Retinal Hemorrhage Associated with Human Granulocytic Anaplasmosis.

This case report describes a 51-year-old female with presented to the emergency department with high-grade fever, headache, nausea, vomiting, dizziness, diffuse arthralgias, and new-onset worsened vision that had developed over 3 days.

Genetic considerations in cerebral small vessel diseases.

Cerebral small vessel disease (CSVD) encompasses a broad clinical spectrum united by pathology of the small vessels of the brain. CSVD is commonly identified using brain magnetic resonance imaging with well characterized markers including covert infarcts, white matter hyperintensities, enlarged perivascular spaces, and cerebral microbleeds. The pathophysiology of CSVD is complex involving genetic determinants, environmental factors, and their interactions. While the role of vascular risk factors in CSVD is well known and its management is pivotal in mitigating the clinical effects, recent research has identified novel genetic factors involved in CSVD. Delineating genetic determinants can promote the understanding of the disease and suggest effective treatments and preventive measures of CSVD at the individual level. Here we review CSVD focusing on recent advances in the genetics of CSVD. The knowledge gained has advanced understanding of the pathophysiology of CSVD, offered promising early results that may improve subtype identification of small vessel strokes, has led to additional identification of mendelian forms of small vessel strokes, and is getting closer to influencing clinical care through pharmacogenetic studies.

Intravascular Lymphoma-Associated Stroke: A Systematic Review of Case Studies.

Intravascular lymphoma (IVL) is an aggressive systemic large B-cell lymphoma that is a rare cause of stroke. The clinical characteristics of stroke associated with IVL remain underexplored, contributing to diagnostic complexities and a high mortality rate. This study endeavors to elucidate the salient clinical and investigative features of stroke linked to this condition. A systematic review was performed using the PubMed database from the incident to August 2023 including search categories for IVL and stroke. All studies, excluding review articles, were included in this study. There were 58 cases with a confirmed diagnosis of IVL associated with stroke, with a mean age of 62.9 ± 9.6 years (female 50%). Classical lateralizing stroke symptoms were noted in only 69% of cases. Other clinical syndromes included altered sensorium (31%), rapidly progressive cognitive impairment (23%), seizures (22%), and gait disturbances (19%). Common hematological abnormalities included elevated lactate dehydrogenase (LDH, 97%), erythrocyte sedimentation rate (ESR, 79%), C-reactive protein (CRP, 61%), interleukin-2, microglobulins, and cerebrospinal fluid (CSF) protein. CSF flow cytometry was not diagnostic, and cytology was mostly negative. The dynamic pattern for DWI/T2 lesions was predominant and primarily located in the subcortical regions. Diffuse background slowing (64%) was a major finding in the electroencephalogram. Seventy-one percent of cases died (n=45) mostly due to delayed diagnosis. Only 31% were treated with first-line R-CHOP (rituximab, cyclophosphamide, doxorubicin hydrochloride, vincristine, prednisone) chemotherapy, among whom 25% died. This study suggests that IVL-associated strokes carry a high mortality rate, largely due to challenges in timely diagnosis and therapy. Unlike classical stroke syndrome, key indicators to aid in early diagnosis include a clinical syndrome of multiple non-lateralizing neurological symptoms, dynamic MRI DWI/T2-lesions primarily located in subcortical regions, elevated serum LDH, ESR, CRP, interleukins, microglobulin, CSF protein, and CSF polymerase chain reaction analysis, apart from tissue examination. Larger studies should be performed to establish diagnostic and predictive scores.

The Unique Case of Lumbar Dural Metastasis From Cervical Carcinoma on Pembrolizumab.

Lumbar metastasis is a rare manifestation of cervical carcinoma which may present as lower extremities symptoms. Immune-checkpoint inhibitors like pembrolizumab, used for the treatment of various metastatic carcinomas, have been linked to Guillain-Barré Syndrome (GBS), which is characterized by ascending weakness and areflexia. A 52-year-old female with metastatic cervical cancer on pembrolizumab for two months presents with lower back pain, progressive bilateral lower extremities ascending weakness, and areflexia of affected limbs. Cerebrospinal fluid analysis showed albuminocytologic disassociation. Spine magnetic resonance imaging showed L4-L5 fracture associated with metastasis and contrast-enhancing adjacent dura and nerve roots. Despite the clinical presentation being consistent with lumbar metastasis, the possibility of masked pembrolizumab-induced GBS presented a therapeutic challenge. Plasmapheresis conferred no clinical improvement and later nerve conduction study was unrevealing for GBS. Subsequently, laminectomy of lumbar vertebrae improved the symptoms. The unique clinical scenario of lumbar dural metastasis from cervical cancer on pembrolizumab, a condition with a similar clinical presentation, is explored in this case.

