A retrospective review of sex differences of white matter hyperintensities in brain MRI of patients with migraine.

OBJECTIVES: The primary objective of this study was to evaluate the prevalence of white matter hyperintensities (WMHs) in patients who experience migraine and compare findings between adult male and female patients. Specific symptoms and comorbidities also were analyzed to determine whether they were associated with WMH prevalence or the sex of patients with migraine. We hypothesized that females would have a higher prevalence of WMHs, experience more frequent and more severe migraine headaches, and be more likely to have certain comorbidities associated with migraine than males. BACKGROUND: An increased prevalence of WMHs in patients with migraine has been proposed, although this relation is not well-supported by data from population-based MRI studies. The difference in brain morphology between males and females is of research interest, and females in the general population appear to have a higher prevalence of WMHs. Sex differences and various comorbidities in patients with migraine relative to the number of WMHs on brain imaging have not been fully investigated. METHODS: This was a cross-sectional study of 177 patients aged 18 years and older with a diagnosis of migraine who were seen in the Lehigh Valley Fleming Neuroscience Institute's Headache Center between January 1, 2000, and January 1, 2017. Patients' baseline characteristics were extracted from electronic medical records, including demographics, review of systems documentation, and brain imaging from MRI. Variables including headache severity, frequency of head pain, insomnia, and comorbidities (anxiety, depression, diabetes, hyperlipidemia, hypertension, and neck pain) also were analyzed for associations with the presence of WMHs. RESULTS: Females were found to have a significantly higher number of WMHs than males (median 3 [IQR: 0-7] vs. 0 [IQR: 0-3], p = 0.023). Patients with WMHs were significantly more likely than those without WMHs to have hypertension (39.8% of patients with WMHs vs. 20.3% without WMHs, p = 0.011), constipation (20.9% vs. 8.3%, p = 0.034), and sleep disorder (55.7% vs. 37.3%, p = 0.022). Females with migraine were significantly more likely to experience constipation than males (20.0% vs. 2.9%, p = 0.015). None of the migraine characteristics studied (frequency, severity, presence of aura) were different between sexes, nor were they significantly associated with the presence of WMHs. CONCLUSION: This study suggests that females with migraine may be more likely to have WMHs and experience constipation than males with migraine. Migraine frequency and severity were not different between sexes, nor were they significantly associated with the presence of WMHs. The findings of this study do not support a specific etiology of WMH development in individuals with migraine that differs from findings in the general population. Further studies are warranted.

Hyperacute hyponatremia mimicking acute ischemic stroke.

We present a case of hyperacute hyponatremia with stroke like symptoms on presentation. Symptoms included confusion, left-sided facial droop, right-sided hemiparesis, dysarthria and aphasia, with an NIH stroke score of 5. Sodium level at the time of presentation was 119 mmol/L which dropped acutely from 138 mmol/L seven hours prior. Symptoms improved after treatment with 3% saline and no evidence of stroke, intracranial hemorrhage or space-occupying lesion was seen on imaging. The most likely cause of the hyponatremia was increased free water consumption and ADH surge. The patient remained symptom free after discharge with resolution of hyponatremia. Acute hyponatremia can cause focal neurological complaints and deficits, mimicking acute ischemic stroke. We advise clinicians to be aware of this entity when considering interventions for possible acute ischemic stroke and evaluating a patient with focal neurological deficits.

The Effect of Regional Anesthetic Sphenopalatine Ganglion Block on Self-Reported Pain in Patients With Status Migrainosus.

BACKGROUND: Status migrainosus (SM) is defined as a debilitating migraine attack lasting more than 72 hours in patients previously known to suffer from migraine headache. Typically, these attacks fail to respond to over the counter and abortive medications. The sphenopalatine ganglion (SPG) plays a critical role in propagating both pain and the autonomic symptoms commonly associated with migraines. SPG block via transnasal lidocaine is moderately effective in reducing migraine symptoms, but this approach is often poorly tolerated and the results are inconsistent. We proposed that an SPG block using a suprazygomatic injection approach would be a safe and effective option to abort or alleviate pain and autonomic symptoms of SM. METHODS: Through a retrospective records review, we identified patients with a well-established diagnosis of migraine, based on the International Headache Society criteria. Patients selected for study inclusion were diagnosed with SM, had failed to respond to 2 or more abortive medications, and had received a suprazygomatic SPG block. Patients had also been asked to rate their pain on a 1-10 Likert scale, both before and 30 minutes after the injection. RESULTS: Eighty-eight consecutive patients (20 men and 68 women) received a total of 252 suprazygomatic SPG block procedures in the outpatient headache clinic after traditional medications failed to abort their SM. At 30 minutes following the injections, there was a 67.2% (±26.6%) reduction in pain severity with a median reduction of 5 points (IQR= -6 to -3) on the Likert scale (ranging from 1 to 10). Overall, patients experienced a statistically significant reduction in pain severity (P < .0001). CONCLUSION: The SPG is known to play an integral role in the pathophysiology of facial pain and the trigeminal autonomic cephalalgias, although its exact role in the generation and maintenance of migraine headache remains unclear. Regional anesthetic suprazygomatic SPG block is potentially effective for immediate relief of SM. We believe the procedure is simple to perform and has minimal risk.

