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.

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.

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.

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.

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.

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.

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.

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.

Recurrent ischemic stroke in paroxysmal nocturnal hemoglobinuria: paroxysmal nocturnal hemoglobinuria or missed patent foramen ovale?

Paroxysmal nocturnal hemoglobinuria (PNH) is a rare, acquired bone-marrow disorder characterized by hemolytic anemia, hemoglobinuria, and cytopenia. Most patients die from venous thrombotic events. Stroke is a common cause of morbidity and mortality in PNH and it is almost exclusively a result of cerebral venous thrombosis. Case reports of ischemic stroke complicating PNH have implicated a similar propensity for arterial events caused by the disease. We present a case of recurrent cerebral infarctions complicating PNH initially attributed to arterial thrombosis but ultimately determined to be a result of the disease and a concomitant patent foramen ovale identified only after repeated evaluations. This case emphasizes the pitfalls of diagnostic testing and the importance of a persistent search for a venous cause for cerebral embolic events in patients with hematologic diseases not classically known to involve the arterial system.