Treatment of Symptomatic Carotid Webs: A Single-Center Review.

BACKGROUND: Carotid webs are abnormal thin shelf-like or flap-like tissue in the carotid bulb (proximal internal carotid artery). Rarely are carotid webs detected prior to symptoms since routine carotid artery surveillance is not performed in younger individuals without traditional risk factors for carotid disease. The cause and natural history remain unknown. In general, they are not common but should be considered in the differential diagnosis of a patient who presents with ischemic neurologic symptoms. The web can create a flow disturbance, potentiating local thrombus formation, which can embolize producing resulting in cerebral ischemia. Current treatment is to reduce thrombus formation (antithrombotics and/or anticoagulation) or to alter the flow disturbance caused by the web (surgical removal or stent). METHODS: We retrospectively identified all patients presenting with acute ischemic stroke to our Comprehensive Stroke Center that were diagnosed with carotid web from January 2020 to December 2023. Patient demographics, presentation, hospital course including treatment and complications were collected and reported. RESULTS: Fifteen patients presented with carotid web and stroke from 2020 to 2023 and 13 underwent carotid artery stenting or endarterectomy with no periprocedural complications. Most (40%) carotid webs were not primarily identified by the initial radiology interpretation. CONCLUSIONS: We discuss our experience of carotid web and its management as well as review of the current literature.

Fatal Brain Injury Following Carbon Dioxide Angiography.

INTRODUCTION: Carbon dioxide (CO2) as a contrast agent has been in use as early as the 1920s for visualization of retroperitoneal structures. Digital subtraction angiography (DSA) using CO2 as a contrast agent for vascular imaging was developed in the 1980s. Currently, CO2  angiography is an alternative agent in patients with chronic kidney disease (CKD) and those who are at risk of developing contrast-induced nephropathy. However, CO2 causes neurotoxicity if the gas inadvertently enters the cerebrovascular circulation leading to fatal brain injury. CASE PRESENTATION: A 71-year-old female with h/o sickle cell trait, hypertension, obesity, metastatic renal cell cancer status post nephrectomy, bone metastasis, chronic kidney disease was admitted for elective embolization of the humerus bone metastasis. Given the high probability of contrast-induced nephropathy, CO2 angiography was chosen for embolization of the metastasis. During the procedure, the patient became unresponsive. Emergent medical management with hyperventilation, 100% fraction oxygen inhalation was performed. Her neuroimaging showed global cerebral edema. An intracranial pressure monitor was placed which confirmed intracranial hypertension. Hyperosmolar therapy was administered with no improvement in clinical examination. She progressed to brain stem herniation. Given poor prognosis, the family opted for comfort measures and the patient expired. DISCUSSION AND CONCLUSIONS: Inadvertent carbon dioxide entry into cerebrovascular circulation during angiography can cause fatal brain injury. Caution must be exercised while performing CO2  angiography in blood vessels above the diaphragm.

Anti-N-methyl-D-aspartate Encephalitis Concomitantly with Tall-cell Variant Papillary Thyroid Carcinoma.

Anti-N-methyl-D-aspartate (NMDA) encephalitis is an autoimmune-mediated process characterized by psychosis, seizures, dyskinetic movements, and autonomic instability. At least half of the reported cases are paraneoplastic, particularly associated with an ovarian teratoma. None have been reported to be associated with thyroid tumor. We present a case of anti-NMDA encephalitis concomitantly occurring in setting of papillary thyroid carcinoma in a woman who presented with headaches, myalgia and somnolence mimicking meningoencephalitis. A 29-year-old African female presented with fever, headache, myalgia, somnolence and behavioral changes. Initial evaluation was significant for lymphocytic pleocytosis with normal glucose and protein. She was started on broad spectrum empiric antibiotics. Despite antibiotics, she continued to have worsening encephalopathy, hallucinations, epileptic seizures, and multifocal dyskinesias involving the face and extremities with no electroencephalogram correlate. Extensive infectious workup was unremarkable. Whole-body CT with contrast and ovarian ultrasound were unremarkable for malignancy. Serum auto-antibodies to the NMDA receptor and thyroid peroxidase were detected. She was treated with high-dose intravenous steroids, plasmapheresis, intravenous immunoglobulin, and rituximab with no clinical or serological response. Fluorodeoxyglucose positron emission tomography (FDG-PET) showed a hypermetabolic thyroid nodule. Fine needle aspiration of the nodule revealed papillary thyroid carcinoma. She underwent total thyroidectomy and pathology showed two foci of tall-cell variant papillary thyroid carcinoma. Serological and clinical response followed shortly after tumor resection. The NMDA receptor stain of the papillary thyroid carcinoma was nonreactive. We describe the coincidentally co-occurrence of NMDA encephalitis in a patient with papillary thyroid carcinoma. This case highlights the importance of presumed cases of non-paraneoplastic NMDA encephalitis, FDG-PET may help in detecting occult malignancies.

Intracranial Calcification Masquerading as Hemorrhage in a Patient with Multiple Myeloma Presenting with Facial Neuropathy.

