Faculty Bibliography

This evidence-based clinical practice guideline for the prevention, diagnosis, and treatment of Lyme disease was developed by a multidisciplinary panel representing the Infectious Diseases Society of America (IDSA), the American Academy of Neurology (AAN), and the American College of Rheumatology (ACR). The scope of this guideline includes prevention of Lyme disease, and the diagnosis and treatment of Lyme disease presenting as erythema migrans, Lyme disease complicated by neurologic, cardiac, and rheumatologic manifestations, Eurasian manifestations of Lyme disease, and Lyme disease complicated by coinfection with other tick-borne pathogens. This guideline does not include comprehensive recommendations for babesiosis and tick-borne rickettsial infections, which are published in separate guidelines. The target audience for this guideline includes primary care physicians and specialists caring for this condition such as infectious diseases specialists, emergency physicians, internists, pediatricians, family physicians, neurologists, rheumatologists, cardiologists and dermatologists in North America.

This evidence-based clinical practice guideline for the prevention, diagnosis, and treatment of Lyme disease was developed by a multidisciplinary panel representing the Infectious Diseases Society of America (IDSA), the American Academy of Neurology (AAN), and the American College of Rheumatology (ACR). The scope of this guideline includes prevention of Lyme disease, and the diagnosis and treatment of Lyme disease presenting as erythema migrans, Lyme disease complicated by neurologic, cardiac, and rheumatologic manifestations, Eurasian manifestations of Lyme disease, and Lyme disease complicated by coinfection with other tick-borne pathogens. This guideline does not include comprehensive recommendations for babesiosis and tick-borne rickettsial infections, which are published in separate guidelines. The target audience for this guideline includes primary care physicians and specialists caring for this condition such as infectious diseases specialists, emergency physicians, internists, pediatricians, family physicians, neurologists, rheumatologists, cardiologists and dermatologists in North America.

Epilepsy is a heterogenous group of disorders defined by recurrent seizure activity due to abnormal synchronized activity of neurons. A growing number of epilepsy cases are believed to be caused by genetic factors and copy number variants (CNV) contribute to up to 5% of epilepsy cases. However, CNVs in epilepsy are usually large deletions or duplications involving multiple neurodevelopmental genes. In patients who underwent seizure focus resection for treatment-resistant epilepsy, whole genome DNA methylation profiling identified 3 main clusters of which one showed strong association with receptor tyrosine kinase (RTK) genes. We identified focal copy number gains involving epidermal growth factor receptor (EGFR) and PDGFRA loci. The dysplastic neurons of cases with amplifications showed marked overexpression of EGFR and PDGFRA, while glial and endothelial cells were negative. Targeted sequencing of regulatory regions and DNA methylation analysis revealed that only enhancer regions of EGFR and gene promoter of PDGFRA were amplified, while coding regions did not show copy number abnormalities or somatic mutations. Somatic focal copy number gains of noncoding regulatory represent a previously unrecognized genetic driver in epilepsy and a mechanism of abnormal activation of RTK genes. Upregulated RTKs provide a potential avenue for therapy in seizure disorders.

Recurrent episodes of neurological dysfunction and white matter lesions in a young adult raise suspicion for multiple sclerosis (MS). However, occlusive retinopathy, hearing loss and absence of CSF oligoclonal bands are atypical for MS and should make the clinician consider an alternative diagnosis. We describe a man with hearing loss, visual signs and symptoms, and an accumulating burden of brain lesions, who was treated for a clinical diagnosis of MS for nearly two decades. Genetic testing revealed a unifying diagnosis.

OBJECTIVE:To analyze the association between peri-ictal brainstem posturing semiologies with post-ictal generalized electroencephalographic suppression (PGES) and breathing dysfunction in generalized convulsive seizures (GCS). METHODS:Prospective, multicenter analysis of GCS. Ictal brainstem semiology was classified as (1) decerebration: bilateral symmetric tonic arm extension, (2) decortication: bilateral symmetric tonic arm flexion only, (3) hemi-decerebration: unilateral tonic arm extension with contralateral flexion and (4) absence of ictal tonic phase. Post-ictal posturing was also assessed. Respiration was monitored using thoraco-abdominal belts, video and pulse oximetry. RESULTS:= 0.035). CONCLUSIONS:recovery. Peri-ictal brainstem posturing may be surrogate biomarkers for GCS severity identifiable without in-hospital monitoring. CLASSIFICATION OF EVIDENCE/METHODS:This study provides Class III evidence that peri-ictal brainstem posturing is associated with the GCS with more prolonged PGES and more severe breathing dysfunction.

