Neurosarcoidosis

BACKGROUND: Studies on sarcoidosis in elderly patients are scarce and none have specifically evaluated patients aged ≥75 at onset. AIM: We aimed to analyse the characteristics of patients with sarcoidosis diagnosed after 75 and to compare them with those of younger patients. DESIGN: Multicenter case-control study comparing elderly-onset sarcoidosis (EOS) with young-onset sarcoidosis (YOS) seen at Lyon University Hospitals between 2006 and 2018...

INTRODUCTION: Neurosarcoidosis is a rare complication of sarcoidosis. There are small series on the condition and very few from Spain. We conducted a retrospective study of neurosarcoidosis in Virgen de la Victoria Hospital over the last 10 years. PATIENTS AND METHOD: the medical records of patients diagnosed with sarcoidosis in our setting in the last 10 years were reviewed. RESULTS: One hundred twenty patients with sarcoidosis, 20 patients with probable neurosarcoidosis: 30% at the beginning of the illness, 55% later, 15% had isolated neurosarcoidosis...

PURPOSE OF REVIEW: This article provides an overview and update on the neurologic manifestations of sarcoidosis. RECENT FINDINGS: The 2018 Neurosarcoidosis Consortium diagnostic criteria emphasize that biopsy is key for diagnosis and determines the level of diagnostic certainty. Thus, definite neurosarcoidosis requires nervous system biopsy and probable neurosarcoidosis requires biopsy from extraneural tissue. Without biopsy, possible neurosarcoidosis can be diagnosed if the clinical, imaging, and laboratory picture is compatible and other causes are ruled out...

A 58-year-old woman experienced 4 months of headache, fatigue, and depression, as well as 1 week of confusion and hallucinations. F-FDG PET/CT showed numerous hypermetabolic lesions in the pituitary gland and stalk, dura mater, glossopharyngeal nerve, and cauda equina, as well as in cervical and mediastinal lymph nodes. Surgical biopsy of the mediastinal lymph node confirmed the diagnosis of sarcoidosis. The follow-up F-FDG PET/CT, performed at the eighth month after the treatment of glucocorticoids and immunosuppressive agents, demonstrated complete remission of the previous hypermetabolic lesions...

Sarcoidosis affects the nervous system in 10% of cases. When it does so it can affect any part of the nervous system and with all degrees of severity. It forms part of the differential diagnosis in inflammatory, infective, neoplastic and degenerative neurological diseases and may be very difficult to diagnose without histological confirmation. Recent clinical studies and the increasing availability of new biological treatments allow a much clearer understanding of the disease. This review summarises its clinical features, imaging and laboratory characteristics, treatment and outcome...

OBJECTIVE: To characterize patients with neurosarcoidosis within the University of Utah healthcare system, including demographics, clinical characteristics, treatment, and long-term outcomes. METHODS: We describe the clinical features and outcomes of patients with neurosarcoidosis within the University of Utah healthcare system (a large referral center for 10% of the continental United States by land mass). Patients were selected who met the following criteria: (1) at least one International Classification of Diseases Clinical Modification , 9th revision code 135 or International Classification of Diseases Clinical Modification , 10th revision code D86* (sarcoidosis) and (2) at least one outpatient visit with a University of Utah clinician in the Neurology Department within the University of Utah electronic health record...

Distinguishing neurosarcoidosis (NS) from multiple sclerosis (MS) remains challenging and available parameters lack discriminatory power. Comprehensive flow cytometry data of blood and CSF leukocytes of patients with NS (n = 24), MS (n = 49) and idiopathic intracranial hypertension (IIH, n = 52) were analyzed by machine learning algorithms. NS featured a specific immune cell pattern with increased activated CD4+ T cells in CSF and increased plasma cells in blood. Combining blood and CSF parameters improved the differentiation...

OBJECTIVE: To characterize a cohort of patients with neurosarcoidosis with particular focus on CSF analysis and to investigate whether CSF values could help in distinguishing it from multiple sclerosis (MS). METHODS: This retrospective cohort study enrolled 85 patients with a diagnosis of neurosarcoidosis (possible, probable, or definite). CSF total protein, white cell count, and angiotensin-converting enzyme levels were measured. CSF and serum oligoclonal immunoglobulin G (IgG) patterns were analyzed with the use of odds ratios and binary logistic regression...

OBJECTIVES: Neurological complications of sarcoidosis are uncommon and the natural history and optimal treatments under-researched. With the advent of biological therapies, it is important to define the clinical characteristics and immunopathology of the disease. PATIENTS AND METHODS: Patients referred to and treated within the Centre for Neurosarcoidosis over a 15 year period who had biopsy proven "highly probable" disease of the central nervous system were studied prospectively...

