New-Onset Chorea Post-COVID-19 Infection: A Case Report.

Although the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) primarily involves the cardiovascular and respiratory systems, neurological manifestations, including movement disorders such as myoclonus and cerebellar ataxia, have also been reported. However, the occurrence of post-SARS-CoV-2 chorea is rare. Herein, we describe a 91-year-old female with a past medical history of hypothyroidism who developed chorea after two weeks of contracting a mild coronavirus disease (COVID-19).

Adult-Onset Tics After Being Crushed by an Air Conditioner: A Case Report.

Tics are sudden, repetitive, non-rhythmic movements and/or vocalizations. Generally, tics begin during childhood as a part of Tourette syndrome (TS) and rarely have an onset during adulthood. We describe a 30-year-old male who presented with multiple motor and vocal tics two weeks following a closed head injury with alteration of consciousness as a result of being crushed against the wall by a 4,100-pound air-conditioning unit. He started having motor tics that developed in a rostrocaudal distribution, followed by simple and complex vocal tics. His tics increased in severity over several months following the injury until presentation. He was started on pimozide and received hyperbaric oxygen treatment which improved both motor and vocal tics.

Abdominal Compartment Syndrome with Super-K (Ketamine) for Super-R(efractory) Status Epilepticus: A Case Report.

Refractory status epilepticus is commonly defined as status epilepticus that fails to respond to two or more appropriately dosed intravenous anti-seizure medications including at least one non-benzodiazepine drug. Super-refractory status epilepticus (SRSE) is when status epilepticus continues for ≥24 h despite anesthetic treatment or recurs on an attempted wean of the anesthetic drugs. There is little evidence to guide the management of SRSE. Of late, unconventional therapies have been described in the literature regarding the management of SRSE, with ketamine leading the pack. Studies have noted ketamine's therapeutic efficacy up to 91% in SRSE cessation. Common side effects of ketamine include nausea, vomiting, headache, and hallucinations; but to our knowledge, ketamine has not been implicated in the pathogenesis of abdominal compartment syndrome. We describe a 74-year-old male who developed severe abdominal compartment syndrome in the setting of ketamine infusion for new-onset SRSE to increase awareness about this potential complication.

Primary Central Nervous System Lymphoma Mimicking Longitudinally Extensive Transverse Myelitis.

Primary central nervous system lymphoma (PCNSL) is an uncommon variant of extra-nodal non-Hodgkin's lymphoma. Three regions can be involved in PCNSL: the brain, the spine, or the vitreus and retina. Spinal PCNSL is rare. It can mimic neoplasm, infection, and inflammation. Diagnostic confirmation is by tissue biopsy, and even then, tissue corroboration may be altered by an inflammatory overlay. We report a 59-year-old woman who we saw after she had 4 weeks of ascending tetraparesis plus bowel and bladder incontinence. Upon presentation, the patient was ventilator-dependent and locked-in. She reported normal sensation through eye-blinking. Magnetic resonance imaging (MRI) brain revealed signal intensity in the bilateral corona radiata and restricted diffusion in the right thalamus, whereas, MRI cervical, and thoracic spine showed T2 prolongation in the anterior medulla and upper cervical cord, with enhancement to C2-C3, and long segment hyperintensity from T1-T9 levels, respectively, suggestive of neuromyelitis optica spectrum disorder. Cerebrospinal fluid cytomorphology and flow cytometry were inconclusive for lymphoma/leukemia, but oligoclonal bands were present. Serum aquaporin-4 (AQP-4) antibodies were negative. MR spectroscopy demonstrated NAA reduction, mild lipid lactate peak, and relative reduction of choline on the side of the lesion, favoring demyelination. She received 5-days of intravenous methylprednisolone, followed by 7 sessions of plasma exchange without clinical improvement. Stereotactic biopsy of the right thalamic lesion revealed diffuse large B-cell lymphoma. PCNSL can mimic a demyelinating process early on, as steroid treatment could disrupt B-cell lymphoma cells, thus masking the correct diagnosis.

Meningeal Hemangiopericytoma Presenting as Pure Gerstmann Syndrome: A Double Rarity.

