Huntington's disease (HD), a neurodegenerative disease, affects approximately 30,000 people in the United States, with 200,000 more at risk. Mitochondrial dysfunction caused by mutant huntingtin (mHTT) drives early HD pathophysiology. mHTT binds the translocase of mitochondrial inner membrane (TIM23) complex, inhibiting mitochondrial protein import and altering the mitochondrial proteome.
View Article and Find Full Text PDFBackground And Objectives: The management of blunt cerebrovascular injuries (BCVIs) remains an important topic within trauma and neurosurgery today. There remains a lack of consensus within the literature and significant variation across institutions. The purpose of this study was to evaluate management of BCVI at a large, tertiary referral trauma center.
View Article and Find Full Text PDFA 41-year-old female presented with a headache and left inferior quadrantanopia. Imaging demonstrated a clot spanning the atrium of the ventricle to the superior parietal lobule (SPL), with a small arteriovenous malformation (AVM) nidus outside the atrium of the ventricle. The nidus was supplied by parieto-occipital arterial (P4) feeders with a single atrial draining vein.
View Article and Find Full Text PDFPrimary 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.
View Article and Find Full Text PDFOper Neurosurg (Hagerstown)
August 2021
Traumatic spinal cord injury (SCI) is a devastating neurological condition that involves both primary and secondary tissue loss. Various cytotoxic events including hypoxia, hemorrhage and blood lysis, bioenergetic failure, oxidative stress, endoplasmic reticulum (ER) stress, and neuroinflammation contribute to secondary injury. The HIF prolyl hydroxylase domain (PHD/EGLN) family of proteins are iron-dependent, oxygen-sensing enzymes that regulate the stability of hypoxia inducible factor-1α (HIF-1α) and also mediate oxidative stress caused by free iron liberated from the lysis of blood.
View Article and Find Full Text PDFOper Neurosurg (Hagerstown)
February 2021
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.
Background: Patients who survive aneurysmal subarachnoid hemorrhage (aSAH) are at risk for delayed neurological deficits (DND) and cerebral infarction. In this exploratory cohort comparison analysis, we compared in-hospital outcomes of aSAH patients administered a low-dose intravenous heparin (LDIVH) infusion (12 U/kg/h) vs those administered standard subcutaneous heparin (SQH) prophylaxis for deep vein thrombosis (DVT; 5000 U, 3 × daily).
Objective: To assess the safety and efficacy of LDIVH in aSAH patients.
Ruptured vertebrobasilar dissecting aneurysms require urgent, often challenging treatment as they have with a high re-hemorrhage rate within the first 24 hours. The patient is a 57-year-old woman who presented with severe-sudden onset headache. Further work up showed a ruptured dissecting aneurysm of the caudal loop of the posterior inferior cerebellar artery (PICA) with associated narrowing distally, in the ascending limb.
View Article and Find Full Text PDFCOVID-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.
View Article and Find Full Text PDFYucatan miniature pigs (YMPs) are similar to humans in spinal cord size as well as physiological and neuroanatomical features, making them a useful model for human spinal cord injury. However, little is known regarding pig gait kinematics, especially on a treadmill. In this study, 12 healthy YMPs were assessed during bipedal and/or quadrupedal stepping on a treadmill at six speeds (1.
View Article and Find Full Text PDFBackground: 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.
View Article and Find Full Text PDFObjective: Endovascular thrombectomy (ET) for acute large vessel occlusion reduces infarct size, and it should hypothetically decrease the incidence of major ischemic strokes requiring decompressive craniectomy (DC). The aim of this retrospective cohort study is to determine trends in the utilization of ET versus DC for stroke in the United States over a 10-year span.
Methods: We extracted data from the Nationwide Inpatient Sample using International Classification of Diseases-9/10 codes from 2006-2016.
The anterior choroidal artery (AChA) supplies important cerebral structures such as the internal capsule, optic tract, and lateral geniculate body. It is normally a branch of the communicating segment of the internal carotid artery (ICA) with significant neurological symptomatology if injured. Typically, the AChA originates distal to the posterior communicating artery (PCoA), but rare variants have previously been described.
