Neuropsychiatric lupus erythematosus: Focusing on autoantibodies.

Patients with systemic lupus erythematosus (SLE) frequently suffer from nervous system complications, termed neuropsychiatric lupus erythematosus (NPLE). NPLE accounts for the poor prognosis of SLE. Correct attribution of NP events to SLE is the primary principle in managing NPLE.

Primary CNS vasculitis (PCNSV): a cohort study.

Primary CNS Vasculitis (PCNSV) is a rare inflammatory disorder affecting the blood vessels of the central nervous system. Patients present with a combination of headaches, seizures, and focal neurological deficits. There is usually a diagnostic delay.

Vertebrobasilar circulation hemorrhages in childhood primary angiitis of the central nervous system.

Childhood primary angiitis of the CNS (cPACNS) is a poorly understood, rare, and diagnostically challenging neurologic disease. We describe an unusual and autopsy-confirmed case of cPACNS presenting as vertebrobasilar circulation hemorrhagic strokes in a 4-year-old girl. The presentation and clinical features were inconsistent with primary CNS vasculitis and skewed the diagnosis.

Right arm weakness and mouth deviation as a presentation of Primary Angiitis of the Central Nervous System treated with rituximab: A case-report.

Introduction: Primary Angiitis of the Central Nervous System (PACNS) is a rare form of vasculitis that solely affects the Central Nervous System (CNS). Its presentation varies widely from a simple headache to a stroke-like presentation. PACNS management is divided into an induction phase, which includes corticosteroids, cyclophosphamide and rituximab, and a maintenance phase which includes: methotrexate, mycophenolate mofetil, rituximab and azathioprine.

Pseudo-insular glioma syndrome: illustrative cases.

Background: Lower-grade insular gliomas often appear as expansile and infiltrative masses on magnetic resonance imaging (MRI). However, there are nonneoplastic lesions of the insula, such as demyelinating disease and vasculopathies, that can mimic insular gliomas.

Observations: The authors report two patients who presented with headaches and were found to have mass lesions concerning for lower-grade insular glioma based on MRI obtained at initial presentation.

A 5-year-old child presenting with tumor-like primary angiitis of the central nervous system.

Introduction: Primary angiitis of the central nervous system (PACNS) is a vasculitis confined to the CNS. A small proportion of the lesions may present as a tumor-like mass, which is rarely seen in children.

Case Presentation: A 5-year-old girl was admitted to our hospital because of an intermittent headache.

Childhood Cerebral Vasculitis : A Multidisciplinary Approach.

Cerebral vasculitis is increasingly recognized as a common cause of pediatric arterial stroke. It can present with focal neurological deficits, psychiatric manifestations, seizures, and encephalopathy. The etiopathogenesis of childhood cerebral vasculitis (CCV) is multifactorial, making an inclusive classification challenging.

[Primary CNS Vasculitis - An Overview].

Primary CNS Vasculitis - An Overview Cerebral vasculitis, especially the primary vasculitis of the central nervous systems (primary CNS vasculitis), are rare inflammatory diseases of the small- and medium-sized vessels of the CNS. The pathogenesis of primary CNS vasculitis is unclear. Infectious triggers are hypothesized to induce an activation of the immune system with resulting inflammation of the blood vessels within the CNS.

A 14-year-old Girl with Right-Sided Weakness and Speech Difficulty.

We herein report a case of Childhood Primary Central Nervous System (CNS) Angiitis. This case consisted of a 14-year-old girl who presented with right-sided weakness, aphasia, and a fever. A Magnetic Resonance Imaging of the brain showed multifocal areas of ischemia.

Inpatient Management of Encephalopathy.

Unexplained encephalopathy is a common occurrence in tertiary care centers and neurologic disorders should be considered after ruling out the infectious, toxic and metabolic etiologies. Neuroimaging combined with a thorough history and examination is often helpful in ruling out stroke and fulminant demyelinating encephalopathies. Autoimmune encephalopathy should be suspected in any patient with unexplained acute or subacute onset encephalopathy or rapidly progressing dementia.

Central nervous system vasculitis in a patient with axial spondyloarthritis treated with infliximab: A case report and literature review.

Central nervous system (CNS) vasculitis is a rare form of vasculitis involving the blood vessels of the brain. It may be primary when it is confined to the CNS or secondary in the context of systemic inflammatory conditions such as systemic lupus erythematosus, rheumatoid arthritis, or infections. However, there is no known association with axial spondyloarthritis.