Multiple myeloma is an immunoglobulin-producing plasma cell neoplasm that commonly affects the bones, kidneys, the hematopoietic system, and rarely the nervous system. Peripheral nervous system involvement in the form of cranial neuropathy, radiculopathy, and polyneuropathy are common. Compressive myelopathy constitutes the majority of central nervous system disorders followed by cerebrovascular disorders, intracranial plasmacytomas, and leptomeningeal myelomatosis. Cerebrovascular complications such as acute ischemic stroke and transient ischemic attack are not uncommon. Intracerebral hemorrhage, although infrequent, can be secondary to refractory hypertension from renal failure and intratumoral hemorrhage in intracerebral plasmacytomas. Metastatic calcifications in lungs, liver, and skin with high levels of serum calcium and phosphate are seen in patients with multiple myeloma, but intracerebral calcifications are not common. We report an unusual case of intracranial calcification masquerading as acute intracerebral hemorrhage in a patient presenting with acute facial weakness.

Wernekink commissure syndrome with palatal myoclonus at onset: a case report and review of the literature.

BACKGROUND: Wernekink commissure syndrome causes a peculiar combination of internuclear ophthalmoplegia, dysarthria, and delayed-onset palatal myoclonus. Palatal myoclonus is thought to be secondary to delayed hypertrophic degeneration of the bilateral inferior olivary nuclei secondary to involvement of bilateral dentatoolivary tract. We describe a case of a patient with early-onset palatal myoclonus. CASE PRESENTATION: A 53-year-old Caucasian man with several vascular risk factors presented to our emergency room with slurred speech, double vision, difficulty with swallowing and walking, and rhythmic contractions of the soft palate. Brain magnetic resonance imaging showed an acute infarct of the right caudal midbrain and an old infarct of the right medulla. We hypothesize that the cause of early palatal myoclonus in our patient was a two-hit mechanism with degeneration of the right olivary nucleus resulting from prior right medullary lacunar stroke with the new infarct affecting the dentato-rubro-olivary tract on the left side, causing bilateral dysfunction initiating palatal myoclonus. CONCLUSIONS: Wernekink commissure syndrome with palatal myoclonus at onset suggests the presence of a prior ischemic insult in the medulla. Careful examination is important to identification of this presentation.

Brugada syndrome in a patient with amyotrophic lateral sclerosis: a case report.

BACKGROUND: Amyotrophic lateral sclerosis is a fatal neuromuscular disorder characterized by progressive death of the upper and lower motor neurons in the central nervous system. Patients with this disease die mostly as a result of respiratory failure; however, owing to prolonged survival through assisted ventilation, cardiovascular causes are increasingly responsible for mortality. We report what is to the best of our knowledge the first case of type 2 Brugada syndrome causing ventricular tachyarrhythmia and cardiac arrest in a patient with upper limb onset amyotrophic lateral sclerosis. CASE PRESENTATION: A 48-year-old Caucasian woman with a significant past medical history of papillary thyroid carcinoma status postresection, pulmonary embolism on anticoagulation, and a recent diagnosis of right upper limb-onset amyotrophic lateral sclerosis presented to the emergency department of our hospital with acute on chronic shortness of breath. On further evaluation, she was found to have hypoxic and hypercapnic respiratory failure and was placed on bilevel positive airway pressure ventilation. Her 12-lead electrocardiogram showed sinus rhythm with J-point elevation, saddle-shaped ST segment elevation, predominantly in V1 and V2 with no significant QTc prolongation. No troponin elevation was noted in her laboratory workup. Because she was unable to protect her airway, a decision was made to intubate her. After 1 minute of induction with etomidate and succinylcholine, she went into pulseless ventricular tachycardia and fibrillation requiring three cycles of cardiopulmonary resuscitation with high-quality chest compressions, three doses of epinephrine, and a loading dose of amiodarone prior to return of spontaneous circulation. She was further evaluated by cardiology services and was diagnosed with type 2 Brugada syndrome, for which she was started on quinidine. Her respiratory failure and the drugs she received for intubation likely caused her ventricular tachycardia to occur in conjunction with an underlying Brugada pattern seen on an electrocardiogram. The results of evaluation of her genetic panel for Brugada syndrome were negative. She was subsequently discharged to home in stable condition after a 10-day hospital stay. CONCLUSIONS: Amyotrophic lateral sclerosis is a progressive neuromuscular disorder with significant mortality. Respiratory failure is the leading cause of death, but lately, owing to increased survival associated with early tracheostomy and positive pressure ventilation, there has been an increasing trend in the identification of cardiovascular causes of mortality, especially arrhythmias, that may need periodic electrocardiographic surveillance.

Pediatric familial neuromyelitis optica in two sisters with long term follow-up.

Neuromyelitis optica causes bilateral optic neuritis and longitudinal extensive transverse myelitis. Although usually sporadic, 3% of cases of neuromyelitis optica are familial. The interval over which attacks continue and the long term prognosis for pediatric-onset neuromyelitis optica are not well defined. We describe two patients with pediatric familial neuromyelitis optica with the longest clinical follow-up of a pediatric case reported in the literature to our knowledge. One woman developed blindness with bilateral eye involvement within a few weeks at age 3. This was followed by transverse myelitis with paraparesis at age 19 leading to diagnosis of neuromyelitis optica. Her serum anti-aquaporin 4 antibody was later found to be positive. She continued with sporadic myelitis-related relapses but remained ambulant until age 40 when she had a more severe relapse. There was evidence of longitudinal extensive T2 hyperintensity in the thoracic spinal cord. Her sister also developed blindness at age 3.5 followed by myelitis 1year later with multiple relapses of gait impairment until her death from pneumonia at age 21. These patients represent the rare occurrence of neuromyelitis optica in children within the same family and show that this disease can have prolonged periods of remission but a continued tendency to relapse, supporting the need for lifelong immunosuppression.