BACKGROUND:The COVID-19 pandemic led to profound changes in the organization of health care systems worldwide. AIMS/OBJECTIVE:We sought to measure the global impact of the COVID-19 pandemic on the volumes for mechanical thrombectomy (MT), stroke, and intracranial hemorrhage (ICH) hospitalizations over a 3-month period at the height of the pandemic (March 1 to May 31, 2020) compared with two control 3-month periods (immediately preceding and one year prior). METHODS:Retrospective, observational, international study, across 6 continents, 40 countries, and 187 comprehensive stroke centers. The diagnoses were identified by their ICD-10 codes and/or classifications in stroke databases at participating centers. RESULTS:The hospitalization volumes for any stroke, ICH, and MT were 26,699, 4,002, and 5,191 in the 3 months immediately before versus 21,576, 3,540, and 4,533 during the first 3 pandemic months, representing declines of 19.2% (95%CI,-19.7 to -18.7), 11.5% (95%CI,-12.6 to -10.6), and 12.7% (95%CI,-13.6 to -11.8), respectively. The decreases were noted across centers with high, mid, and low COVID-19 hospitalization burden, and also across high, mid, and low volume stroke/MT centers. High-volume COVID-19 centers (-20.5%) had greater declines in MT volumes than mid- (-10.1%) and low-volume (-8.7%) centers (p<0.0001). There was a 1.5% stroke rate across 54,366 COVID-19 hospitalizations. SARS-CoV-2 infection was noted in 3.9% (784/20,250) of all stroke admissions. CONCLUSION/CONCLUSIONS:The COVID-19 pandemic was associated with a global decline in the volume of overall stroke hospitalizations, MT procedures, and ICH admission volumes. Despite geographic variations, these volume reductions were observed regardless of COVID-19 hospitalization burden and pre-pandemic stroke/MT volumes.

The aim of this study was to investigate the effects of Equistasi^®, a wearable device, on the relationship between muscular activity and postural control changes in a sample of 25 Parkinson's disease (PD) subjects. Gait analysis was carried out through a six-cameras stereophotogrammetric system synchronized with two force plates, an eight-channel surface electromyographic system, recording the activity of four muscles bilaterally: Rectus femoris, tibialis anterior (TA), biceps femoris, and gastrocnemius lateralis (GL). The peak of the envelope (PoE) and its occurrence within the gait cycle (position of the peak of the envelope, PPoE) were calculated. Frequency-domain posturographic parameters were extracted while standing still on a force plate in eyes open and closed conditions for 60 s. After the treatment with Equistasi^®, the mid-low (0.5-0.75) Hz and mid-high (0.75-1 Hz) components associated with the vestibular and somatosensory systems, PoE and PPoE, displayed a shift toward the values registered on the controls. Furthermore, a correlation was found between changes in proprioception (power spectrum frequencies during the Romberg Test) and the activity of GL, BF (PoE), and TA (PPoE). Results of this study could provide a quantitative estimation of the effects of a neurorehabilitation device on the peripheral and central nervous system in PD.

