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Objective: To identify the incidence of seizures within 48 hours of COVID-19 vaccination in PWE Methods: This is a retrospective cross-sectional observational study performed at two Northern Virginia neurology clinics. PWE that had been vaccinated against COVID-19 were surveyed to report occurrence of a seizure within 48 hours of COVID-19 vaccination and the presence of additional triggers surrounding the seizure. Results: Of the 189 patients included in the analysis, 13 (7%) reported a seizure within 48 hours of vaccination. Of the 13 participants with reported seizures, 10 identified possible triggers present at the time of their seizure. Additionally, patients with intractable epilepsy were found to have a non-statistically significant (p = 0.16) increased risk of seizure (odds ratio = 2.2) within 48 hours of vaccination. Conclusion: The results show a low incidence of seizure within 48 hours of receiving a COVID-19 vaccination in this cohort of PWE. Those that reported seizures had additional provoking factors present that may have triggered the seizure. For patients with intractable epilepsy it appears they may have some increased risk of breakthrough seizures within 48 hours of vaccination. It is recommended that clinicians counsel their epilepsy patients, especially those with an intractable diagnosis, to mitigate potential seizure triggers prior to vaccine administration. Further research is recommended to observe for long term effects if present and to control for provoking seizure factors/triggers.

Individuals with autism spectrum disorder (ASD) have comorbid epilepsy at much higher rates than the general population, and about 30% will be refractory to medication. Patients with drug-resistant epilepsy (DRE) should be referred for surgical evaluation, yet many with ASD and DRE are not resective surgical candidates. The aim of this study was to examine the response of this population to the responsive neurostimulator (RNS) System.This multicenter study evaluated patients with ASD and DRE who underwent RNS System placement. Patients were included if they had the RNS System placed for 1 year or more. Seizure reduction and behavioral outcomes were reported. Descriptive statistics were used for analysis.Nineteen patients with ASD and DRE had the RNS System placed at 5 centers. Patients were between the ages of 11 and 29 (median 20) years. Fourteen patients were male, whereas five were female. The device was implanted from 1 to 5 years. Sixty-three percent of all patients experienced a50% seizure reduction, with 21% of those patients being classified as super responders (seizure reduction90%). For the super responders, two of the four patients had the device implanted for2 years. The response rate was 70% for those in whom the device was implanted for2 years. Improvements in behaviors as measured by the Clinical Global Impression Scale-Improvement scale were noted in 79%. No complications from the surgery were reported.Based on the authors' experience in this small cohort of patients, the RNS System seems to be a promising surgical option in people with ASD-DRE.

The leading cause of autoimmune encephalitis is N-methyl-D-aspartate receptor (NMDAR) encephalitis. Symptoms can present as prominent behavioral abnormalities prompting inaccurate psychiatric diagnoses. Psychiatric features such as bizarre behavior, agitation, anxiety, delusions, and hallucinations are well noted in the current literature, but a manifestation of foreign accent syndrome has, to our knowledge, never been reported in cases of encephalitis. Once diagnosed, initiation of therapy can result in effective treatment. Here, we present a case of a 32-year-old female with new onset seizures and marked behavioral changes, such as speaking in a foreign accent, who was empirically treated for NMDAR encephalitis due to strong clinical suspicion, showed no improvement with first line therapy with IVIG and IV steroids, and finally had rapid resolution of symptoms with the early initiation of second line therapy of rituximab. In a young female presenting with nonspecific behavioral changes, NMDAR encephalitis should be on the differential and, although CSF antibodies are definitively diagnostic, there should be a low threshold to start empiric therapy and escalate to second line treatment.