Safety and efficacy of endovascular thrombectomy in patients with severe cerebral venous thrombosis: A meta-analysis.

Background: Cerebral venous thrombosis (CVT) is a rare thrombotic condition which is traditionally treated with anti-coagulation therapy. Subsets of patients with severe CVT have been treated with endovascular thrombectomy (EVT). Despite the high estimated mortality associated with severe CVT, there has been only one randomized control trial done regarding safety and efficacy of EVT in severe CVT compared to standard medical management. Evidence in this area is lacking. Objective: The aim of this systematic review is to analyze all existing literature and generate robust information regarding the role of EVT in the management of patients with severe CVT. Methods: This systematic review and meta-analysis followed PRISMA guideline. PubMed, Embase, Google Scholar, and CNKI were searched for eligible studies from 2007 to 2021. Safety and efficacy of EVT were evaluated by meta-analyzing recanalization status, the good functional outcome at follow-up, recurrent CVT, new hematoma. A pooled proportion with a 95% confidence interval was derived from a meta-analysis of various outcomes (CI). Results: A total of 33 studies comprising 610 patients treated with EVT were included for analysis which comprised one randomized control trial, one prospective study and 31 retrospective studies. Based on pooled data, 85% of patients had good functional outcome, 62% had complete recanalization, 5% had all-cause mortality, and 3% had catheter related complications. The efficacy outcomes in this analysis had a significant heterogeneity and a subgroup analysis was also done to explain these findings. The minimum time of follow up was 3 months and varied EVT techniques were used across the studies. Conclusion: This meta-analysis suggests EVT may be safe and efficacious in treating patients with severe CVT. Registration: Our protocol was registered with PROSPERO: International prospective register of systematic reviews with the registration number CRD42021254760.

Low-dose alteplase for the management of acute ischemic stroke in South Asians: A systematic review on cost, efficacy and safety.

INTRODUCTION: South Asia is responsible for more than 40% of the stroke burden and stroke mortality in the developing world. South Asia, which is home to one-fourth of the world's population, is the most densely populated and one of the poorest regions. The majority of patients in this region are unable to afford intravenous thrombolysis (IVT) for acute ischemic stroke (AIS). If low-dose alteplase proves effective and safe in South Asians, it may be a more cost-effective treatment option. METHODS: The study was conducted according to the PRISMA (Preferred Reporting Items for Systematic Reviews and meta-Analyses) guideline. Researchers searched PubMed, EMBASE, and Google Scholar for English literature from 2005 to 2021. END, ENI, good functional outcome, SICH, and all-cause mortality were used to assess efficacy and safety. RESULTS: In the low-dose alteplase treated patients, different studies reported 32 to 57% ENI 24 h after IVT, and 7% to 9.7% END. At 3 months follow-up, good functional outcome was achieved by 48%-76.92% of low-dose alteplase treated patients. SICH rates ranged from 0% to 16.6% across studies. Asymptomatic ICH occurred in 5-14% of patients. The mortality rate in all included studies varied from none to 25%. CONCLUSION: Our systematic review demonstrates that the use of low-dose alteplase for AIS in the South Asians offer comparable efficacy and reduced risk of SICH at a significantly lower cost than standard alteplase dose. Future well-randomized clinical trials are necessary to validate the findings of our study.

C-reactive protein levels in patients with amyotrophic lateral sclerosis: A systematic review.

INTRODUCTION: Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease affecting cortical and spinal motor neurons. There is a lack of optimal biomarkers to diagnose and prognosticate the ALS patients. C-reactive protein (CRP), an inflammatory marker, has shown promising results in ALS patients. MATERIALS AND METHODS: PubMed, Embase, and Google Scholar databases were searched from 2000 to June 1, 2021 for suitable studies showing the relationship between CRP and ALS. The concentration of CRP levels was assessed between ALS patients and controls. Further, end outcomes like ALS functional rating scale (ALSFRS-R), survival status, and mortality risks were assessed in relation to CRP levels. RESULTS: Eleven studies including five case-control, five cohorts, and one randomized control study were assessed. There were 2785 ALS patients and 3446 healthy controls. A significant increment in CRP levels among ALS patients in comparison with healthy controls were seen in most of the studies. ALSFRS-R and disease progression were found to be significantly correlated with CRP levels. Overall accuracy of CRP in CSF was 62% described in a single study. CONCLUSION: Although CRP has shown promise as a prognostic biomarker, extensive cohort studies are required to assess its prognostic value and accuracy in diagnosing ALS taking into account the confounding factors.

