Predicting the final clinical phenotype after the first attack of optic neuritis.

Background And Objectives: To evaluate the factors determining the final clinical phenotype after an initial isolated attack of optic neuritis (ON). ON could be an isolated event or the initial presentation of a chronic neuroimmunological condition.

Methods: This was a retrospective analysis of patients presenting to University Hospitals Cleveland Medical Center for an initial, isolated attack of ON.

Comparison between radiometry and spectrophotometry for the determination of angiotensin-converting enzyme activity in cerebrospinal fluid.

Determination of angiotensin-converting enzyme (ACE) activity in cerebrospinal fluid (CSF) can help for establishing the diagnosis of neurosarcoidosis. We investigated the performance characteristics of two assays for ACE determination in 57 CSF, radiometry with [glycine-1-14C] benzoyl-L-histidyl-L-leucine and spectrophotometry with furylacryloyl-phenylalanyl-L-glycyl-L-glycine (FAPGG) as substrates. We compared both kinetic assays to an ELISA specific for human ACE.

Neurosarcoidosis causing hydrocephalus: A case series.

Sarcoidosis is a granulomatous inflammatory disease that rarely affects the central nervous system as neurosarcoidosis. Neurosarcoidosis can affect any part of the nervous system causing a wide variety of clinical presentations ranging from seizures to optic neuritis. Here, we highlight rare cases of obstructive hydrocephalus in patients with neurosarcoidosis to make clinicians aware of this potential disease complication.

Resolution of Possible Neurosarcoidosis With Early Initiation of Glucocorticoids in the Acute Inpatient Setting: A Case Report.

Neurosarcoidosis is an autoimmune disorder of unknown etiology. We report a case of a 27-year-old African American male presenting with fever, vomiting, and seizure. Initially, bacterial meningitis was suspected, and empiric antibiotics with dexamethasone were started.

Cerebrospinal fluid protein levels are elevated 100 times in a Leptomeningeal metastasis patient: a case report and literature review.

Leptomeningeal metastasis (LM) has a high degree of malignancy and high mortality. We describe a patient admitted to hospital with acute lower extremity weakness, dysuria, and high intracranial pressure. Enhanced magnetic resonance imaging (MRI) showed extensive enhancement of the leptomeningeal and spinal meninges with multiple nodular changes and extensive fusion.

Neurosarcoidosis: Diagnostic Challenges and Mimics A Review.

Purpose Of Review: Neurosarcoidosis is a rare manifestation of sarcoidosis that is challenging to diagnose. Biopsy confirmation of granulomas is not sufficient, as other granulomatous diseases can present similarly. This review is intended to guide the clinician in identifying key conditions to exclude prior to concluding a diagnosis of neurosarcoidosis.

Tumefactive brain parenchymal neurosarcoidosis.

Background: Enhancing brain parenchymal disease, and especially tumefactive lesions, are an uncommon manifestation of neurosarcoidosis. Little is known about the clinical features of tumefactive lesions and their impact on management and outcomes, which this study aims to characterize.

Methods: Patients with pathologically-confirmed sarcoidosis were retrospectively reviewed and included if brain lesions were: (1) intraparenchymal, (2) larger than 1 cm in diameter, and (3) associated with edema and/or mass effect.

Neurosarcoidosis Presenting as Longitudinally Extensive Transverse Myelitis and Orbital Mass: A Case Report.

We describe a case of neurosarcoidosis in a 64-year-old female who presented with proptosis and orbital inflammation together with bilateral lower extremity neuropathy and longitudinally extensive transverse myelitis. These two entities are not commonly associated, and the etiology of the transverse myelitis was facilitated by an orbital biopsy. The transverse myelitis caused numbness in her lower extremities and tightness in her chest and abdomen, which progressed over weeks to difficulty walking and bilateral neuromuscular weakness.

Bilateral Vocal Fold Paralysis in a Patient With Neurosarcoidosis: A ChatGPT-Driven Case Report Describing an Unusual Presentation.

