Neuroinvasive West Nile Virus (WNV) Encephalitis With Anton Syndrome: Epidemiology and Pathophysiology Review

2022

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Susac syndrome (SuS) is a rare autoimmune endotheliopathy that affects the retina, cochlea, and central nervous system (CNS). Even fewer cases of SuS have been reported with dermatological findings, including livedo reticularis and racemosa. The case of SuS reported here presents with encephalopathy, visual disturbances, hearing loss, and a diffuse rash on the abdomen and flank. Magnetic resonance imaging (MRI) of the brain confirmed lesions within the corpus callosum, and an audiogram revealed a unilateral biphasic sensorineural hearing loss in the right ear. A skin biopsy was completed that revealed congested dermal vessels with lymphocytic perivascular infiltrates consistent with livedo reticularis and vasculopathy. Management included intravenous methylprednisolone (IVMP) and a tapering oral dose of prednisone. The patient was also administered 1000 mg of cyclophosphamide with a two-week follow-up for a repeat infusion.Cytotoxic T-lymphocytes (CTLs) and auto-endothelial cell antibodies (AECAs) are hypothesized to play a key role in the pathogenesis of SuS. Livedo reticularis occurs due to congestion of dermal vessels and can be both physiological and pathological in etiology. Pathological etiologies include autoimmune vasculopathies, connective tissue disorders, and drugs (catecholaminergic agents, amantadine, quinidine, etc.). A literature review of SuS cases with associated dermatologic findings is included. Five cases were identified, and neurologic manifestations, dermatologic manifestations, and interventions are described.

Section: Discussionmentioning

confidence: 99%

Susac Syndrome With Livedo Reticularis: Pathogenesis and Literature Review

2022

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Section: Discussionmentioning

confidence: 99%

“…Inflammatory astrocytes, and microglia release chemokines that increase blood-brain barrier permeability (BBB) and lead to the recruitment of other inflammatory cells (Figure 5). Increased BBB permeability is also implicated in other CNS infections such as flaviviruses [6]. The figure was partly generated using Servier Medical Art (Servier Laboratories, Suresnes, France), licensed under a Creative Commons Attribution 3.0 unported license.…”

Section: Table 1: the Four Stages Of Brain Abscess Formationmentioning

confidence: 99%

Plasma Microbial Cell-Free DNA (CF-DNA) Next-Generation Sequencing in Diagnosing Intracranial Abscesses: Pathophysiology and a Scoping Review of the Literature

2022

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Plasma microbial cell-free DNA (cf-DNA) from next-generation sequencing (NGS) provides improved sensitivity and specificity compared to standard microbial blood cultures. cf-DNA sequencing also has an improved turnaround time (TAT) and allows quicker commencement of antibiotics in life-threatening infections such as a brain abscess. Brain abscesses carry significant morbidity and mortality. Empiric treatment and management are critical in improving functional neurological outcomes. Reported here is the case of a severe central nervous system (CNS) infection with multiple ring-enhancing lesions seen throughout the cerebrum on magnetic resonance imaging (MRI). Standard microbial blood cultures were inconclusive and definitive identification of the pathogen was achieved through microbial cf-DNA NGS.Brain abscesses develop in four distinct phases: early cerebritis, late cerebritis, early capsule formation, and late capsule formation. The pathogenesis of cerebral abscess involves direct parenchymal inflammation of the pathogen, the recruitment of inflammatory CNS cell types (microglia, inflammatory astrocytes, etc), and the chemotaxis of immune cells. cf-DNA is released into the bloodstream in response to pathogen opsonization and immune-mediated cell death. A scoping literature review includes cases of intracranial abscesses diagnosed via cf-DNA NGS.

“…Many cases of CJD often present with diffusion restriction within the cerebral cortex and basal ganglia. Neuroinfectious causes of subcortical lesions include flaviviruses such as the West Nile virus [ 7 ].…”

Section: Discussionmentioning

confidence: 99%

Sporadic Creutzfeldt-Jakob Disease With Status Epilepticus: Molecular Mechanisms and a Scoping Review of the Literature

2022

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Creutzfeldt-Jakob disease (CJD) is a rapidly progressing neurodegenerative disorder and is a spongiform encephalopathy. A 59-year-old male presented with subacute-onset worsening encephalopathy and was found to be in non-convulsive status epilepticus (NCSE) requiring intubation and a midazolam infusion for refractory seizures. Electroencephalogram (EEG) revealed triphasic repeats with focal epileptogenic originating from the left parietal region. The patient continued to have up to 25-40 non-convulsive seizures per day. Cerebrospinal fluid (CSF) analysis revealed elevated 14-3-3 and tau protein. A real-time quakinginduced conversion assay in CSF was positive. The patient was diagnosed with probable sporadic CJD based on criteria from the Centers for Disease Control. Supportive treatment was provided.Cellular prion protein (PrP C ) plays an important role in myelination of the peripheral nervous system, regulation of the neuronal membrane, and circadian rhythm. The molecular mechanisms of CJD involve the catalyzation of the physiological PrP C into the pathological prion protein (PrP Sc ). This post-translational change in conformation leads to the generation of PrP Sc and is involved in spongiform encephalopathies. Mechanisms of neurodegeneration include astrocytosis, neuronal apoptosis, and amyloid plaque formation. A scoping literature review was conducted in three databases on cases of CJD with SE. A total of 13 cases are identified that include the type of CJD and the morphology of the seizures. NCSE is the most prevalent form of SE in patients with CJD.