Intractable epilepsy remains a significant medical challenge, resulting in recurrent and prolonged intensive care unit (ICU) admissions. Autoimmune encephalitis is emerging as a treatable cause of intractable epilepsy. It is characterized by antibodies against cerebral antigens, such as potassium channels such as leucine-rich, glioma inactivated 1 (LGI1) and contactin-associated protein 2 (CASPR2), calcium channels such as the voltage-gated calcium channel (VGCC), or neurotransmitter receptors such as the α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor (AMPAR), gamma aminobutyric acid receptor (GABAR), and N-methyl-D-aspartate receptor (NMDAR). Diagnosis requires a syndrome consistent with an antibody identified in serum or cerebrospinal fluid (CSF) using methods that minimize risk of false-positives. Although there is no officially approved therapy for these disorders, typical approaches involve chronic high-dose steroids, intravenous immunoglobulin (IVIG), or plasma exchange. Rituximab is effective for antibody-associated disorders such as lupus, myasthenia gravis, and neuromyelitis optica. Here, we present three patients who were admitted with recalcitrant status epilepticus and demonstrated serum antibodies against NMDAR, LGI1, or VGCC using a cell-based assay. All patients demonstrated complete, long-term epilepsy control and improvement in symptoms with rituximab.

Rationale: Epilepsy patients are known to have multiple comorbidities. Comorbid psychiatric diagnosis contributes to the poor outcome, especially undiagnosed psychiatric conditions. The goal of the study is to properly identify specific psychiatric diagnosis in this patient population, providing targeted treatment recommendation. Methods: All patients admitted to Epilepsy Monitoring Unit (EMU) from October 2016 to May 2017 are included in this analysis. Psychiatric evaluation was completed from all ninety-seven patients except one due to family refusal (N = 96). All patients have pre-existing epilepsy diagnosis or suspicion of epilepsy. Psychiatric evaluation includes patient interview, family interview, chart review, and discussion with neurology team. Results: Ninety-seven patients were admitted to the EMU between October 2016-November 2017; 96 of those patients received psychiatric intervention. There were 53 (55%) female participants and 43 (45%) male participants; mean age was 43 years old. Of ninety-six epilepsy and epilepsy suspect patients, 61 (64%) reported history of psychiatric illness; 34 (56%) of these patients were treated by their neurologist or primary care doctor for depression or anxiety. Four patients (4.2%) reported pre-existing Post-Trauma Stress Disorder (PTSD) with history of severe trauma. Four patients (4.2%) had autistic spectrum disorder diagnosed at young age; all related to early-onset epilepsy. Five patients (5.2%) had documented, pre-existing Psychogenic Non-Epileptic Seizure (PNES) or conversion disorder evidenced by negative EEG. A few other psychiatric diagnoses were unrelated to epilepsy. Thirty-five patients (36%) who reported no pre-existing psychiatric diagnosis had never had a psychiatric evaluation. After formal psychiatric screening at EMU, 56 out of 96 (58%) of patients’ psychiatric diagnosis has changed. Ten out of 41 (24%) of the patients with pre-existing diagnosis of depression or anxiety were found to have different types of somatic symptoms. With EEG correlation, 13 (14%) patients were confirmed to have PNES or conversion disorder with mixed symptoms during EMU admission from 5 (5%) diagnosed PNES cases before EMU admission. Nine patients met the criteria of somatic symptom disorder with chronic, non-neurological symptoms. Seven (7%) patients received a new diagnosis of adjustment disorder; four of them were due to uncontrolled epilepsy. Five patients received a new diagnosis of PTSD with severe early life trauma; among them, three patients also received another new diagnosis of chronic somatic symptom disorder. Conclusions: Proper diagnosis of psychiatric comorbidities is the first step in treatment. Inpatient psychiatric evaluation during EMU admission identifies more specific psychiatric diagnoses, leading to more targeted treatment recommendations. We strongly recommend integrated psychiatric evaluation for all EMU admissions. Psychiatric consultation with daily inpatient follow-up during EMU admission identified PNES, conversion disorder with mixed symptoms, other somatic symptom disorders and adjustment disorder related to epilepsy, which led to more targeted treatment recommendations. Unrecognized and untreated conversion patients are just as disabled as patients with epilepsy. The misdiagnosis of PNES leads to inappropriate treatment of presumed epilepsy, with significant risk of iatrogenic injury, morbidity and increased cost to patient and to the health care system.

Peri-ictal magnetic resonance imaging (MRI) findings following seizure activity are a recognized phenomenon that is not well understood (Cole, 2004). Transient changes are not usually expected to be present in postictal MRI studies because of their rarity. Here, we present a unique case of peri-ictal MRI findings located in the occipital lobe, present in a 34-year-old female with recurrent occipital seizures occurring twice in four years. MRI changes completely resolved after both episodes with no residual focal damage. The peri-ictal occipital changes on MRI in this patient are unique because they have been captured on more than one occasion. Peri-ictal MRI findings are a known phenomenon with unknown pathophysiology, although attempts have been made to understand these findings. Though the MRI findings and presentation appear to be stroke-like or PRES-like, seizures should be kept in the differential for better treatment outcomes.