This ChatGPT-driven case report describes a unique presentation of neurosarcoidosis. The patient, a 58-year-old female, initially presented with hoarseness and was found to have bilateral jugular foramen tumors and thoracic lymphadenopathy. Imaging revealed significant enlargement and thickening of the vagus nerve and a separate mass of the cervical sympathetic trunk.

Red Flags for Chronic Inflammatory Demyelinating Polyradiculoneuropathy Associated with Sarcoidosis or Connective Tissue Diseases.

Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is a rare autoimmune disorder of the peripheral nervous system. Diagnosis relies on clinical and electrophysiological criteria. Various disorders requiring specific treatment regimens may be associated with CIDP, including sarcoidosis (SAR-CIDP) and connective tissue disease (CTD-CIDP).

Common Clinical and Molecular Pathways between Migraine and Sarcoidosis.

Migraine and sarcoidosis are two distinct medical conditions that may have some common biological and clinical pathways. Sarcoidosis is a chronic granulomatous disease characterized by the formation of granulomas in various organs, including the lungs, skin, cardiovascular system, lymph nodes, and brain. Migraine is a common comorbidity in sarcoidosis patients and a common neurological disorder characterized by recurrent headaches that can be accompanied by other symptoms, such as nausea, vomiting, and sensitivity to light and sound.

A case of oculocutaneous sarcoidosis.

Purpose: To present a case of extrapulmonary sarcoidosis presenting with ocular and cutaneous involvement.

Observations: We report a 54-year-male who presented with bilateral redness of eyes, photophobia, and diminished vision for a week. The best corrected visual acuity in the right eye was 6/60 and the left eye was counting fingers close to face (CFCF).

Cerebrospinal fluid B-cell activating factor levels as a novel biomarker in patients with neurosarcoidosis.

Objectives: Neurosarcoidosis (NS) is a severe complication of sarcoidosis. Patients with NS often have poor outcomes. To improve both the quality of life and prognosis in patients with NS, accurate and reliable methods for early diagnosis and determining the efficacy of treatment are needed.

Cerebral Vasculitis Revealing Systemic Sarcoidosis: A Case Report and Review of the Literature.

Vasculitis in neurosarcoidosis is rare, with only a few cases reported in the literature. We report the clinical observation of a 51-year-old patient with no previous medical history, who was admitted to the emergency department due to sudden onset confusion, fever, sweating, weakness, and headaches. The first brain scan was normal, but a further biological exam with a lumbar puncture revealed lymphocytic meningitis.

Characteristics of Progressive Multifocal Leukoencephalopathy Associated With Sarcoidosis Without Therapeutic Immune Suppression.

Importance: Progressive multifocal leukoencephalopathy can occur in the context of systemic sarcoidosis (S-PML) in the absence of therapeutic immune suppression and can initially be mistaken for neurosarcoidosis or other complications of sarcoidosis. Earlier recognition of S-PML could lead to more effective treatment of the disease.

Objective: To describe characteristics of patients with S-PML.

A comprehensive diagnostic approach in suspected neurosarcoidosis.

Neurosarcoidosis presents a diagnostic challenge in clinical settings, as it has no pathognomonic symptoms or signs and a wide range of differential diagnoses. The aim of this report is to present the pathological features of our group of patients, obtained through a systematic diagnostic approach. This retrospective cohort study enrolled all adult patients primarily diagnosed with neurosarcoidosis at the neurology department of a tertiary center in Sweden over a period of 30 years, from 1990 to 2021.

Neurosarcoidosis - the Role of Magnetic Resonance Imaging in Diagnostics.

Background: Sarcoidosis is a multisystem granulomatous disease of unknown etiology, characterized by presence of granulomas in affected tissues with variety in clinical presentations and presents a differential diagnostic and therapeutic dilemma. Clinical presentation of neurosarcoidosis is very variable. Diagnosis is based on clinical and radiological criteria and histological findings of disseminated non-necrotic granuloma followed by negative cultures for bacteria and fungi.

