Neurosarcoidosis

BACKGROUND: Sarcoidosis, a multisystem inflammatory non-caseating granulomatous disease, can present with neurologic lesions in up to 10% of patients. CASE DESCRIPTION: A 57-year-old male presented with three months of worsening upper extremity radicular pain associated with dysmetria, hyperreflexia, bilateral Hoffman's, and positive Babinski signs. The contrast magnetic resonance imaging (MRI) showed a diffuse T2 signal hyperintensity and T1-enhancing 2.5 cm lesion extending sagittally between C4 and C6...

Sarcoidosis is a chronic granulomatous disease of unknown cause characterized by the presence of non-caseating granulomas. The disease can affect any organ including the nervous system. Neurosarcoidosis occurs in about 5% patients with sarcoidosis. The clinical presentation of neurosarcoidosis is varied, and it can involve the brain, spinal cord and peripheral nervous system, separately or in different combinations. The diagnosis of neurosarcoidosis is challenging, as biopsies from the nervous system are not readily available...

BACKGROUND: Neurosarcoidosis (NS) is a challenging diagnosis for clinicians and pathologists. NS most often presents with leptomeningeal involvement where it mimics infectious or neoplastic meningitis, and in up to half of cases, systemic signs of sarcoidosis are lacking. Rare presentations include dural-based mass(es) (mimicking meningioma), hypothalamic/sellar-based lesions (mimicking pituitary adenoma), or as myelopathy (mimicking tumor or neurodegenerative condition). For pathologists, the morphological effects of prior therapy are not well documented...

Sarcoidosis is a multisystem granulomatous disorder of unknown etiology characterized by non-caseating granulomas in involved organs. Approximately 10% of patients with sarcoidosis exhibit central nervous system involvement. However, the occurrence of isolated neurosarcoidosis without concurrent systemic signs is very rare, affecting less than 1% of patients. We report a case of isolated neurosarcoidosis in a previously healthy patient who initially presented with a single episode of seizure and loss of consciousness...

Sarcoidosis is a granulomatous inflammatory disease of uncertain cause. It occurs most commonly in young and middle-aged adults and less frequently in children; therefore, few data on pediatric sarcoidosis exist in the literature. The diagnosis and management of sarcoidosis remain challenging because of diverse and often nonspecific clinical and imaging findings. In addition, the clinical picture varies widely by age. Prepubertal and adolescent patients often present with adult-like pulmonary disease; however, early-onset sarcoidosis is typically characterized by the triad of arthritis, uveitis, and skin rash...

Sarcoidosis is a granulomatous disease of indeterminate etiology. Women are more commonly affected than men at nearly twice the incidence with black women most commonly afflicted in the United States. Osseous spinal sarcoidosis (SS) is thought to be uncommon. Such lesions are often mistaken for metastatic disease, multiple myeloma, or disseminated fungal/granulomatous infection complicating the diagnosis, clinical course, and treatment. Patients presenting with clinical and imaging features of sarcoidosis may have normal serum laboratory values further complicating diagnosis...

Sarcoidosis is a rare, chronic, granulomatous disease of unknown etiology and primarily effects the lymphatic and respiratory systems. The central nervous system (CNS) is unusually implicated in sarcoidosis patients. We describe a rare magnetic resonance imaging (MRI) findings in a case of isolated neurosarcoidosis. The evaluation of suspect patients requires radiological imaging studies, especially MRIs. The diagnosis of neurosarcoidosis is clinically challenging, MRI studies are effective in detecting CNS inflammation but lack specificity...

Neurosarcoidosis (NS) is a protean illness with multiple clinical and radiological presentations giving it the moniker of "a chameleon" or the great mimic. NS can present as a wide spectrum of neurological syndromes localizing both to the central and peripheral nervous systems. The absence of a diagnostic serum test makes it difficult to diagnose with certainty and remains largely a histopathological diagnosis and one of exclusion. A high index of suspicion should be there in suspecting NS, and it should always be excluded among patients presenting with acute to subacute neurological deficits...

Sarcoidosis is defined as an immune-mediated multi-organ granulomatous disease with unknown etiology, which is characterized by the presence of multiple non-caseating granulomas in the absence of a definite infective or toxic cause. Neurosarcoidosis (NS) occurs when sarcoid granulomas invade the central or peripheral nervous systems. Sarcoidosis usually presents with non-specific manifestations, including dry cough, fatigue, night sweats, weight loss, skin changes, and eye manifestations. Many patients who develop NS present with neurological manifestations within two years of being diagnosed with sarcoidosis...

