Pseudoanginal chest pain associated with vagal nerve stimulation: a case report.

BACKGROUND: Vagal nerve stimulation (VNS) can be an effective therapy for patients with epilepsy refractory to anti-epileptic drugs or intracranial surgery. While generally well tolerated, it has been associated with laryngospasm, hoarseness, coughing, dyspnea, throat and atypical chest pain, cardiac symptoms such as bradycardia and occasionally asystole. We report on a patient receiving vagal nerve stimulation who experienced severe typical anginal chest pain during VNS firing without any evidence of cardiac ischemia or dysfunction. Thus, the pain appeared to be neuropathic from the stimulation itself rather than nociceptive secondary to an effect on heart function. CASE PRESENTATION: A 29-year-old man, with a history of intractable frontal lobe epilepsy refractory to seven anti-epileptic medications and subsequent intracranial surgery, underwent VNS implantation without complications. On beginning stimulation, he began to have intermittent chest pain that corresponded temporally to his intermittent VNS firing. The description of his pain was pathognomonic of ischemic cardiac chest pain. On initial evaluation, he displayed Levine's sign and reported crushing substernal chest pain radiating to the left arm, as well as shortness of breath walking upstairs that improved with rest. He underwent an extensive cardiac workup, including 12-lead ECG, cardiac stress test, echocardiogram, 12-day ambulatory cardiac monitoring, and continuous ECG monitoring each with and without stimulation of his device. The workup was consistently negative. Inability to resolve the pain necessitated the disabling and eventual removal of the device. CONCLUSION: To our knowledge, this is the first report of pseudoanginal chest pain associated with VNS. This occurrence prompted our review of the mechanisms of cardiac chest pain and suggests that vagal afferents may convey anginal pain separately or in parallel with known spinal cord pain mechanisms. These insights into the physiology of chest pain may be of general interest and important to surgeons implanting VNS devices who may potentially encounter such symptoms.

Cortical gene expression correlates of temporal lobe epileptogenicity.

INTRODUCTION: Despite being one of the most common neurological diseases, it is unknown whether there may be a genetic basis to temporal lobe epilepsy (TLE). Whole genome analyses were performed to test the hypothesis that temporal cortical gene expression differs between TLE patients with high vs. low baseline seizure frequency. METHODS: Baseline seizure frequency was used as a clinical measure of epileptogenicity. Twenty-four patients in high or low seizure frequency groups (median seizures/month) underwent anterior temporal lobectomy with amygdalohippocampectomy for intractable TLE. RNA was isolated from the lateral temporal cortex and submitted for expression analysis. Genes significantly associated with baseline seizure frequency on likelihood ratio test were identified based on >0.90 area under the ROC curve, P value of <0.05. RESULTS: Expression levels of forty genes were significantly associated with baseline seizure frequency. Of the seven most significant, four have been linked to other neurologic diseases. Expression levels associated with high seizure frequency included low expression of Homeobox A10, Forkhead box A2, Lymphoblastic leukemia derived sequence 1, HGF activator, Kelch repeat and BTB (POZ) domain containing 11, Thanatos-associated protein domain containing 8 and Heparin sulfate (glucosamine) 3-O-sulfotransferase 3A1. CONCLUSIONS: This study describes novel associations between forty known genes and a clinical marker of epileptogenicity, baseline seizure frequency. Four of the seven discussed have been previously related to other neurologic diseases. Future investigation of these genes could establish new biomarkers for predicting epileptogenicity, and could have significant implications for diagnosis and management of temporal lobe epilepsy, as well as epilepsy pathogenesis.

Primary Spinal Glioblastoma Mimicking Neuroschistosomiasis: A Case Report.

Primary glioblastoma of the spinal cord (sGB) is a rare and challenging diagnosis. In the diagnostic algorithm, reversible causes should be considered while the diagnosis of sGB is under evaluation. We present a case of cervical sGB mimicking neuroschistosomiasis. A 21-year-old Somali man presented with neck pain, sensory disturbances, and spastic tetraplegia. Cervical spine magnetic resonance imaging with contrast showed a heterogeneously enhancing intramedullary mass spanning from the level of the C1 to T3 vertebrae. Cerebrospinal fluid analysis showed a lymphocytic predominance and elevated protein. Due to the patient's history of poorly treated schistosomiasis, praziquantel and dexamethasone were initiated while the diagnostic work-up was completed. Three days after the patient was discharged to a rehabilitation facility where he experienced worsened motor function with radiographic progression of the lesion and increased cord edema. The patient underwent a surgical biopsy which confirmed a diagnosis of primary sGB. sGB is an unusual diagnosis that can masquerade as a non-neoplastic lesion. However, the diagnosis of sGB should be considered in patients with an intramedullary spinal cord lesion who exhibit rapid radiographic and clinical progression.

Thecal sac ligation for the treatment of post-infectious intrathecal-prevertebral CSF fistula in a paraplegic patient.