Ibrutinib in Refractory or Relapsing Primary Central Nervous System Lymphoma: A Systematic Review.

Primary Central Nervous System Lymphoma (PCNSL) is a rare variant of Non-Hodgkin Lymphoma (NHL) representing 1-2% of all NHL cases. PCNSL is defined as a lymphoma that occurs in the brain, spinal cord, leptomeninges, or eyes. Efforts to treat PCNSL by traditional chemotherapy and radiotherapy have generally been unsuccessful as a significant proportion of patients have frequent relapses or are refractory to treatment. The prognosis of patients with Refractory or Relapsed (R/R) PCNSL is abysmal. The optimal treatment for R/R PCNSL is poorly defined as there are only a limited number of studies in this setting. Several studies have recently shown that ibrutinib, a Bruton tyrosine kinase (BTK) inhibitor, has promising results in the treatment of R/R PCNSL. However, these are preliminary studies with a limited sample size. In this systematic review, we explored and critically appraised the evidence about the efficacy of the novel agent ibrutinib in treating R/R PCNSL.

Assessment of computed tomography perfusion RAPID estimated core volume accuracy in patients following thrombectomy.

Background: Computed Tomography Perfusion (CTP) maps ischemic core volume (CV) and penumbra following a stroke; however, its accuracy in early symptom onset is not well studied. We compared the accuracy of CTP RAPID estimated CV with diffusion weighted imaging (DWI) infarct volume (IV) in patients following thrombectomy. Methods: Charts of anterior circulation large vessel occlusion post-thrombectomy cases with thrombolysis in cerebral infarction (TICI) 2b/3 reperfusion from 2017 to 2019 were reviewed. CTP time was dichotomized as 0-3 hours and ≥ 3 hours from the last known normal (LKN) cognition. The volumetric difference (VD), defined as DWI IV minus CTP CV, core volume overestimation (CVO), defined as CTP CV minus DWI IV and Alberta stroke programme early CT score (ASPECTS) were calculated. Large CV was defined as ≥ 50 ml CV. Modified Rankin Score (mRS) at 90 days were reviewed. We performed independent sample t-test and Spearman correlation coefficient test. Results: Total cases (n) were 61. In < 3 hours window from LKN (n = 27), the mean VD was 58.3 ± 0.1 ml (P = 0.990) and CVO (n = 11; 40.7%) was 39.6 ± 35.7 ml (P = 0.008). Mean large CV (n = 8) was 78.3 ± 25.4 ml with median ASPECTS of 8 [interquartile range (IQR) = 6.5-9.0] and median mRS at 90 days of 2 (IQR = 0.8-3.3). In ≥ 3 hours window from LKN (n = 34), CVO (n = 5) was uncommon and large CV had median mRS at 90 days of 5 (IQR = 4.0-6.0). Conclusion: CTP more frequently overestimates CV in patients who are < 3 hours from LKN. The treated patients with large CV in < 3 hours and > 3 hours had good and poor functional outcomes, respectively.

Safety and efficacy of Direct Oral Anticoagulants in cerebral venous thrombosis: A meta-analysis.

Cerebral venous thrombosis (CVT) is caused by partial or complete occlusion of the major cerebral venous sinuses or the smaller feeding cortical veins which predispose to the risk of venous infarction and hemorrhage. Current guidelines recommend treating CVT with either low-molecular-weight heparin (LMWH) or unfractionated heparin (UFH) followed by an oral vitamin K antagonist (VKA) for 3-12 months. Direct oral anticoagulants (DOACs) have already established benefit over warfarin as a long-term treatment of symptomatic venous thromboembolic disorder like deep vein thrombosis (DVT), and pulmonary embolism (PE) given its equal efficacy and better safety profile. The benefit of DOACs over warfarin as a long-term anticoagulation for CVT has likewise been extensively studied, yet it has not been approved as first-line therapy in the current practice. We therefore performed a systematic review and meta-analysis of relevant studies to generate robust evidence regarding the safety and efficacy of DOACs in CVT. This meta-analysis demonstrates that the use of DOACs in CVT has similar efficacy and safety compared to VKAs with better recanalization rate.