Transient Capgras Syndrome Secondary to Bilateral Ischemic Stroke: A Case Report.

Capgras syndrome is one of a variety of delusional misidentification syndromes that can be associated with acute ischemic stroke, neurodegenerative disease, or metabolic conditions. Most cases reported in the literature are associated with frontal and/or parietal lobe involvement. Transient Capgras syndrome is rare but has been reported. We present a case of transient Capgras syndrome following bilateral cerebral ischemic infarcts in the frontal, parietal, and temporal regions, and involving the right prefrontal cortex. To our knowledge, transient Capgras syndrome with rapid resolution over a period of days is rare.

Open Heart Surgery Does Not Increase the Incidence of Ipsilateral Ischemic Stroke in Patients with Asymptomatic Severe Carotid Stenosis.

BACKGROUND AND PURPOSE: We evaluated the incidence of perioperative stroke following the institution's 2007 practice change of discontinuing combined carotid endarterectomy and open heart surgery (OHS) for patients with severe carotid stenosis. METHODS: In this retrospective cohort study, we compared 113 patients undergoing coronary artery bypass grafting, aortic valve replacement, or both from 2007 to 2011 with data collected from 2001 to 2006 from a similar group of patients. Our aim was to assess whether the practice change led to a greater incidence of stroke. RESULTS: A total of 7350 consecutive patients undergoing OHS during the specified time period were screened. Of these, 3030 had OHS between 2007 and 2011 but none were combined with carotid artery surgery (new cohort). The remaining 4320 had OHS before 2007 and 44 had combined procedures (old cohort). Of patients undergoing OHS during the 10-year period of observation, 230 had severe (>80%) carotid stenosis. In the old cohort (before 2007), carotid stenosis was associated with perioperative stroke in 2.5% of cases. None of the 113 patients having cardiac procedures after 2007 received combined carotid artery surgery; only 1 of these patients harboring severe carotid stenosis had an ischemic stroke (.9%) during the perioperative period. The difference in stroke incidence between the 2 cohorts was statistically significant (P = .002). CONCLUSION: The incidence of stroke in patients with severe carotid artery stenosis undergoing OHS was lower after combined surgery was discontinued. Combined carotid and OHS itself seems to be an important risk factor for stroke.

Focal Cerebral Vasculitis due to SARS-CoV-2 Infection With a Robust Response to Intravenous Steroids.

INTRODUCTION: Severe and less common neurological manifestations of SARS-CoV-2 infection include acute ischemic stroke, intracerebral hemorrhage, central venous sinus thrombosis, and vasculitis. In this report, we present a case of a 42-year-old man with acute ischemic stroke due to SARS-CoV-2 infection-associated central nervous system vasculitis that improved with steroid therapy. CASE REPORT: A 42-year-old man with SARS-CoV-2 infection presented with non-fluent aphasia and right-sided hemiparesis. Computed tomography angiography revealed an occlusion of the proximal left middle cerebral artery (MCA), with acute infarcts in the left posterior parietal, lentiform nucleus, and cortical frontal cortex on magnetic resonance imaging (MRI). Patient developed pulmonary emboli and was discharged on apixaban and atorvastatin. Four weeks later, the patient presented with recurring symptoms and was found to have worsening left MCA stenosis. MRI and MR angiography revealed a penumbra within the left MCA territory and pruning of the distal branches with severe stenosis. Laboratory workup for autoimmune causes of vasculitis was unrevealing. High-dose intravenous steroid treatment was initiated. Subsequent MRI and MR angiography revealed improved flow in the left cerebral vasculature and no novel ischemic infarcts. CONCLUSION: Central nervous system vasculitis is a rare manifestation of SARS-CoV-2 infection. This case suggests that high-dose intravenous steroids may have a therapeutic role in this patient population. Steroid use, in combination with vasopressor support to augment cerebral blood flow, may prevent further stroke burden.

Mild Encephalopathy With Partial Reversible Splenium Lesion Associated With SARS-CoV-2 Infection.