Cerebral hyperperfusion syndrome (CHS) is a rare life-threatening complication of carotid endarterectomy (CEA) and carotid artery stenting (CAS) for carotid artery stenosis. The incidence varies between 0 and 3%, depending on the severity of the stenosis, perioperative hypertension, and contralateral carotid stenosis. This case report reports a 53-year-old female patient presenting with decreased alertness and multiple tonic-clonic seizures, in the background of bilateral CEA. She was found to have bilateral carotid stenosis. Her left CEA was performed three months prior and right CEA was four days prior to her current presentation with seizures. After bilateral CEA, the imaging showed extensive pathologic process involving primarily the subcortical white matter and overlying cortex, more on the right cerebral hemisphere. On follow-up six weeks later, she reported no recurrent seizures and imaging showed decrease in abnormal signal intensity of the grey and white matter. This was indicative of near complete resolution of hyperperfusion damage. CHS is a rare complication due to the loss of autoregulation of the cerebrovascular system and increased blood flow status after bilateral CEA. This case is reported because of a rare and unique presentation of seizures in the background of bilateral CEA.

Sarcoidosis is a systemic disease with cardiac involvement occurring in 20-50% of cases. Cardiogenic stroke caused by cardiac sarcoidosis, especially PCA infarction, is a rare clinical presentation that necessitates timely diagnosis and may warrant treatment prophylaxis against CVA. In this case report, we describe a 54-year-old Caucasian male presenting with left PCA stroke in the setting of cardiac and pulmonary sarcoidosis, and hypertension. His presenting symptoms included right partial hemianopia, difficulty with naming, memory, and recall, and alexia without agraphia. Cardiogenic stroke is an uncommon manifestation of cardiac sarcoidosis, and given the disabling nature of these sequelae, the importance of early diagnosis and prevention with anticoagulation is crucial to prevent morbidity and mortality.

Background The goal of the present study was to determine the prevalence of left ventricular systolic dysfunction (LVSD) and associated clinical correlates in African Americans (AA) diagnosed with ischemic stroke (IS). Methods Retrospective chart analysis was done on all diagnosed AA IS patients between January 2010 and March 2012. Patients with atrial fibrillation were excluded. A total of 147 patients were included in the study. Transthoracic 2-dimensional echocardiography was used to assess left ventricular systolic function, and study groups were categorized as normal, mild, moderate, and severely abnormal, based on the ejection fraction (EF). Available imaging studies were analyzed for data collection. Logistic regression and Pearson chi-square tests were performed. Results Normal EF was present in 114 of 147 patients (78%). Mild abnormality was present in 9 of 147 (6%), moderate in 8 of 147 (5%), and severe in 16 of 147 (11%) patients. In patients with mildly reduced EF, smoking was the most common (RF). In patients with moderately and severely reduced EFs, hypertension was the most common RF. History of smoking was commonly found in systolic dysfunction group compared with normal group ( P = .001). Logistic regression analysis revealed that smoking and advanced age were the significant predictors for LVSD. Large-vessel IS were more common in systolic dysfunction group than normal EF group ( P = .017). Conclusions Prevalence of LVSD in AA with IS was 22% in our study. Smoking was a significant modifiable RF associated with systolic dysfunction. A history of smoking and higher age could predict the occurrence of LVSD. There were more large-vessel IS in patients with LVSD.