Isolated Agenesis of Septum Pellucidum and Adult-Onset Seizure Tendency With Eye Closure Sensitivity.

Septum pellucidum is a thin midline membrane that separates the anterior horns of the lateral ventricle. Agenesis of septum pellucidum (ASP) is considered a continuum of forebrain maldevelopment. Isolated ASP is a rare radiographic finding of unclear significance. We report a case of a 42-year-old male with ASP who presented with a new-onset seizure and eye closure sensitivity seen in the electroencephalogram. Magnetic resonance imaging of the brain confirmed the ASP. In the absence of data about the association between seizure and ASP, further studies are needed to determine its significance.

Systolic blood pressure variability following endovascular thrombectomy and clinical outcome in acute ischemic stroke: A meta-analysis.

Blood pressure variability (BPV) has been linked with the outcome of acute ischemic stroke (AIS) after endovascular thrombectomy (EVT). However, the association of the stroke outcome with specific short-term BPV parameters is unclear. We did a systematic literature search for studies published from January 2010 to September 2020. Eligibility criteria included studies with (1) AIS patients treated with EVT with or without thrombolysis; and (2) analysis of the association between short-term systolic BPV parameter and clinical outcomes. Systolic BPV parameters included standard deviation (SD), coefficient of Variation (CoV), successive Variation (SV), and Variation independent of mean. A total of 11 studies were meta-analyzed, comprising 3520 patients who underwent EVT. Lower odds of achieving good functional outcome at 3 months; that is, modified Rankin Scale (mRS) score ≤2 was associated with SD (OR, 0.854; p = .02), CoV (OR, 0.572; p = .04), SV (OR 0.41; p = .00) of systolic blood pressure (SBP). Likewise, higher odds of one-point increase in mRS score was associated with SD (OR 1.42; p = .03), CoV (OR 1.464; p = .00) and SV (OR 2.605; p = .00) of SBP. However, high BPV was not associated with symptomatic intracranial hemorrhage and all-cause mortality at 90 days. The association of BPV and early neurological deterioration was inconclusive. Based on the available studies, short-term systolic BPV is indicative of the clinical outcome of patients following EVT in AIS. Further research should focus on personalized blood pressure management strategies, rather than a one-size-fits-all approach.

New-Onset Seizure With Possible Limbic Encephalitis in a Patient With COVID-19 Infection: A Case Report and Review.

With the outbreak of COVID-19 (coronavirus disease 2019) as a global pandemic, various of its neurological manifestations have been reported. We report a case of a 54-year-old male with new-onset seizure who tested positive for severe acute respiratory syndrome coronavirus 2 from a nasopharyngeal swab sample. Investigative findings, which included contrast-enhancing right posterior temporal lobe T2-hyperintensity on brain magnetic resonance imaging, right-sided lateralized periodic discharges on the electroencephalogram, and elevated protein level on cerebrospinal fluid analysis, supported the diagnosis of possible encephalitis from COVID-19 infection. The findings in this case are placed in the context of the existing literature.

Facial Diplegia with Preserved Reflexes: An Unusual Case of Anti-GD1b Antibody Associated Guillain-Barré Syndrome.

Anti-ganglioside D1b (GD1b) Immunoglobulin G (IgG) positive Guillain-Barré Syndrome (GBS) is rare and usually presents with acute sensory or cerebellar ataxia, ascending paralysis, and loss of deep tendon reflexes (DTRs). A 19-year-old female individual with recent Influenza A infection had an acute onset of facial diplegia and minimal leg weakness with preserved DTRs. Cerebrospinal fluid analysis showed albuminocytologic dissociation with positive serum anti-GD1b IgG antibody (52 IV; reference range 0-50). Magnetic resonance imaging of the cervical spine showed nerve root enhancement. Following intravenous immunoglobulin therapy and subsequent physiotherapy, the patient reached the nadir of leg weakness by one month and had complete motor recovery after one year. Sensory ataxia was observed in the fourth month of the illness, which subsided by eight months. DTRs were normal throughout the course of the disease. This case showed an unusual evolution of GBS with a positive anti-GD1b antibody presenting with acute facial diplegia, normal DTR and delayed sensory ataxia.

Gasperini Syndrome: A Case Report and Systematic Review and Proposing a New Definition.