A Case of Metastatic CNS Melanoma of Unknown Primary Presenting with Seizures.

Seizures are a common occurrence. The goal of evaluating a seizure is to identify the etiology and to determine the likelihood of recurrence as well as guide management. We present a unique presentation of a 47-year-old female that presented with late onset seizures admitted due to status epilepticus.

Case Report: Meningoencephalitis With Thrombotic Occlusive Vasculopathy in a Young EBV-Naïve Boy Is Associated With a Novel Mutation.

X-linked lymphoproliferative disease (XLP1) is a combined immunodeficiency characterized by severe immune dysregulation caused by mutations in the gene. Loss or dysfunction of SH2D1A is associated with the inability in clearing Epstein-Barr-Virus (EBV) infections. Clinical manifestation is diverse and ranges from life-threatening hemophagocytic lymphohistiocytosis (HLH) and fulminant infectious mononucleosis (FIM) to lymphoma and antibody deficiency.

TLR8/TLR7 dysregulation due to a novel TLR8 mutation causes severe autoimmune hemolytic anemia and autoinflammation in identical twins.

Our study presents a novel germline c.1715G>T (p.G572V) mutation in the gene encoding Toll-like receptor 8 (TLR8) causing an autoimmune and autoinflammatory disorder in a family with monozygotic male twins, who suffer from severe autoimmune hemolytic anemia worsening with infections, and autoinflammation presenting as fevers, enteritis, arthritis, and CNS vasculitis.

Tocilizumab for severe refractory primary central nervous system vasculitis: A center experience.

Background: Primary Central Nervous System Vasculitis (PCNSV) is responsible for 3%-5% of strokes before age 50. It presents with clinical, radiological, and pathological variability. Optimal management is unknown given the absence of randomized clinical trials.

Update on treatment strategies for vasculitis affecting the central nervous system.

Vasculitis affecting the nervous system is a rare disease that can not only present with nonspecific initial symptoms, but also run a severe course without accurate treatment. Although improvements have been achieved, diagnosis of vasculitis remains challenging, because many classification criteria are unspecific or inconclusive with regard to central nervous system (CNS) manifestations. Currently, beside an isolated primary CNS vasculitis, several systemic types of vasculitis are known to affect the nervous system.

Focal status epilepticus may trigger relapse of primary angiitis of the CNS.

The role of neuroinflammation in epileptogenesis is extensively investigated, but short-term effects of seizures on established CNS pathologies are less studied and less predictable. We describe the case of a woman with previous recurrent episodes of focal cerebral haemorrhage of unknown cause who developed a pseudo-tumoural oedema triggered by provoked focal status epilepticus. A brain biopsy revealed that the underlying condition was primary angiitis of the CNS.

High-Resolution Vessel Wall Imaging in Primary Angiitis of Central Nervous System.

Background: High-resolution vessel wall imaging (HRVWI) can aid in differentiating the various intracranial vasculopathies, but has been sparingly used in the diagnosis of primary angiitis of central nervous system (PACNS). This study is aimed to describe the vessel wall imaging characteristics of PACNS.

Materials And Methods: Patients with confirmed diagnosis of PACNS according to the Calabrese and Mallek criteria who had abnormal HRVWI were included in this retrospective descriptive study.

Diagnosis and Treatment of Small Vessel Childhood Primary Angiitis of the Central Nervous System (sv-cPACNS): An International Survey.

Childhood primary angiitis of the Central Nervous System (cPACNS) is a rare autoimmune and inflammatory disease. It can result in significant neuronal damage, neurodevelopmental delay and potentially death. Childhood PACNS is divided into subcategories: angiography-positive p-cPACNS that affects medium and large vessels, and angiography-negative small vessel sv-cPACNS.

Fused F-Choline PET/MRI: A potential non-invasive biomarker imaging tool in cerebral vasculitis.

Primary CNS vasculitis is an inflammatory brain disease commonly misdiagnosed affecting the medium and small vessels of the CNS. Due to its broad and non-specific clinical and radiological manifestations; Its diagnosis remains challenging. New diagnostic tools and biomarkers which increase specificity and facilitate the diagnosis for patients with suspected vasculitis are highly desirable to enable physicians to start therapy that can alter its potential aggressive course like immunosuppressant.