neurolupus

Depicting the cellular and molecular bases of the continuous dialogue existing between the peripheral immune and the central nervous systems, as in neurolupus, is fundamental to improve, and better apprehend the role played by immune cells and mediators in the initiation and progression of neurological and psychiatric diseases, which nowadays remain a major public health issue. The relative frequency of neurological symptoms occurring in systemic autoimmunity is particularly worrying as, for example, two-thirds of patients with lupus will eventually experience the disabling effects of neuropsychiatric lupus...

This article estimates the frequency of cardiovascular (CV) events that occurred after diagnosis in a large Spanish cohort of patients with systemic lupus erythematosus (SLE) and investigates the main risk factors for atherosclerosis. RELESSER is a nationwide multicenter, hospital-based registry of SLE patients. This is a cross-sectional study. Demographic and clinical variables, the presence of traditional risk factors, and CV events were collected. A CV event was defined as a myocardial infarction, angina, stroke, and/or peripheral artery disease...

Cerebellar ataxia is a rare manifestation of neuropsychiatric systemic lupus erythematosus (SLE). Development of vasculitic infarcts in the cerebellum is the most plausible reason of this manifestation. We report the case of a patient who presented with characteristic skin rashes of lupus along with cerebellar signs. Imaging of brain in this patient revealed prominent cerebellar atrophy. She was treated with mycophenolate mofetil and oral corticosteroid, and there was no further progression of her neurological signs after the initiation of therapy...

Neuropsychiatric manifestations can be a serious complication of systemic lupus erythematosus, affecting nearly 56% of these patients. Frequently, acceptable clinical outcome is observed in neurolupus with immunosuppressive therapy. Different metabolites identified with MR spectroscopy may be associated with modifications in the natural history of this disease, specifically in the central nervous system. We report a case of neurolupus with progressive neurologic impairment despite aggressive immunosuppressive treatment...

We report the case of a 30-year-old woman, without any previous comorbidities presenting with acute onset headache, altered sensorium and unsteadiness of gait. Neurological evaluation revealed a drowsy patient with papilloedema, bilateral lateral rectus palsy, generalised hyper-reflexia and up going plantar responses. Urgent imaging performed showed extensive cortical venous sinus thrombosis. Workup for secondary causes of cortical venous sinus thrombosis revealed very high titres of antinuclear antibody and anti-dsDNA, but negative antiphospholipid antibodies (APLA)...

The antiphospholipid syndrome (APS) is defined by the association of high titers of antiphospholipid antibodies (aPLs) with thrombotic events and/or obstetrical problems. APS can be isolated or associated with immune system diseases. Several central nervous system (CNS) manifestations have been reported in APS, but are still not included in the international diagnostic criteria. We present here three cases of APS revealed by CNS manifestations. The first patient had a primary APS with stroke, dementia, epilepsy and the "so-called" Liebman-Sacks disease, a subacute thrombotic non-bacterial valvulopathy...

OBJECTIVE: To define the pattern of disease expression and to gain better understanding in patients with juvenile onset systemic lupus erythematosus (SLE) in Portugal. METHODS: The features of unselected patients with systemic lupus erythematosus who had disease onset before the age of 18 years were retrospectively analysed in three Portuguese centres with Pediatric Rheumatology Clinic over a 24-year period (1987-2011). Demographic, clinical and laboratory manifestations, therapy and outcome were assessed...

INTRODUCTION: While the neurolupus criteria are well-established, global psychiatric manifestations are of variable frequency in patients with systemic lupus erythematosus (SLE); their relation with disease activity is unknown. OBJECTIVE: To evaluate the frequency of psychiatric symptoms in SLE using the Adult Psychiatric Morbidity Questionnaire (APMQ); to correlate APMQ changes with disease activity and socio-economic variables. MATERIALS AND METHODS: This cross-sectional study evaluated patients with active or inactive SLE as to the prevalence of psychiatric symptoms utilizing, for the first time, the APMQ...

Complex movement disorder is a relatively rare presentation of neurolupus. Antiphospholipid antibodies are associated with movement disorders likely via aberrant neuronal stimulation. Antiribosomal P antibodies have been previously associated with neuropsychiatric disorders but their correlation with movement disorder was not previously established. Our case report involves a 17-year-old Caucasian female patient positive for only antiribosomal P antibody and lupus anticoagulant who presented with a sudden onset of complex movement disorder...

Delirium is common in older people. It is a crucial diagnosis because it raises the morbidity and the mortality. Diagnostic tools like Mini Mental State Examination (MMSE), the confusion assessment method (CAM), and the Diagnostic and Statistical Manual of Mental Disorders, Fourth Edition (DSMIV), can help with the diagnosis. We report a case of a woman with neurological symptoms which look like dementia. We diagnosed systemic lupus erythematosus (SLE). The apparition of late-onset SLE is more insidious, which leads to misdiagnosis...