Gasperini syndrome (GS), a rare brainstem syndrome, is featured by ipsilateral cranial nerves (CN) V-VIII dysfunction with contralateral hemibody hypoesthesia. While there have been 18 reported cases, the GS definition remains ambiguous. We report a new case and reviewed the clinical features of this syndrome from all published reports to propose a new definition. A 57-year-old man with acute brainstem stroke had right CN V-VIII and XII palsies, left body hypoesthesia and ataxia. Brain MRI showed an acute stroke in the right caudal pons and bilateral cerebellum. After a systematic review, we classified the clinical manifestations into core and associate features based on the frequencies of occurring neurological deficits. We propose that a definitive GS requires the presence of ipsilateral CN VI and VII palsies, plus one or more of the other three core features (ipsilateral CN V, VIII palsies and contralateral hemibody hemihypalgesia). Additionally, GS, similar to Wallenberg's syndrome, represents a spectrum that can have other associated neurological features. The revised definition presented in this study may enlighten physicians with the immediate recognition of the syndrome and help improve clinical localization of the lesions and its management.

A Case of Isolated Dysarthria in a COVID-19 Infected Stroke Patient: A Nondisabling Neurological Symptom With Grave Prognosis.

Isolated dysarthria is a speech abnormality characterized by slurring without any language dysfunction, or other neurological deficits. In an acute setting, it is commonly associated with stroke. In the context of social distancing during the current corona virus disease 2019 (COVID-19) pandemic, nondisabling symptoms such as isolated dysarthria can delay a patient's perception to seek immediate medical care. We present a rare case of isolated dysarthria in a COVID-19 infected stroke patient with a grave prognosis. A 79-year-old African American male presented with isolated dysarthria that manifested two days prior to his hospital visit. The dysarthria assessment showed impaired articulation, phonation, and prosody. Other neurological examinations were normal. He tested positive for the COVID-19 infection. His pulmonary CT scan showed bilateral ground glass opacities. An electrocardiogram showed atrial fibrillation (AF). Brain MRI revealed a punctate acute infarction in the left frontal lobe. Initially, he was treated with IV anticoagulation, oral beta-blocker, azithromycin and hydroxychloroquine, but he dramatically deteriorated within a week exhibiting a highly elevated cytokine level eventually resulting in multi-system organ failure. Despite aggressive treatment with steroids, tocilizumab and other supportive measures, the patient died of cardiac arrest. Our case highlights that acute stroke could manifest as an isolated dysarthria, which is an indicator of increased severity and high mortality with COVID-19 infection. Public awareness about the stroke symptom awareness should be emphasized.

Reoccurrence of Stroke in a Patient With Peri-Device Leak of WATCHMAN Device.

Atrial fibrillation is the leading cause of cardioembolic stroke, with emboli most commonly originating from the left atrial appendage. We report the case of a 71-year-old male with left atrial appendage closure via implantation of the WATCHMAN device, due to possible anticoagulation therapy failure and increased bleeding risk, following a stroke. Following a new stroke over a year later, a 1.8-mm peri-device leak was observed. Surgical records noted a minimal (<5 mm jet flow) peri-device leak after the installation, which was considered successful WATCHMAN implantation per protocol. This case highlights the persistent risk of cardioembolic stroke in patients with nonvalvular atrial fibrillation despite device implantation and questions the significance of peri-device leak and further management with anticoagulation for recurrent stroke.

Generalized tonic-clonic seizures with post-ictal atrial fibrillation.

Convulsive seizures are known to cause severe cardiopulmonary changes and increased autonomic activity. Limited reports describe peri-ictal cardiac arrhythmias such as atrial fibrillation (AF) with generalized tonic-clonic seizures (GTCS). We present a unique case of a healthy 23-year-old male patient with new onset prolonged AF in the setting of new onset seizures, occurring on three independent occasions. Over two years, our patient had multiple hospitalizations for seizures with an electrocardiogram (ECG) diagnosis of AF made on three different occasions, occurring during his post-ictal state (all within 30 min of seizure onset). These seizures were never captured by electroencephalography (EEG) or witnessed by the medical staff, but were reported by family and/or reviewed on video provided by them. After his first GTCS, his AF persisted and was medically cardioverted. Two additional instances of AF after witnessed GTCS have been captured. After his second unprovoked seizure, an anti-seizure drug (ASD) was prescribed. A multi-disciplinary approach may be adopted to address comorbidities associated with seizures. Aggressive evaluation and treatment should be employed for newly diagnosed and refractory seizure patients associated with arrhythmias, in our case AF. Peri-ictal arrhythmias may be considered a potential marker for increased sudden unexpected death in epilepsy (SUDEP) risk.