Individuals with upper extremity (UE) amputation abandon prostheses due to challenges with significant device weight-particularly among myoelectric prostheses-and limited device dexterity, durability, and reliability among both myoelectric and body-powered prostheses. The Modular Prosthetic Limb (MPL) system couples an advanced UE prosthesis with a pattern recognition paradigm for intuitive, non-invasive prosthetic control. Pattern recognition accuracy and functional assessment-Box & Blocks (BB), Jebsen-Taylor Hand Function Test (JHFT), and Assessment of Capacity for Myoelectric Control (ACMC)-scores comprised the main outcomes. 10 participants were included in analyses, including seven individuals with traumatic amputation, two individuals with congenital limb absence, and one with amputation secondary to malignancy. The average (SD) time since limb loss, excluding congenital participants, was 85.9 (59.5) months. Participants controlled an average of eight motion classes compared to three with their conventional prostheses. All participants made continuous improvements in motion classifier accuracy, pathway completion efficiency, and MPL manipulation. BB and JHFT improvements were not statistically significant. ACMC performance improved for all participants, with mean (SD) scores of 162.6 (105.3), 213.4 (196.2), and 383.2 (154.3), p = 0.02 between the baseline, midpoint, and exit assessments, respectively. Feedback included lengthening the training period to further improve motion classifier accuracy and MPL control. The MPL has potential to restore functionality to individuals with acquired or congenital UE loss.

BACKGROUND:Middle meningeal artery (MMA) embolization has emerged as a promising treatment for chronic subdural hematoma (cSDH). OBJECTIVE:To determine the safety and efficacy of MMA embolization. METHODS:Consecutive patients who underwent MMA embolization for cSDH (primary treatment or recurrence after conventional surgery) at 15 centers were included. Clinical details and follow-up were collected prospectively. Primary clinical and radiographic outcomes were the proportion of patients requiring additional surgical treatment within 90 d after index treatment and proportion with > 50% cSDH thickness reduction on follow-up computed tomography imaging within 90 d. National Institute of Health Stroke Scale and modified Rankin Scale were also clinical outcomes. RESULTS:A total of 138 patients were included (mean age: 69.8, 29% female). A total of 15 patients underwent bilateral interventions for 154 total embolizations (66.7% primary treatment). At presentation, 30.4% and 23.9% of patients were on antiplatelet and anticoagulation therapy, respectively. Median admission cSDH thickness was 14 mm. A total of 46.1% of embolizations were performed under general anesthesia, and 97.4% of procedures were successfully completed. A total of 70.2% of embolizations used particles, and 25.3% used liquid embolics with no significant outcome difference between embolization materials (P > .05). On last follow-up (mean 94.9 d), median cSDH thickness was 4 mm (71% median thickness reduction). A total of 70.8% of patients had >50% improvement on imaging (31.9% improved clinically), and 9 patients (6.5%) required further cSDH treatment. There were 16 complications with 9 (6.5%) because of continued hematoma expansion. Mortality rate was 4.4%, mostly unrelated to the index procedure but because of underlying comorbidities. CONCLUSION/CONCLUSIONS:MMA embolization may provide a safe and efficacious minimally invasive alternative to conventional surgical techniques.

BACKGROUND:Resection of large nerve sheath tumors in the lumbar spine using minimally invasive approaches is challenging, as approaches to tumors in this region may require facetectomy or partial resection of adjacent ribs for access to the involved neuroforamen and instrumentation across the involved joint to prevent subsequent kyphotic deformity. OBJECTIVE:To describe a robot-assisted retroperitoneal approach for resection of a lumbar nerve sheath tumor, obviating the need for facetectomy and instrumentation. The operation is described, together with intraoperative images and an annotated video, in the context of a schwannoma arising from the right L1 root. METHODS:The operation was performed by a urologic surgeon and a neurosurgeon. The patient was placed in lateral position, and the da Vinci Xi robot was used for retroperitoneal access via 5 ports along the right flank. Ultrasound was used to localize the tumor within the psoas. The tumor capsule was defined and released. Encountered nerves were stimulated, allowing small sensory nerves to be identified and safely divided. The tumor was traced into the right L1-L2 neuroforamen and removed. RESULTS:Complete en bloc resection of the tumor was achieved, including the paraspinal and foraminal components, without any removal of bone and without violation of the dura. CONCLUSION/CONCLUSIONS:In selected patients, a robot-assisted retroperitoneal approach represents a minimally invasive alternative to traditional approaches for resection of lumbar nerve sheath tumors. This approach obviates the need for bone removal and instrumented spinal fusion. Interdisciplinary collaboration, as well as use of adjunctive technologies, including intraoperative ultrasound and neurophysiologic monitoring, is advised.