The occurrence of a symptomatic post-infectious intrathecal to prevertebral fistula is rare. We report the presentation and management of a 38-year-old chronically paraplegic male with longstanding thoracic osteomyelitis and epidural infection who developed a cerebrospinal fluid (CSF) fistula causing symptomatic intracranial hypotension (IH). During an interventional radiology (IR)-guided aspiration of what was thought to be residual abscess, pulsatile, clear fluid was observed. A subsequent CT myelogram showed air in the spinal canal and a CSF fistula between the thecal sac and the pre-vertebral space. Upon intraoperative exploration, a large ventral dural defect was identified with insufficient native dura for primary closure and the thecal sac was tied off cranial to the level of the fistula. Given the large ventral dural defect, the fistula was likely the result of longstanding infection in the epidural space rather than the IR guided aspiration. The aspiration likely transgressed an existing fistula and may have exacerbated the symptoms of IH by providing another route for CSF egress. The patient's postural headaches completely resolved post-operatively. Thecal sac ligation is a viable treatment option in select circumstances with symptomatic CSF fistula.

Minimally Invasive Endoscopy for Acute Subdural Hematomas: A Report of 3 Cases.

BACKGROUND: Acute subdural hematomas (aSDHs) occur in approximately 10% to 20% of all closed head injury and represent a significant cause of morbidity and mortality in traumatic brain injury patients. Conventional craniotomy is an invasive intervention with the potential for excess blood loss and prolonged postoperative recovery time. OBJECTIVE: To evaluate the outcomes of minimally invasive endoscopy for evacuation of aSDHs in a pilot feasibility study. METHODS: We retrospectively reviewed the records of consecutive patients with aSDHs who underwent surgical treatment at our institution with minimally invasive endoscopy using the Apollo/Artemis Neuro Evacuation Device (Penumbra, Alameda, California) between April 2015 and July 2018. RESULTS: The study cohort comprised three patients. The Glasgow Coma Scale on admission was 15 for all 3 patients, median preoperative hematoma volume was 49.5 cm3 (range 44-67.8 cm3), median postoperative degree of hematoma evacuation was 88% (range 84%-89%), and median modified Rankin Scale at discharge was 1 (range 0-3). CONCLUSION: Endoscopic evacuation of aSDHs can be a safe and effective alternative to craniotomy in appropriately selected patients. Further studies are needed to refine the selection criteria for endoscopic aSDH evacuation and evaluate its long-term outcomes.

Intracranial hemorrhage in a young COVID-19 patient.

COVID-19 patients are increasingly understood to develop multisystem manifestations, including neurologic involvement. We report the case of a 42-year old COVID-19 positive patient with a fatal intracerebral hemorrhage (ICH). The patient presented with fever and dyspnea, requiring intubation due to medical complications. After prolonged sedation and anticoagulation, the patient suddenly developed bilaterally fixed and dilated pupils, caused by a right-sided intracranial hemorrhage with uncal herniation. The course of this case illustrates the delicate balance between hypercoagulability and coagulation factor depletion; especially in the intubated and sedated patient, in whom regular neurological assessments are impeded. As we expand our understanding of the neurological ramifications of COVID-19, clinicians need to be increasingly aware of the precarious coagulation balance.

Motor neurons and glia exhibit specific individualized responses to TDP-43 expression in a Drosophila model of amyotrophic lateral sclerosis.

Amyotrophic lateral sclerosis (ALS) is a fatal disease characterized by complex neuronal and glial phenotypes. Recently, RNA-based mechanisms have been linked to ALS via RNA-binding proteins such as TDP-43, which has been studied in vivo using models ranging from yeast to rodents. We have developed a Drosophila model of ALS based on TDP-43 that recapitulates several aspects of pathology, including motor neuron loss, locomotor dysfunction and reduced survival. Here we report the phenotypic consequences of expressing wild-type and four different ALS-linked TDP-43 mutations in neurons and glia. We show that TDP-43-driven neurodegeneration phenotypes are dose- and age-dependent. In motor neurons, TDP-43 appears restricted to nuclei, which are significantly misshapen due to mutant but not wild-type protein expression. In glia and in the developing neuroepithelium, TDP-43 associates with cytoplasmic puncta. TDP-43-containing RNA granules are motile in cultured motor neurons, although wild-type and mutant variants exhibit different kinetic properties. At the neuromuscular junction, the expression of TDP-43 in motor neurons versus glia leads to seemingly opposite synaptic phenotypes that, surprisingly, translate into comparable locomotor defects. Finally, we explore sleep as a behavioral readout of TDP-43 expression and find evidence of sleep fragmentation consistent with hyperexcitability, a suggested mechanism in ALS. These findings support the notion that although motor neurons and glia are both involved in ALS pathology, at the cellular level they can exhibit different responses to TDP-43. In addition, our data suggest that individual TDP-43 alleles utilize distinct molecular mechanisms, which will be important for developing therapeutic strategies.