Viral-associated encephalitis/encephalopathy includes a wide spectrum of syndromes reported often in children. A rare form presents with mild encephalitis/encephalopathy and reversible splenial lesion(s). This report describes a case of this rare presentation associated with severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection in a 68-year-old woman. The patient presented to the hospital with altered mental status. Examination revealed mild encephalopathy with disorientation to date and time. Initial laboratory workup was significant for mild hypernatremia and acute kidney injury, and a polymerase chain reaction (PCR) test for SARS-CoV-2 was positive. MRI of the brain revealed an area of hyperintensity and water restriction in the corpus callosum. The patient was treated with tocilizumab, dexamethasone, and remdesivir. MRI of the brain five weeks later revealed partial resolution of the hyperintensity, and complete resolution of the restricted diffusion previously seen in the corpus callosum, which confirmed the diagnosis of mild encephalitis/encephalopathy with a reversible splenial lesion. We highlight the importance of recognizing this phenomenon in association with SARS-CoV-2 infection.

A Treatable Cause of Pulsatile Tinnitus: A Case of Sigmoid Sinus Dehiscence.

Tinnitus is a symptom of an underlying condition that can be neurological, ontological, or infectious in origin. This case report describes a patient with pulsatile tinnitus caused by sigmoid sinus dehiscence, which was successfully treated by sigmoid sinus dehiscence repair. We recommend computed tomography angiography/magnetic resonance angiography or digital subtraction angiography to rule out vascular malformation, such as arteriovenous fistula, prior to surgical intervention. In addition, we recommend imaging of the brain and formal evaluation by an ophthalmologist and lumbar puncture prior to surgical intervention to rule out idiopathic intracranial hypertension if suspected.

Guillain-Barré syndrome after H1N1 vaccination in the United States: a report using the CDC/FDA Vaccine Adverse Event Reporting System (2009).

BACKGROUND: Although the Guillain-Barré syndrome (GBS) can be associated with the seasonal influenza vaccine, there is no definite evidence that GBS is associated with H1N1 influenza vaccination. The objective of this report is to study the occurrence and characteristics of GBS after H1N1 vaccine administration in the United States in 2009. METHODS: Data were acquired from the Vaccine Adverse Event Reporting System and supplemented by additional information obtained from the Center for Biologics Evaluation and Research, under the Federal Freedom of Information Act. RESULTS: A total of 62 individuals (mean age 46.51 ± 22.41 years), 33 of whom were men, developed GBS associated with the H1N1 influenza vaccination in 2009. Sixty GBS cases were reported within 6 weeks after vaccination, with 31 cases (50.0%) reported in the first 2 weeks. The estimated rate of occurrence of GBS was 6.2 cases per 10 million vaccinations, which is comparable to the rate of GBS in the general population. CONCLUSION: The higher rate of GBS reports in the first 6 weeks after H1N1 vaccination suggests that some GBS cases may be triggered by H1N1 vaccination. This warrants early recognition, treatment, and active surveillance in the postvaccination setting.

Trends in outcome and hospitalization charges of adult patients admitted with botulism in the United States.

BACKGROUND: To assess the impact of new therapeutic strategies on outcomes and hospitalization charges among adult patients with botulism in the United States. METHODS: We determined in-hospital outcomes and charges for patients with botulism hospitalized in 1993-1994 and compared them with those observed among patients hospitalized in 2006-2007. Mortality, length of stay, and hospitalization charges were calculated. Age, sex, race, ethnicity, and discharge status were also reported. RESULTS: There were 66 and 132 admissions of adult patients with botulism in 1993-1994 and 2006-2007, respectively. Men predominance was observed in 2006-2007 compared to women predominance during the 1993-1994 time period. There was no significant difference in the average length of stay and in-hospital mortality rate between the two groups studied. However, in the 2006-2007 group, there was a significant increase in the mean hospitalization charges (USD 126,092 ± 120,535 vs. USD 83,623 ± 82,084; p = 0.0107) and in the proportion of patients requiring mechanical ventilation when compared to 1993-1994 (34 vs. 13.6%; p < 0.0001). CONCLUSION: Botulism continues to be an infrequent cause of hospitalization, with a significant increase in the average hospitalization charges in 2006-2007 when compared to 1993-1994, despite a nonsignificant change in the mortality rate and average length of hospitalization.

Stiff-person syndrome: an atypical presentation and a review of the literature.