Neuromyelitis optica (NMO) was long considered a clinical variant of multiple sclerosis (MS). However, the discovery of a novel and pathogenic anti-astrocytic serum autoantibody targeting aquaporin-4 (termed NMO-IgG or AQP4-Ab), the most abundant water channel protein in the central nervous system, led to the recognition of NMO as a distinct disease entity in its own right and generated strong and persisting interest in the condition. NMO is now studied as a prototypic autoimmune disorder, which differs from MS in terms of immunopathogenesis, clinicoradiological presentation, optimum treatment, and prognosis...

INTRODUCTION: In the clinical setting, the diagnosis of neurosarcoidosis in patients with central diabetes insipidus (CDI) is typically based both on symptoms (i.e. polydipsia or polyuria) and brain magnetic resonance imaging (MRI) findings (e.g. pituitary abnormality). However, inconsistent changes in the patient's symptoms and brain MRI findings may occur during the clinical course of the disease. This review was performed to summarise the relationship between symptoms and brain MRI findings in previously reported cases of neurosarcoidosis with CDI...

A 41-year-old lady presented with headache and ophthalmoplegia of the left eye for 1 week and underwent magnetic resonance imaging (MRI) of the brain, which showed homogeneously enhancing nodular thickening of meninges in the left anterior and middle cranial fossa with perilesional white matter edema of the left fronto-temporal lobes which appeared suspicious for neurosarcoidosis. She subsequently underwent 18F-fluorodeoxyglucose positron emission tomography-computed tomography (18F-FDG PET/CT) to determine the disease extent and to target the optimal biopsy site...

OBJECTIVES: To describe a unique case of isolated bilateral sarcoidosis of the cerebellopontine angle as well as the related imaging in the case. To conduct a literature review of the published articles regarding sarcoidosis of the cerebellopontine angle. DATA SOURCES: Representative case report from a single institution as well as PubMed and Scopus database searches. METHODS: In addition to a retrospective review, all published case reports and case series of sarcoidosis involving the cerebellopontine angle from 1960 to July 2018 in the English language were reviewed...

No abstract text is available yet for this article.

PURPOSE OF REVIEW: Advanced sarcoidosis is an important cause of morbidity and mortality in sarcoidosis. Over the past few years, several studies have been published clarifying the prevalence and severity of this condition. RECENT FINDINGS: Pulmonary involvement is the most common form of sarcoidosis. Increased morbidity and significant mortality is encountered in advanced lung disease. Although many sarcoidosis patients with pulmonary fibrosis have a normal life expectancy, at least 20% develop progression and may die from this complication...

Plasma exchange is a highly efficient technique to remove circulating autoantibodies and other humoral factors rapidly from the vascular compartment. It was the first effective acute treatment for peripheral disorders such as Guillain-Barré syndrome and myasthenia gravis before intravenous immunoglobulin became available. The recent recognition of rapidly progressive severe antibody-mediated central nervous system disorders, such as neuromyelitis optica spectrum disorders and anti-N-methyl-D-aspartate-receptor encephalitis, has renewed interest in using plasma exchange for their acute treatment also...

The cause of sellar region masses in large retrospective series is overwhelmingly pituitary adenomas (84.6%), followed by craniopharyngiomas (3.2%), cystic nonneoplastic lesions (2.8%), inflammatory lesions (1.1%), meningiomas (0.94%), metastases (0.6%), and chordomas (0.5%) (1). While other rare lesions were also identified (collectively 6.0%), single unusual entities in the above-cited series numbered <1-2 examples each out of the 4122 cases, underscoring their rarity. We searched our joint files for rare, often singular, sellar/suprasellar masses that we had encountered over the past several decades in our own specialty, tertiary care specialty pituitary center practices...

The differential diagnosis of swollen optic nerves differs according to whether the swelling is unilateral or bilateral, or whether visual function is normal or affected. Patients with a unilaterally swollen optic nerve and normal visual function most likely have optic nerve head drusen. Patients with abnormal visual function most likely have demyelinating optic neuritis or non-arteritic anterior ischaemic optic neuropathy. Patients with bilaterally swollen optic nerve heads and normal visual function most likely have papilloedema, and require neuroimaging followed by lumbar puncture...

No abstract text is available yet for this article.

PURPOSE OF REVIEW: To review the diagnostic approach to sarcoidosis. There is no gold standard diagnostic test, procedure or algorithm for sarcoidosis. The diagnosis is based on a compatible clinical presentation, histologic findings of granulomatous inflammation, exclusion of alternative diseases, and evidence of systemic involvement. Occasionally, there may be exceptions to several of these requirements. RECENT FINDINGS: The diagnostic specificity of several specific clinical features are discussed...