muscle specific promoter (tMCK).

Limb-girdle muscular dystrophy type 2D gene therapy restores α-sarcoglycan and associated proteins†‡

α‐Sarcoglycan deficiency results in a severe form of muscular dystrophy (limb‐girdle muscular dystrophy type 2D [LGMD2D]) without treatment. Gene replacement represents a strategy for correcting the underlying defect. Questions related to this approach were addressed in this clinical trial, particularly the need for immunotherapy and persistence of gene expression.

Evidence-based guideline update: Intraoperative spinal monitoring with somatosensory and transcranial electrical motor evoked potentials: Report of the Therapeutics and Technology Assessment Subcommittee of the American Academy of Neurology and the American Clinical Neurophysiology Society

Objective: To evaluate whether spinal cord intraoperative monitoring (IOM) with somatosensory and transcranial electrical motor evoked potentials (EPs) predicts adverse surgical outcomes. Methods: A panel of experts reviewed the results of a comprehensive literature search and identified published studies relevant to the clinical question. These studies were classified according to the evidence-based methodology of the American Academy of Neurology. Objective outcomes of postoperative onset of paraparesis, paraplegia, and quadriplegia were used because no randomized or masked studies were available. Results and Recommendations: Four Class I and 8 Class II studies met inclusion criteria for analysis. The 4 Class I studies and 7 of the 8 Class II studies reached significance in showing that paraparesis, paraplegia, and quadriplegia occurred in the IOM patients with EP changes compared with the IOM group without EP changes. All studies were consistent in showing all occurrences of paraparesis, paraplegia, and quadriplegia in the IOM patients with EP changes, with no occurrences of paraparesis, paraplegia, and quadriplegia in patients without EP changes. In the Class I studies, 16%–40% of the IOM patients with EP changes developed postoperative-onset paraparesis, paraplegia, or quadriplegia. IOM is established as effective to predict an increased risk of the adverse outcomes of paraparesis, paraplegia, and quadriplegia in spinal surgery (4 Class I and 7 Class II studies). Surgeons and other members of the operating team should be alerted to the increased risk of severe adverse neurologic outcomes in patients with important IOM changes (Level A).

Evidence-based guideline update: intraoperative spinal monitoring with somatosensory and transcranial electrical motor evoked potentials*.

Objective To evaluate whether spinal cord intraoperative monitoring (IOM) with somatosensory and transcranial electrical motor evoked potentials (EPs) predict adverse surgical outcomes. Methods A panel of experts reviewed the results of a comprehensive literature search and identified published studies relevant to the clinical question. These studies were classified according to the evidence-based methodology of the American Academy of Neurology. Objective outcomes of postoperative onset of paraparesis, paraplegia, and quadriplegia were used because no randomized or masked studies were available. Results and Recommendations Four class I and eight class II studies met inclusion criteria for analysis. The four class I studies and seven of the eight class II studies reached significance in showing that paraparesis, paraplegia, and quadriplegia occurred in the IOM patients with EP changes compared with the IOM group without EP change. All studies were consistent in showing all occurrences of paraparesis, paraplegia, and quadriplegia in the IOM patients with EP changes, with no occurrences of paraparesis, paraplegia, and quadriplegia in patients without EP change. In the class I studies, 16% to 40% of the IOM patients with EP changes developed postoperative-onset paraparesis, paraplegia, or quadriplegia. IOM is established as effective to predict an increased risk of the adverse outcomes of paraparesis, paraplegia, and quadriplegia in spinal surgery (four class I and seven class II studies). Surgeons and other members of the operating team should be alerted to the increased risk of severe adverse neurologic outcomes in patients with important IOM changes (level A).

Intraoperative neurophysiologic monitoring in 80 patients with Chiari I malformation: role of duraplasty.

Neurophysiologic intraoperative monitoring of the brainstem auditory evoked potentials (BAEPs) is a widely used method to assess the functional integrity of the central auditory system during surgery involving the brainstem or the cranial nerves. The purpose of this study is to describe our experience with neurophysiologic intraoperative monitoring of BAEPs during posterior fossa decompression (PFD) surgery for the management of Chiari I malformation. Although suboccipital craniectomy is the standard surgical technique applied in all cases undergoing PFD, the role of dural patch grafting (duraplasty) remains controversial. In most cases, the PFD was supplemented by duraplasty only when the Chiari I malformation was complicated by the presence of syringomyelia. Our study reviewed the intraoperative BAEP changes during the different surgical stages of Chiari repair and correlated these with clinical and radiological findings present. Our data revealed that for both groups of patients, with or without associated syringomyelia, the predominant improvement in central conduction in most cases occurred during the period of bony decompression without significant additional improvement after the duraplasty procedure.

Neurophysiologic Intraoperative Monitoring in Pediatrics

Neurophysiologic intraoperative monitoring, using somatosensory, brainstem auditory, and visual evoked potentials, transcranial electric motor stimulation, and electromyography, is typically used during complex surgeries involving the motor and sensory cortex, brainstem, cranial nerves, spinal cord, nerve root, peripheral roots, brachial plexus, lumbar plexus, and peripheral nerves. The particular type of surgery and the neurologic structures at risk determine the type of monitoring chosen. Although many methods are the same in adult and pediatric patients, some differences in the pediatric population will be discussed here. In general, monitoring consists of two types. The first involves monitoring data which is obtained on an ongoing basis, with comparisons to data obtained at the outset of surgery (baseline). The second form of monitoring involves mapping neural structures, so that a neural structure in the field is identified accurately, to avoid injuring it, or to demonstrate its degree of neurophysiologic function or impairment. In this paper we discuss both forms of monitoring and their general applications, including unique features or modifications needed in the pediatric population.

Intraoperative monitoring: do you know where your neurophysiologist is?

Objective: The professional practice of intraoperative monitoring has evolved over the past 30 years. This report describes the field’s current state and how site of service affects practice. Methods: A survey queried American Academy of Neurology intraoperative monitoringneurologist members about their intraoperative monitoring volume, case type, duration, numbers of simultaneous cases, and location of the monitoring physician. Results: Physicians located locally typically monitored fewer cases annually and simultaneously compared with physicians who monitored from remote locations. Physicians at remote locations monitored proportionally more spine procedures, whereas physicians who monitored locally monitored more intracranial procedures and a greater variety of cases. Conclusions: The remote monitoring practice model is different from local models in annual volume, simultaneous cases, work per case, and types of cases.