Introduction: Stiff-person syndrome (SPS) is a rare autoimmune neurological disorder associated with muscle rigidity and spasms. A number of antibodies have been associated with disorder, including anti-glutamic acid decarboxylase and anti-amphiphysin.Case report; In this report, we present a rare case of a 79-year-old woman who presented with bilateral lower extremity weakness who was ultimately diagnosed with stiff-limb syndrome, a rare variant of SPS. Extensive laboratory and CSF studies were unrevealing. Electromyography showed significant peroneal motor neuropathy and complex repetitive discharges in the left tibialis anterior muscle. Antibodies to glutamic acid decarboxylase were significantly elevated at 124 units/mL. She was subsequently started on oral diazepam with significant improvement in her symptoms.Conclusion: The presentation of SPS can vary based on epidemiologic factors, clinical symptoms, and associated disorders. These forms can have overlapping features which may make the categorization of patients into one of these forms challenging.

Late-Onset Encephalopathy Associated With SARS-CoV-2 Infection.

Several typical and atypical neurological manifestations of viral pandemics have been reported. Neurological manifestations of the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) have recently been reported. In this case report, we present a patient with encephalopathy as a late neurologic manifestation of SARS-CoV-2 infection. The patient initially tested positive for the novel coronavirus after presenting with fever, cough, and altered mental status. The symptoms resolved within 5 - 7 days and the patient was discharged home. He subsequently developed worsening encephalopathy in the absence of respiratory symptoms, required hospitalization, and tested positive for SARS-CoV-2. Complete workup was unrevealing otherwise. We advise clinicians to be aware of late neurological manifestations of coronavirus disease 2019 (COVID-19) including encephalopathy.

Pituitary apoplexy mimicking meningoencephalitis: case report and scoping study.

Background: Pituitary apoplexy (PA) is a rare but potentially life-threatening condition that may require urgent surgical intervention.Case Presentation: We report a case of a patient who was initially diagnosed with meningoencephalitis (ME) based on clinical presentation and cerebrospinal fluid (CSF) analysis, but was eventually diagnosed with PA. We present a summary of other cases reported in the literature of PA mimicking ME and analyze their clinical features and CSF findings.Results: Among all 22 PA cases reviewed, headache was the most commonly reported symptom. Hypopituitarism was seen in 94.4% of the cases; of these, panhypopituitarism was noted in 38.9%. The sensitivity of magnetic resonance image (MRI) for detecting PA was 94.7%, much higher than that of computed tomography (CT), which was only 31.6%. Neutrophil predominant pleocytosis was present in all cases with a neutrophil percentage ranging from 73% to 98%. CSF leukocyte count was less than 1000/ul in 86% of the cases. CSF erythrocytosis was seen in 92.9% of the cases with a count ranging from 15 to 2030/ul. Elevated CSF protein was present in all cases with a range of 69.8 to 239 mg/dl. CSF glucose level varied with a range between 12 and 136 mg/dl; the level was greater than 40 mg/dl in 73% of the cases.Conclusion: PA tends to be misdiagnosed as ME due to the similarities of semiology and CSF findings. PA should be considered in refractory acute headache cases, especially those with visual and endocrine abnormalities. Early recognition and treatment may lead to significant reduction in morbidity and mortality.Abbreviations: ACTH: adrenocorticotropic hormone; CSF: cerebrospinal fluid; CT: computed tomography; GRE: gradient echo; HRT: hormone replacement therapy;HSV: Herpes Simplex Virus; IV: intravenous; ME: meningoencephalitis; MRI: magnetic resonance image; PA: pituitary apoplexy; RBC: red blood cell; WBC: white blood cell.

Facial Diplegia as a Rare Late Neurologic Manifestation of SARS-CoV-2 Infection.

Multiple recent publications have reported numerous neurologic complications of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection. Among these is Guillain-Barre syndrome and its variants, including facial diplegia. In this case we present a patient with facial diplegia following a confirmed SARS-CoV-2 infection. The patient initially presented with respiratory symptoms and subsequently developed bilateral facial weakness approximately 3 weeks later prompting an emergency department (ED) visit. Extensive laboratory and imaging workup was negative for other etiologies. Cerebrospinal fluid (CSF) analysis was notable only for mild elevation in white blood cells and protein. Patients with acute neurologic symptoms should be evaluated carefully regarding recent infections or possible exposures to help identify and minimize late complications of this novel virus.

Central Pain Mimicking Trigeminal Neuralgia as a Result of Lateral Medullary Ischemic Stroke.

BACKGROUND: Central pain mimicking trigeminal neuralgia (TN) as a result of lateral medullary infarction or Wallenberg syndrome has been rarely reported. CASE REPORT: We discuss a patient who presented with a lateral medullary infarct and shortly after developed facial pain mimicking TN. We also elaborate on the anatomical pathway of the trigeminal nerve explaining facial pain as a result of a lateral medullary lesion. DISCUSSION: Clinicians should be aware of this typical complication of lateral medullary infarct in order to attain proper management and work-up.