Myasthenia gravis (MG) is an autoimmune disorder characterized by weakness in specific muscle groups, especially the ocular and bulbar muscles. Guillain-Barre syndrome (GBS) presents with ascending paralysis and areflexia, often secondary to an infection. Several theories have been proposed regarding the etiology behind GBS, with many studies pointing to a possible autoimmune cause. If this is in fact true, it is also possible that the two diseases may develop concurrently. While this is unusual, several recently published studies highlight such cases of concurrent MG and GBS. This co-occurrence could involve certain common proteins, as the two diseases can present somewhat similarly. This is an unusual case of a patient with no significant past medical history, presenting with generalized weakness and symptoms of new-onset diabetes, who developed bilateral ptosis, distal weakness, and areflexia while in the hospital, raising the possibility of concurrent MG and GBS. Although the diagnosis of MG was confirmed by the positive anticholinesterase antibodies and tensilon test, several features, including sudden onset of ascending paralysis and areflexia, were more common in GBS than MG. It is possible, albeit rare, that these two syndromes could have developed concurrently and that the untreated diabetes mellitus could have contributed to the neurological symptoms. This case is reported because of the rarity of its features, diagnostic and management challenges.

Hypercoagulability occurs in 15% of patients with malignancy and represents a clinical spectrum ranging from abnormal coagulation tests but no clinically evident thromboembolic disease, to arterial and venous thrombosis, migratory thrombophlebitis, nonbacterial thrombotic endocarditis (NBTE) and disseminated intravascular coagulation. The combination of increased procoagulant activity and decreased fibrinolytic activity accelerates the prothrombotic potential of endothelial cells in malignancy. NBTE is a rare manifestation of cancer-induced hypercoagulability and is commonly seen with mucin-producing adenocarcinomas, but rarely seen with ovarian clear cell carcinoma (OCCC). Cerebrovascular embolization ranges from 14–91% in NBTE. We report a rare case of a 62-year-old female presenting with occipital stroke as a consequence of NBTE in OCCC. Association of NBTE in OCCC has only been reported in 2 cases so far, but presentation with stroke has never been reported in the literature.

A rare case of subependymoma in a young patient presenting with sensory dysesthesia is reported. Computed tomography scan and magnetic resonance imaging revealed a posterior fossa mass occluding the fourth ventricle with infiltration to the right side immediately behind the pontine tegmentum and impinging on the right spinothalamic tract. Postoperative tumor histopathology revealed the classical appearance of subependymoma. Subependymoma is a rare, asymptomatic, slow-growing, low-grade glioma of the central nervous system. If symptomatic, the clinical features are commonly secondary to hydrocephalus, but subependymoma presenting with sensory dysesthesia has never been reported in the literature.

Acute disseminated encephalomyelitis is a demyelinating disease, typically occurring in children following a febrile infection or a vaccination. Primary and secondary immune responses contribute to inflammation and subsequent demyelination, but the exact pathogenesis is still unknown. Diagnosis of acute disseminated encephalomyelitis is strongly suggested by temporal relationship between an infection or an immunization and the onset of neurological symptoms. Biopsy is definitive. In general, the disease is self-limiting and the prognostic outcome is favorable with anti-inflammatory and immunosuppressive agents. Locked-in syndrome describes patients who are awake and conscious but have no means of producing limb, speech, or facial movements. Locked-in syndrome is a rare complication of acute disseminated encephalomyelitis. We present a case of incomplete locked-in syndrome occurring in a 34-year-old male secondary to acute disseminated encephalomyelitis. Our case is unique, as acute disseminated encephalomyelitis occurred in a 34-year-old which was poorly responsive to immunosuppression resulting in severe disability.

Immune reconstitution inflammatory syndrome (IRIS) development in HIV with preexistent progressive multifocal leukoencephalopathy (PML) has been extensively studied. PML-IRIS typically manifests clinically as new or worsening neurologic symptoms in conjunction with enlarging CNS lesions and occurs in approximately 10–20 percent of HIV-infected patients with PML who begin HAART. Likewise, Multicentric Castleman’s Disease (MCD), a rare malignant lymphoproliferative disorder, has a strong and well-known association with HIV. Our case provides a rare instance of PML-IRIS in combination with MCD in an HIV-positive individual. The combination of all three diseases has never been reported in the literature. Both MCD and PML were present during initial determination of HIV infection in our patient and their disease courses were altered during the subsequent development of IRIS.

