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.

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.

The role of carotid ultrasound in patients with non-lateralizing neurological complaints.

OBJECTIVES: In the United States, approximately 18-25% of carotid duplex ultrasound (CUS) studies are ordered to assess patients with non-lateralizing neurological complaints such as syncope, blurry vision, lightheadedness, headache, and altered mental status. The purpose of this study is to evaluate the benefit of CUS in the evaluation of patients presenting with non-lateralizing signs or symptoms. MATERIALS AND METHODS: We conducted a retrospective analysis to assess the degree and laterality of carotid stenosis among patients with non-lateralizing neurological complaints who underwent CUS interpreted by certified vascular neurologists over a period of 3 years. The primary endpoint was to identify the prevalence of moderate-to-severe carotid artery stenosis among 280 patients who met inclusion criteria. RESULTS: A total of 17.7% of CUS studies were ordered for non-lateralizing symptoms. Two hundred and sixty-one patients (93.21%) had either normal imaging or mild carotid stenosis of <50%. Nineteen patients (6.79%) were found to have stenosis of ≥50%. In this subgroup, age and known preexisting carotid artery atherosclerotic disease were the only variables found to have a statistically significant association with the level of stenosis found on CUS. Two patients with asymptomatic stenosis of >70% underwent a revascularization procedure. CONCLUSION: At least 17.7% of CUS studies were completed for non-lateralizing symptoms. The study is of low-yield with the prevalence of moderate-to-severe stenosis being comparable to that in the general asymptomatic population. We conclude that there is minimal clinical value in the use of CUS to investigate non-lateralizing neurological complaints, resulting in unnecessary healthcare costs.

Carotid Web as an Embolic Source of Acute Ischemic Stroke.

INTRODUCTION: Carotid webs are shelf-like projections of the carotid bulb lumen associated with ipsilateral ischemic stroke in young patients. Given its rarity, a limited number of studies have evaluated the optimal management of symptomatic carotid webs to prevent further ischemic stroke. CASE REPORT: A 40-year-old Caucasian man presented with a sudden onset of left-sided facial droop, hemiparesis, and dysarthria. Computed tomography angiography revealed occlusion of the distal M1 and proximal M2 segments of the right middle cerebral artery and a small intimal flap at the right internal carotid artery origin. Intravenous alteplase was administered without clinical improvement. The patient underwent successful mechanical thrombectomy with complete revascularization of the middle cerebral artery and no residual neurologic deficits. Magnetic resonance angiography confirmed a curvilinear, shelf-like projection from the right carotid bulb posterior wall, consistent with a carotid web. The patient underwent carotid endarterectomy with no complications and was discharged home with no residual deficits or recurrence of stroke. CONCLUSION: No randomized, controlled prospective studies have compared the efficacy of endarterectomy to carotid artery stenting in patients with symptomatic carotid web. This case emphasizes the importance of considering carotid web as an embolic source of stroke in the young and provides support for revascularization as a safe and effective mean of secondary stroke prevention.

Adult polyglucosan body disease: an acute presentation leading to unmasking of this rare disorder.

INTRODUCTION: Adult polyglucosan body disease (APBD) is an autosomal recessive leukodystrophy caused by abnormal intracellular accumulation of glycogen byproducts. This disorder is linked to a deficiency in glycogen branching enzyme-1 (GBE-1). Neurologic manifestations include upper and lower motor neuron signs, dementia, and peripheral neuropathy. APBD is typically a progressive disease. In this report, we discuss a novel case of APBD in a patient who had a sudden onset of spastic quadriparesis preceded by gradual difficulty with gait. Genetic and postmortem analysis confirmed the diagnosis of APBD. CASE REPORT: A 65-year-old man was evaluated for a new-onset of spastic quadriparesis, right-gaze preference, and left-sided beat nystagmus. Magnetic resonance imaging (MRI) of the brain revealed areas of white matter hyperintensities most prominent in the brainstem and periventricular regions. MRI of the cervical spine showed marked cord atrophy. Laboratory workup and cerebrospinal fluid analysis were unremarkable. Genetic testing supported the diagnosis of APBD due to GBE-1 deficiency. Postmortem analysis showed multiple white matter abnormalities suggestive of a leukodystrophy syndrome, and histopathologic testing revealed abnormal accumulation of polyglucosan bodies in samples from the patient's central nervous system supporting the diagnosis of APBD. CONCLUSION: APBD is a rare disorder that can affect the nervous system. The diagnosis can be confirmed with a combination of genetic testing and pathologic analysis of affected brain tissue.

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.

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.

Froin Syndrome, a Rare Complication of Multiple Myeloma.

INTRODUCTION: Froin syndrome is a rare condition that is defined as marked coagulability, elevated protein level, and xanthochromia of the cerebrospinal fluid (CSF). Froin syndrome more commonly occurs because of obstruction of CSF flow due to underlying inflammatory or neoplastic conditions. CASE REPORT: We present a case of a 38-year-old man who was found to have Froin syndrome a year after he was diagnosed with multiple myeloma (MM). CSF analysis, laboratory work-up, and magnetic resonance imaging of the neuroaxis supported the diagnosis of Froin syndrome related to leptomeningeal myelomatosis. To our knowledge, this is the first reported case of FS due to MM. CONCLUSION: Leptomeningeal myelomatosis associated with MM can cause blockage of CSF circulation leading to Froin syndrome. Inflammatory or neoplastic conditions should be considered as an underlying etiology.

Reversible Cerebral Vasoconstriction Syndrome Following Carotid Endarterectomy: A Case Report.

INTRODUCTION: Reversible cerebral vasoconstriction syndrome (RCVS) is a cerebrovascular disorder associated with multifocal intracranial arterial constriction and dilation that occurs spontaneously or as a result of a stimulant. The authors present a case of RCVS in a patient who presented with a new-onset thunderclap headache a day after carotid endarterectomy (CEA). RCVS has been rarely reported after CEA. CASE REPORT: A 65-year-old woman was evaluated for a new-onset thunderclap headache a day after left-sided CEA. Computed tomography (CT) of the head revealed left frontal and parietal subarachnoid hemorrhage (SAH). CT angiography did not show any saccular aneurysms or vessel stenosis. The initial impression was SAH related to reperfusion injury after carotid revascularization. Seven days postoperatively, the patient returned to the hospital with a persistent headache. CT revealed SAH in the vertex of the frontal region bilaterally. Magnetic resonance angiogram (MRA) of the head revealed multifocal stenosis of the intracranial circulation bilaterally. A follow-up MRA 9 weeks postoperatively showed interval improvement of the caliber of the circle of Willis branches and significant improvement of the multifocal stenosis. The patient was diagnosed with RCVS as a result of CEA. CONCLUSION: The authors advise clinicians to consider RCVS as a cause of thunderclap headache or recurrence of a severe headache shortly after CEA-particularly with the presence of a nonaneurysmal convexity SAH.

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.

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.

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.

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.

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.

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.