Several autoantibodies are culprits in the pathogenesis of organ damage in systemic lupus erythematosus, by means of established or postulated mechanisms, whereby inducing a perturbation of cell structure and function, with consequent tissue-organ impairment. Common autoantibody-mediated mechanisms of damage include cell surface binding with or without cytolysis, immune complex-mediated damage, penetration into living cells, binding to cross-reactive extracellular molecules. Experimental data from both murine models and humans have recently clarified the key role of autoantibodies in severe organ involvements, including nephritis, neuropsychiatric (NP) dysfunction, and cerebrovascular disease (CVD)...

Acquired ichthyosis and livedoid palmoplantar keratoderma have rarely been described in systemic lupus erythematosus (SLE). In the first case, a 51-year-old man presented with generalized acquired ichthyosis associated with renal glomurelosclerosis and neurolupus. rituximab allowed total resolution of the acquired ichthyosis. A livedoid palmoplantar keratoderma was observed in a 45-year-old woman as the initial clinical manifestation of a systemic lupus erythematosus associated with Raynaud's phenomenon, photosensitivity and inflammatory polyarthralgia...

Almost half of lupus patients will experience neuropsychiatric symptoms during the course of their disease. The etiology of the neuronal damages is still uncertain and probably multiple. Autoantibodies reactive with brain have been postulated to play a role. The observation of pathogenic autoantibodies binding the NR2A and NR2B subunits of the ionotropic glutamate receptor (NMDAR) illustrates this hypothesis. First studies showed that 40% of lupus patients possess serum titers of anti-NR2A/B antibody, but the presence of these autoantibodies is not always associated with the occurrence of neuronal damages or neuropsychiatric symptoms...

Systemic lupus erythematosus (SLE) is not an uncommon condition. Most neurologists are well aware that it can cause a wide range of neurological complications, and SLE almost invariably appears on 'differential diagnosis' lists in cases of clinical uncertainty. However, the precise nature of the manifestations of SLE in the central and peripheral nervous systems is perhaps less widely understood, and misperceptions about phenomenology and treatment are common. Here we survey some of the main primary neurological complications of SLE--'neurolupus'--while acknowledging that secondary problems, either iatrogenic or relating to other consequences of SLE (eg, hypertensive CNS disease, for example, secondary to renal lupus) are neither less serious nor less treatable...

INTRODUCTION: Neurological manifestations of systemic lupus erythematosus are frequent and polymorphic. In 40% of cases, lupus can be revealed by neurological symptoms. Cerebral nervous system complications predominate and can be a negative factor for prognosis. Peripheral features are rare and various and can compromise functional prognosis, sometimes with fatal outcome. CASE REPORT: We report the case of a 30-year-old woman who presented a cerebral venous thrombosis of the superior longitudinal sinus...

OBJECTIVE: Serum IgG antibodies (Abs) to phosphorylated ribosomal (P ribosomal) proteins have been inconsistently associated with neuropsychiatric manifestations in systemic lupus erythematosus (SLE). Our aim was to assess whether serum IgG Abs to ribosomal P proteins are associated with neuropsychiatric SLE. PATIENTS AND METHODS: We examined an inception cohort of 219 SLE patients. Neuropsychiatric SLE manifestations were characterized using the American College of Rheumatology (ACR) definition...

Accurate MRI characterization of brain lesions is critical for planning therapeutic strategy, assessing prognosis and monitoring response to therapy. Conventional MRI with gadolinium-based contrast agents is useful for the evaluation of brain lesions, but this approach primarily depicts areas of disruption of the blood-brain barrier (BBB) rather than tissue perfusion. Advanced MR imaging techniques such as dynamic contrast agent-enhanced perfusion MRI provide physiological information that complements the anatomic data available from conventional MRI...

BACKGROUND: Systemic lupus erythematosus may present with a wide variety of neurological manifestations, which could be related to the presence of vasculopathy and microembolic signals (MES). OBJECTIVES: We aimed to determine the prevalence of MES and altered blood flow velocities (BFVs) by transcranial Doppler (TCD) in patients with acute neuropsychiatric lupus erythematosus (NPLE). METHODS: Fifteen consecutive patients with acute NPLE (cases) and 30 patients with systemic lupus erythematosus but without acute NPLE (controls) underwent TCD testing for comparison purposes...

INTRODUCTION: Progressive multifocal leukoencephalopathy (PML) is an opportunistic infection of the central nervous system, occurring in immunocompromised patients. Treatment, not codified to date, is more often inefficient with a rapid and fatal deterioration. CASE RECORD: A 48-year-old woman, treated with immunosuppressant agents for systemic lupus, presented with PML mimicking neurolupus flare. A complete remission was obtained with cytarabine and cidofovir. CONCLUSION: Combined cytarabine and cidofovir appears a promising therapeutic option in PML associated with autoimmune systemic disorders...

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