Posterior reversible encephalopathy syndrome (PRES) is a clinicoradiological syndrome in which patients present with an acute or subacute clinical presentation of seizures, visual disturbances, headache, and altered mental status. The pathophysiology of PRES may be explained by endothelial dysfunction that leads to transudation of fluids and protein, resulting in vasogenic cerebral edema. PRES is typically associated with many conditions such as hypertension, uremia, immunosuppressive drugs, and sepsis. This is a case report of a 39-year-old woman with untreated HIV infection and end-stage renal disease (ESRD) who developed PRES with a normal blood pressure and no other known causes of PRES. Untreated HIV is associated with known endothelial dysfunction and we believe that this, in combination with her untreated end-stage renal disease, contributed to her unique presentation of PRES. Although uncommon in HIV-infected patients and challenging to diagnose, prompt recognition of PRES is critical to provide appropriate care and ensure reversibility of the vasogenic edema seen in PRES.

Acquired myasthenia gravis is a relatively uncommon disorder, with prevalence rates that have increased to about 20 per 100,000 in the US population. This autoimmune disease is characterized by muscle weakness that fluctuates, worsening with exertion, and improving with rest. In about two-thirds of the patients, the involvement of extrinsic ocular muscle presents as the initial symptom, usually progressing to involve other bulbar muscles and limb musculature, resulting in generalized myasthenia gravis. Although the cause of the disorder is unknown, the role of circulating antibodies directed against the nicotinic acetylcholine receptor in its pathogenesis is well established. As this disorder is highly treatable, prompt recognition is crucial. During the past decade, significant progress has been made in our understanding of the disease, leading to new treatment modalities and a significant reduction in morbidity and mortality.

A rare case of acute ischemic stroke in a young patient with iron deficiency anemia (IDA) is reported. IDA has been suggested to have an association with stroke, but few cases have proven it thus far. Three physiological mechanisms explaining IDA to ischemic stroke include a hypercoagulable state secondary to IDA, thrombocytosis secondary to IDA, and anemic hypoxia induced by IDA. Our paper shows an example of a hypoxia-induced stroke secondary to IDA in a young woman with menorrhagia. Thrombus formation was ruled out as the Magnetic Resonance Angiogram (MRA) showed no evidence. As all other known causes for stroke were ruled out, the patient's IDA is a reasonable cause for her stroke. Iron deficiency decreases the amount of hemoglobin, which consequently decreases the amount of oxygen in the blood resulting in low-oxygen delivery to the brain. This causes hypoxic conditions in the brain, leading to death of brain tissue. Thus, we suggest a possible relationship between IDA and ischemic stroke in young adults. Considering IDA as one of the risk factors for ischemic stroke and treating with timely transfusions would be an important step one can take to prevent stroke.

Objective: To study the prevalence and neurological manifestations associated with human immunodeficiency virus infection in African Americans in the District of Columbia (DC) metropolitan area. Background Approximately two-thirds of patients with HIV infection will develop CNS involvement during the course of their disease. Three percent of the DC population has HIV infection, which is the highest in the nation. Currently, no literature is available regarding the demographics and neurological manifestations in the African American population with HIV infection. Design/Methods: Medical records of 282 consecutive HIV-positive African American patients with concurrent neurological diagnosis admitted in 2010 to Howard University Hospital, Washington, DC were retrospectively reviewed for demographic variables, neurological manifestations and in relation to CD4 counts. Results: Of the 282 hospitalized patients, 152 were males and 130 were females, with a median age of 47. There were 121 patients CD4 counts > 200, and the most common concurrent neurological diagnosis was neuropsychiatric in nature, which was seen in all patients. These manifestations included depression, bipolar disorder, schizophrenia and psychosis. The other non neuropsychiatric manifestations included epilepsy in 23 patients, ischemic stroke in 19 patients, peripheral neuropathy in 14 patients and opportunistic infections in 4 patients. Eighty-nine patients were presented with CD4 counts Conclusions: The study concluded that neuropsychiatric disease is the most common neurologic manifestation among the African American population with HIV infection, with a relatively higher number among those with CD4 >200. There is a paradigm shift of neurological diseases from opportunistic infections to neuropsychiatric manifestations, which may be related to longer survival secondary advent of effective antiretroviral treatment. Disclosure: Dr. Chapman has nothing to disclose. Dr. Pessu has nothing to disclose. Dr. Chimah has nothing to disclose. Dr. Khan has nothing to disclose. Dr. Kurukumbi has nothing to disclose. Dr. Jayam-Trouth has nothing to disclose.