Gerstmann syndrome is a neurobehavioral syndrome characterized by four cardinal symptoms: acalculia, agraphia, finger-toe agnosia, and dysgraphia. The syndrome is caused primarily by lesions at the confluence of parietal, temporal, and occipital lobes, but also can involve the middle frontal lobe of the dominant hemisphere. Documented inciting lesions include stroke, tumor, hemorrhage, arteriovenous malformations, and seizures. A meningeal solitary fibrous tumor (SFT)/hemangiopericytoma (HPC) is a diagnostic challenge due to its resemblance to more common brain tumors like meningioma, with histopathology being the definitive diagnostic test. A 37-year-old male presented to our tertiary center with blurred vision, "not being himself," and "acting funny" for three weeks. On exam, he was found to have a right inferior quadrantanopia, grade II papilledema and demonstrated all four symptoms of Gerstmann syndrome - inability to perform simple calculations (acalculia), or identify his fingers (finger agnosia), could not distinguish his left side from the right (left-right disorientation), nor write out his name (agraphia). Brain imaging showed an extra-axial, highly vascularized 7.6-cm mass compressing the left parietal lobe. He underwent a complete resection of the mass. Postoperatively, he had gradual improvement with complete resolution of agraphia, acalculia, finger agnosia, and left-right disorientation within a week status post-resection. Tumor pathology indicated hemangiopericytoma/solitary fibrous tumor. This case enunciates the enigmatic tetrad of Gerstmann syndrome. Though classically described as a sequela of stroke, the mass effect of the tumor on the parietal lobe may produce the symptoms, which can resolve following resection.

Predictors of Seizure Recurrence after Acute Symptomatic Seizures in Ischemic Stroke Patients.

PURPOSE: Seizure is a well-recognized complication of both remote and acute ischemic strokes. Predictors of seizure recurrence and epilepsy in patients with ischemic stroke who develop acute symptomatic seizures (ASyS) on continuous electroencephalography (cEEG) have not been well studied. METHODS: We present a five-year retrospective study of acute and remote ischemic stroke patients who developed ASyS on cEEG. We then identified risk factors for the development of seizure recurrence. RESULTS: Sixty-five patients with ischemic stroke and ASyS were identified and reviewed. All ASyS were noted to be nonconvulsive seizures. Clinical recurrence of seizures was identified in 19 of these patients (29.2%) at follow-up. Rate of seizure recurrence was higher in remote ischemic stroke patients (84.2%), compared to acute ischemic stroke patients (15.8%, p = 0.0116, OR 0.17, 95% CI 0.049-0.65). Sharp waves/spikes on follow-up EEG significantly correlated with seizure recurrence (p = 0.006, OR 0, 95% CI 0-0.3926). Patients discharged on ≥3 antiepileptic drugs (AEDs) were at a higher risk of having seizure recurrence (p = 0.0015, OR 0.05, 95% CI 0.0089-0.37). CONCLUSION: We identified risk factors of seizure recurrence in patients with ASyS as remote ischemic stroke, requiring multiple AEDs, and the presence of sharp waves on follow-up EEG. This study highlights the usefulness of cEEG in evaluating patients with acute or remote strokes.

Thromboelastography With Platelet Mapping is Not an Effective Measure of Platelet Inhibition in Patients With Spontaneous Intracerebral Hemorrhage on Antiplatelet Therapy.

Thromboelastography with platelet mapping (TEG-PM) is a modality to measure platelet function, especially in patients taking antiplatelet medications. It consists of two components: arachidonic acid (AA), which is sensitive to aspirin, and adenosine diphosphate (ADP), which is sensitive to clopidogrel. In patients with spontaneous intracerebral hemorrhages (sICH), the clinical interpretation of platelet mapping is unclear. The objective of this study was to evaluate TEG-PM in patients with sICH on aspirin and/or clopidogrel who receive platelet transfusions. This study was an IRB-approved, retrospective case-control study over three years at an academic medical center. Adult patients with sICH were included if they had an admission computed tomography head (CTH) and platelet mapping followed by a repeat platelet mapping and CTH post platelet transfusion. A threshold of 50% inhibition was used as the benchmark for both ADP and AA inhibition. Around 248 subjects with sICH were identified, and 107 were excluded for incomplete documentation, leaving 141 for analysis. Of these, nine met our inclusion criteria. No statistical significance was found on the antithrombotic effects of aspirin or clopidogrel on TEG-PM (p=1.00 for both). Sensitivity and specificity of TEG-PM for clopidogrel was 100% and 42.9%, respectively, and 80% and 0%, respectively, for aspirin. Platelet transfusion did not significantly change AA or ADP inhibition (p=1.00). Hemorrhagic expansion on CTH was not associated with a decrease AA or ADP inhibition (p=1.00). TEG-PM is not an effective measure of platelet inhibition in sICH patients who were on antiplatelet medications and is not a reliable measurement following platelet transfusion.