View Article and Find Full Text PDFObjective Acute traumatic cervical spinal cord injury (AcSCI) causes significant morbidity and has an impact on health care utilization. The aim of our study was to analyze health care utilization in patients undergoing surgical decompression and fusion for AcSCI based on timing and type of surgical approaches. Patient and methods Data were extracted using ICD9/10 and CPT codes from MarketScan (IBM Corp.
View Article and Find Full Text PDFBackground: Posterior reversible encephalopathy syndrome (PRES) is a rare neurological disorder usually associated with specific medical conditions that cause a disturbance of the CNS homeostasis. It has seldom been reported to be a consequence of an iatrogenic intervention causing intracranial hypotension.
Case Presentation: We report the case of an individual 69-year-old male presenting with headache and blurred vision following cerebrospinal fluid (CSF) leak from resection of a sellar mass.
Background Cerebral venous thrombosis (CVT) is a rare cerebrovascular disorder, comprising <1% of all strokes. The incidence of CVT is higher in females but a small number of cases suggest that men have a higher risk for CVT in high elevation. The aim of this retrospective cohort study is to investigate this gender-related relationship and to describe the baseline characteristics and treatment outcomes of patients who suffered CVT at high altitude in eastern Nepal.
View Article and Find Full Text PDFActa Neurochir Suppl
October 2019
Aneurysmal subarachnoid hemorrhage (aSAH) remains a significant cause of stroke disability despite gradual reductions in physical morbidity and mortality. Heparin is an effective anti-inflammatory agent and may potentially prevent delayed neurological injury in the days to weeks after the hemorrhage. Various human studies have shown the safety of a continuous infusion of low-dose unfractionated heparin in the setting of subarachnoid hemorrhage as well as its efficacy in minimizing delayed neurological deficits including symptomatic cerebral vasospasm, vasospasm-related infarction, and cognitive dysfunction.
View Article and Find Full Text PDFProc Natl Acad Sci U S A
August 2019
Mutant huntingtin (mHTT), the causative protein in Huntington's disease (HD), associates with the translocase of mitochondrial inner membrane 23 (TIM23) complex, resulting in inhibition of synaptic mitochondrial protein import first detected in presymptomatic HD mice. The early timing of this event suggests that it is a relevant and direct pathophysiologic consequence of mHTT expression. We show that, of the 4 TIM23 complex proteins, mHTT specifically binds to the TIM23 subunit and that full-length wild-type huntingtin (wtHTT) and mHTT reside in the mitochondrial intermembrane space.
View Article and Find Full Text PDFBackground: Spontaneous intracranial hemorrhage (ICH) of the cerebellum can be life threatening because of mass effect on the brainstem and fourth ventricle. Suboccipital craniectomy is currently the treatment of choice for cerebellar ICH evacuation. Minimally invasive surgery (MIS) is currently being investigated for the treatment of supratentorial ICH.
View Article and Find Full Text PDFSpinal cord herniation (SCH) is an uncommon traumatic event that should be considered in patients with vertebral fractures who develop an unusual constellation of autonomic and motor deficits. Herein, we describe a case of rapidly deteriorating neurological function following cervical spine fracture including sequelae such as bilateral lower-extremity weakness, loss of upper extremity motor function, and priapism. Decompression of the spinal cord allowed for the identification of the unusual herniation of the spinal cord and prevention of any further worsening of the neurological injury.
View Article and Find Full Text PDFCavernous hemangiomas with an intrasellar extension are very rare, generally benign lesions that manifest by the compression of nearby structures. The presenting symptoms usually range from visual disturbances to an endocrine imbalance. Occasional extension into the cavernous sinus has been reported, which can cause cranial nerve compression.
View Article and Find Full Text PDFOBJECTIVEThe development and recent widespread dissemination of flow diverters may have reduced the utilization of surgical bypass procedures to treat complex or giant unruptured intracranial aneurysms (UIAs). The aim of this retrospective cohort study was to observe trends in cerebral revascularization procedures for UIAs in the United States before and after the introduction of flow diverters by using the National (Nationwide) Inpatient Sample (NIS).METHODSThe authors extracted data from the NIS database for the years 1998-2015 using the ICD-9/10 diagnostic and procedure codes.
View Article and Find Full Text PDF