Differentiating neurosarcoidosis from multiple sclerosis using combined analysis of basic CSF parameters and MRZ reaction.

Background: Neurosarcodosis is one of the most frequent differential diagnoses of multiple sclerosis (MS) and requires central nervous system (CNS) biopsy to establish definite diagnosis according to the latest consensus diagnostic criteria. We here analyzed diagnostic values of basic cerebrospinal fluid (CSF) parameters to distinguish neurosarcoidosis from MS without CNS biopsy.

Methods: We retrospectively assessed clinical, radiological and laboratory data of 27 patients with neurosarcoidosis treated at our center and compared following CSF parameters with those of 138 patients with relapsing-remitting MS: CSF white cell count (WCC), CSF/serum albumin quotient (Q), intrathecal production of immunoglobulins including oligoclonal bands (OCB), MRZ reaction, defined as a polyspecific intrathecal production of IgG reactive against ≥2 of 3 the viruses measles (M), rubella (R), and zoster (Z) virus, and CSF lactate levels.

Neuroimmunological Disorders: The Gender Effect.

Multiple sclerosis is a disease that tends to affect women during their childbearing years. Although relapse risk decreases during pregnancy, patients should still be optimized on disease-modifying therapy before and after pregnancy to minimize gaps in treatment. Exclusive breastfeeding may reduce the chances of disease relapse postpartum, and many disease-modifying therapies are considered to be safe while breastfeeding.

No evidence of oligoclonal bands, intrathecal immunoglobulin synthesis and B cell recruitment in acute ischemic stroke.

Background: Within the past 10 years, immune mechanisms associated with acute ischemic stroke (AIS) have been brought into focus, but data on B cell activation and intrathecal Ig production is still scarce. In this study, we determined the prevalence of an elevated IgG index, positive oligoclonal bands (OCBs) and chemokine C-X-C motif ligand 13 (CXCL13) levels in the cerebrospinal fluid (CSF) as markers of intrathecal IgG synthesis and B cell activation in patients with AIS.

Methods: In a retrospective study we analyzed the cerebrospinal fluid (CSF) from 212 patients with AIS from December 2013 to May 2018 assessing intrathecal Ig synthesis, OCBs and CXCL13 concentrations.

Isolated Neurosarcoidosis Presenting as Chronic Progressive Pachymeningitis.

Neurologic manifestations of sarcoidosis are rare, and even rarer still are cases of isolated neurosarcoidosis. The clinical presentation of isolated neurosarcoidosis can be highly variable, and diagnosis is particularly challenging, the gold standard being tissue biopsy. We describe a patient with a history of atypical parkinsonian syndrome and chronic right frontal lobe infarct who developed weakness, imbalance, and gait disequilibrium in 2008, with magnetic resonance imaging at that time showing leptomeningeal and nodular enhancements in the bilateral frontal and parietal lobes.

Immune-Mediated Hypertrophic Pachymeningitis and its Mimickers: Magnetic Resonance Imaging Findings.

Hypertrophic pachymeningitis (HP) is a rare and chronic inflammatory disorder presenting as localized or diffuse thickening of the dura mater. It can be idiopathic or an unusual manifestation of immune-mediated, infectious, and neoplastic conditions. Although some cases may remain asymptomatic, HP can lead to progressive headaches, cranial nerve palsies, hydrocephalus, and other neurological complications, which makes its recognition a fundamental step for prompt treatment.

Minor Salivary Gland Biopsy for the Diagnosis of Neurosarcoidosis.

Introduction: The definite diagnosis of neurosarcoidosis is challenging since it requires a compatible histology of the nervous system. When neurosarcoidosis is suspected, other systemic manifestations are investigated to confirm the diagnosis. A minor salivary gland biopsy (MSGB) is often performed since it is minimally invasive.