Hypertrophic cervical spine pachymeningitis due to sarcoidosis: a case report.

Hypertrophic pachymeningitis (HP) is a chronic, progressive diffuse inflammatory condition that leads to thickening of the dura mater and can be idiopathic or associated with sarcoidosis among other disorders. In this case report, we present a rare case of cervical spine HP in a 29-year-old woman in the post-partum period, who had a history of pituitary adenoma and juvenile rheumatoid arthritis. Magnetic resonance imaging (MRI) of the spine revealed a soft tissue mass and moderate cord compression. The patient underwent C3-C7 laminectomy. Pathological analysis of the cervical epidural mass demonstrated a reactive inflammatory cell process. Recurrence of symptoms and worsening of pachymeningitis on imaging studies warranted further work-up which revealed mediastinal/hilar lymphadenopathy. Transbronchial biopsy revealed non-caseating granulomatous disease consistent with sarcoidosis. The patient was started on oral steroids and eventually methotrexate with significant clinical and radiographic improvement. Follow-up imaging studies showed minimal dural thickening in the thoracic spine and eventually complete resolution. HP should be considered in a patient with spinal cord compression, myelopathy, and radicular pain of unclear etiology, and sarcoidosis should be considered in idiopathic cases.

Intracranial hemorrhage in the setting of posterior reversible encephalopathy syndrome: two case reports and a review.

Posterior reversible encephalopathy syndrome (PRES) is clinically characterized by seizures, changes in vision, altered mental status, and headache, with associated radiologic changes on brain imaging. Intraparenchymal hemorrhage is a rare complication of PRES and an atypical initial presentation of this condition. In this report, we discuss two patients who presented with multifocal cerebral hemorrhages that were later attributed to PRES. We further expand on the pathophysiology, management, and prognosis on patients with hemorrhagic PRES. Increased awareness of this complication of PRES is important in guiding prognostication and treatment.

Central Hypoventilation: A Rare Complication of Wallenberg Syndrome.

Central alveolar hypoventilation disorders denote conditions resulting from underlying neurologic disorders affecting the sensors, the central controller, or the integration of those signals leading to insufficient ventilation and reduction in partial pressures of oxygen. We report a patient who presented with a left lateral medullary ischemic stroke after aneurysm repair who subsequently developed a rare complication of CAH. Increased awareness of this condition's clinical manifestations is crucial to make an accurate diagnosis and understand its complications and prognosis.

Spinal racemose cysticercosis: case report and review.

Cysticercosis is a common helminthic infection worldwide, endemic in Central and South America, sub-Saharan Africa, and Southeast Asia. Neurocysticercosis typically involves brain parenchyma, subarachnoid space, and the ventricular system. Although the spinal cord is frequently involved in patients with parenchymal neurocysticercosis, isolated spinal involvement is rare, occurring in only 1-3% of patients. We report a case of racemose spinal neurocysticercosis with brain parenchyma involvement in a 49-year-old Mexican man, who presented with unsteady gait and bilateral arm numbness and weakness. Magnetic resonance imaging revealed multiseptated cystic lesions in the upper cervical spinal canal and nonenhancing intradural extramedullary cystic lesions in the thoracic spine. The patient underwent sub-occipital craniectomy with decompression, followed by a course of steroids and albendazole. Pathology confirmed the diagnosis of neurocysticercosis, and the patient's symptoms resolved after treatment. We include discussion on the symptoms, diagnosis, and treatment of neurocysticercosis with a focus on the spinal form.

Carotid Endarterectomy in a Patient with Severe Internal Carotid Artery Stenosis with Persistent Trigeminal Artery and Ischemia of the Anterior and Posterior Circulation.

Occurrence of cerebral ischemia in the posterior circulation as a result of severe internal carotid artery disease and persistent trigeminal artery is rare. An 81-year-old man with medical history of hypertension and ischemic stroke presented with dizziness, nausea, and mild dysarthria. Magnetic resonance imaging of the brain revealed acute infarcts in the left internal carotid artery territory. CT angiogram revealed a persistent trigeminal artery (PTA) and severe atherosclerosis. The patient developed new neurological symptoms and repeat imaging revealed new acute infarcts in the PTA distribution. After undergoing a left carotid endarterectomy with no complications, the patient was discharged to a skilled nursing facility with no recurrence of ischemic stroke. This case adds a rare complication of an infrequent vascular anomaly to the limited body of the literature.