Responsive Neurostimulation for People With Drug-Resistant Epilepsy and Autism Spectrum Disorder.

Purpose: 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.

Reflex Anoxic Seizures Induced by Needle Stick and Successfully Treated With Intranasal Midazolam.

Reflex anoxic seizures are a type of convulsive syncope seen more commonly in children. In rare cases, they may develop into true epileptic seizures. There is no current consensus on treatment.

Rituximab for Autoimmune Encephalitis with Epilepsy.

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 -amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor (AMPAR), gamma aminobutyric acid receptor (GABAR), and -methyl-D-aspartate receptor (NMDAR).

Anti-NMDAR Encephalitis: A Multidisciplinary Approach to Identification of the Disorder and Management of Psychiatric Symptoms.

Background: The novelty of anti-NMDA receptor encephalitis, for which somatic treatments have only recently been developed, has led to a lack of information on assessment and treatment of its variable behavioral manifestations.

Method: In this article, we discuss 4 challenging cases of anti-NMDAR encephalitis, focusing on the importance of a multidisciplinary approach to identification and management of the disorder and the necessity of close collaboration in the acute hospital setting for management of the behavioral symptoms.

Conclusion: The cases we discuss highlight some of the medication and nonpharmacologic treatment strategies that may facilitate management of psychiatric symptoms, both while the medical workup is ongoing and after the diagnosis has been confirmed.

A Rare Case of Insular Epilepsy: Not To Be Missed in Refractory Epilepsy Patients.

Insular epilepsy often goes under-recognized and misdiagnosed due to the similarity of its features with temporal lobe epilepsy and the common exclusion of the insula during intracranial electroencephalography (iEEG). Here, we present a case of medically refractory epilepsy in a 43-year-old male with a 12-year history of tonic-clonic seizures. Insular epilepsy cases are often considered for diagnosis in the setting of abnormal insular pathology, such as a low-grade central nervous system (CNS) lesion.

New Onset British Accent, Acute Behavioral Changes, and Seizures: A Unique Presentation of NMDAR Encephalitis.

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.

Rare Case of Anti-LGI1 Limbic Encephalitis with New Onset Epilepsy: A Case Report.

Anti-leucine-rich glioma-inactivated 1 (LGI1) limbic encephalitis (LE) has been classified as an autoimmune LE with a subacute course. Many patients with anti-LGI1 LE have normal or minimal cerebrospinal fluid (CSF) findings. Cerebrospinal fluid 14-3-3 protein or neuron specific enolase is usually seen in Creutzfeldt-Jakob disease (CJD) with high sensitivities, but can also be positive in other paraneoplastic and autoimmune encephalitides, which can make diagnosis challenging.

Vagus Nerve Stimulation (VNS) in Super Refractory New Onset Refractory Status Epilepticus (NORSE).

The treatment protocol of status epilepticus has many associated toxicities so there is interest in alternate nonmedicinal therapies for managing New Onset Refractory Status Epilepticus (NORSE) patients. Vagus nerve stimulation (VNS) is an FDA-approved therapy for refractory epilepsy that has been shown to decrease the frequency and severity of seizures. We present the case of a patient with new-onset refractory status epilepticus (NORSE) whose seizures were successfully treated with vagus nerve stimulation.

Cardiac Sarcoidosis as an Uncommon Etiology for Posterior Circulation Stroke Presenting with Alexia without Agraphia.

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.

Recurrent Occipital Seizures with Transient MRI Changes.

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.

Uncommon Etiology for Seizure: Cerebral Hyperperfusion Syndrome.

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.

Unique presentation of akinetic mutism and coexisting thyroid storm relating to stroke.

Akinetic mutism is described in various clinical presentations but typically is defined as a state wherein the patient appears awake but does not move or speak. It can be divided into two different subtypes; the most common subtypes depend on the lesion location, mesencephalic-diencephalic region, also called apathetic akinetic mutism (somnolent mutism), and those involving the anterior cingulate gyrus and adjacent frontal lobes called hyperpathic akinetic mutism. The pathway of akinetic mutism is believed to originate from circuits that link the frontal and subcortical structures.

A rare sequela of acute disseminated encephalomyelitis.

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.

A Rare Case of Immune Reconstitution Inflammatory Syndrome Development in an Immunocompromised Patient with Progressive Multifocal Leukoencephalopathy and Multicentric Castleman's Disease.

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.

Posterior Reversible Encephalopathy Syndrome in a Patient with Newly Diagnosed HIV Infection and End Stage Renal Disease.

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.

Myasthenia gravis presenting like guillain-barré syndrome.

Myasthenia gravis (MG) is an autoimmune disorder characterized by weakness in specific muscle groups, especially the ocular and bulbar muscles. Guillain-Barré 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.

Myasthenia gravis: a review.

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.

Acute ischemic stroke secondary to iron deficiency anemia: a case report.

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.

A rare case of occipital stroke as a consequence of nonbacterial thrombotic endocarditis in ovarian clear cell carcinoma: a case report.

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).

A rare case of subependymoma with an atypical presentation: a case report.

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.

Collapsing glomerulopathy in sickle cell disease: a case report.

Introduction: Sickle cell disease has been associated with many renal structural and functional abnormalities. Collapsing glomerulopathy or the collapsing variant of focal segmental glomerulosclerosis is a rare clinicopathologic entity in patients with sickle cell disease that requires timely diagnosis and aggressive management.

Case Presentation: In this case report we describe a 21-year-old African-American woman with a medical history of significant sickle cell disease and asthma.

Lemierre's syndrome resulting from streptococcal induced otitis media and mastoiditis: a case report.

Introduction: Lemierre's syndrome is an extremely rare and almost universally fatal disease characterized as thrombophlebitis of the internal jugular venous system with subsequent metastatic infection. Fusobacterium necrophorum is the most common organism implicated in causation of Lemierre's syndrome. Group A Streptococcus has mainly been observed as a polymicrobial organism in the syndrome.

Rare association of thymoma, myasthenia gravis and sarcoidosis : a case report.

Introduction: The association of thymoma with myasthenia gravis (MG) is well known. Thymoma with sarcoidosis however, is very rare. We presented an interesting case with coexisting thymoma, MG and sarcoidosis.