Obstructive Sleep Apnea Presenting as Non-epileptic Spells: A Unique Combination.

Obstructive sleep apnea (OSA), an increasingly prevalent sleep disorder, has been extensively studied in both clinical and scientific settings. In most cases, the diagnosis of sleep apnea is straightforward with patients having symptoms of snoring, choking or gasping for air while asleep and witnessed apneas. However, sleep apnea is known to present in some unusual ways. We present a case of a 61-year-old male, with recently diagnosed obstructive sleep apnea (currently not on continuous positive airway pressure (CPAP)) and a history of seizure-like events since the age of 18 years, who came to the epilepsy monitoring unit (EMU) for spell characterization of his frequent seizure-like episodes. A continuous video electroencephalogram (vEEG) performed in order to determine the semiology of these spells showed that all the spells were triggered by an arousal from sleep with an associated apneic event. He was started on positive airway pressure (PAP) therapy, which resulted in the gradual decline in the number as well as the severity of his seizure-like spells. Based on the observations from vEEG monitoring and the patient's response, we concluded these seizure-like events as non-epileptic spells, triggered by apnea-related arousals in the context of OSA.

Brain and Peripheral Atypical Inflammatory Mediators Potentiate Neuroinflammation and Neurodegeneration.

Neuroinflammatory response is primarily a protective mechanism in the brain. However, excessive and chronic inflammatory responses can lead to deleterious effects involving immune cells, brain cells and signaling molecules. Neuroinflammation induces and accelerates pathogenesis of Parkinson's disease (PD), Alzheimer's disease (AD) and Multiple sclerosis (MS). Neuroinflammatory pathways are indicated as novel therapeutic targets for these diseases. Mast cells are immune cells of hematopoietic origin that regulate inflammation and upon activation release many proinflammatory mediators in systemic and central nervous system (CNS) inflammatory conditions. In addition, inflammatory mediators released from activated glial cells induce neurodegeneration in the brain. Systemic inflammation-derived proinflammatory cytokines/chemokines and other factors cause a breach in the blood brain-barrier (BBB) thereby allowing for the entry of immune/inflammatory cells including mast cell progenitors, mast cells and proinflammatory cytokines and chemokines into the brain. These peripheral-derived factors and intrinsically generated cytokines/chemokines, α-synuclein, corticotropin-releasing hormone (CRH), substance P (SP), beta amyloid 1-42 (Aß1-42) peptide and amyloid precursor proteins can activate glial cells, T-cells and mast cells in the brain can induce additional release of inflammatory and neurotoxic molecules contributing to chronic neuroinflammation and neuronal death. The glia maturation factor (GMF), a proinflammatory protein discovered in our laboratory released from glia, activates mast cells to release inflammatory cytokines and chemokines. Chronic increase in the proinflammatory mediators induces neurotoxic Aß and plaque formation in AD brains and neurodegeneration in PD brains. Glial cells, mast cells and T-cells can reactivate each other in neuroinflammatory conditions in the brain and augment neuroinflammation. Further, inflammatory mediators from the brain can also enter into the peripheral system through defective BBB, recruit immune cells into the brain, and exacerbate neuroinflammation. We suggest that mast cell-associated inflammatory mediators from systemic inflammation and brain could augment neuroinflammation and neurodegeneration in the brain. This review article addresses the role of some atypical inflammatory mediators that are associated with mast cell inflammation and their activation of glial cells to induce neurodegeneration.