OBJECTIVES: To evaluate the clinical outcomes and relapse rates in neurosarcoidosis patients administered infliximab. METHODS: A systematic review was conducted using the MEDLINE, EMBASE, SCOPUS, and Cochrane Library databases. The search included studies from their inception to March 2023. We included case-series studies with at least 10 neurosarcoidosis patients undergoing any treatment type. Studies were also required to report at least one of the following outcomes: response rate, overall survival rate, or relapse rate...

Sarcoidosis affects the nervous system in 5% of cases. 60% of cases involve the cranial and peripheral nerves, the remainder the central nervous system, in which a leptomeningitis, a pachymeningitis and a vasculitis may arise. Stroke and cerebral haemorrhage may occur, and certain infections in the brain are more likely with sarcoidosis. Patients respond well to treatment but oftentimes with residual neurological impairments which may be severe. A greater understanding of the disease and the need for early treatment and use of biological therapies have improved treatment outcome in recent times...

Neurosarcoidosis is an uncommon but potentially serious disease of the central nervous system that can cause major sequelae. We analyzed the presence and diagnostic usefulness of specific cutaneous lesions in 58 patients with neurosarcoidosis. Sixteen patients (27.6%) had specific cutaneous lesions (14 men and 2 women; mean age, 50 years [range, 20-84 years]). Twenty-four types of neurological lesions were observed: cranial neuropathy (n=7), parenchymal lesions (n=4), meningeal lesions (n=3), myelopathy (n=3), pituitary lesions (n=1), hydrocephalus (n=2), and peripheral neuropathy (n=4)...

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. The objective of the present study was to assess its performance for the diagnosis of neurosarcoidosis. METHODS: A retrospective single-center study included patients who underwent a MSGB in a tertiary neurological university hospital (Lyon, France) between 2015 and 2018...

Neurosarcoidosis affects 5-26% of patients with systemic sarcoidosis and can be the first or only manifestation of the disease. Neurosarcoidosis can affect any part of the nervous system with heterogeneous clinical manifestations and imaging appearances that overlap with many infectious, inflammatory, and neoplastic disorders, making its diagnosis challenging. In the absence of a reliable biomarker to confirm neurosarcoidosis, the diagnosis is based on identifying a compatible clinical and imaging profile and identifying pathological evidence of non-caseating granulomas by biopsy of other organs or, if needed, in the nervous system, with the exclusion of other causes of granulomatous disease and possible neuroinfectious and neuroinflammatory disorder mimics...

Neurosarcoidosis (NS) is a mimicker of many infectious, neoplastic, and inflammatory diseases. It most commonly involves the cranial nerves followed by meninges, ventricles, hypothalamic-pituitary axis, spinal cord, and brainstem/cerebellum. While NS myelopathy has been increasingly recognized, pathophysiological/prognostic and management principles in NS-mediated cauda equina (CE) and conus medullaris (CM) syndromes, which constitute a small and rare minority of this subset, remain elusive. We present the case of a 49 -year-old Hispanic man who developed a peripheral facial palsy and primary hypogonadism within a span of 12 months and eventually got diagnosed with NS after he presented with CE syndrome...

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PURPOSE OF REVIEW: Sarcoidosis is a granulomatous systemic disease of unknown cause where the lung is the most frequently affected organ. Therapeutic management of the disease is challenging as clinical presentation and prognosis are very heterogeneous. In the present review, we will summarize the main advances in sarcoidosis therapy. RECENT FINDINGS: Current sarcoidosis therapies are categorized in three lines: glucocorticoids (first line), immunosuppressants (second line), and biologics (third line)...

AIM: To assess the prevalence of various imaging manifestations in neurosarcoidosis (NS) patients at presentation and to explore if specific imaging findings may cluster in different sub-groups. MATERIALS AND METHODS: A retrospective, dual-institution, systematic imaging review was undertaken of the magnetic resonance imaging (MRI) findings in 100 consecutive NS patients who presented over a 15-year period. Clustering analysis (k-mode) was performed to evaluate co-occurrence of imaging findings...

OBJECTIVE: To assess the efficacy and risks of treatment with infliximab (anti-tumor necrosis factor alpha) in pathology-confirmed neurosarcoidosis. METHODS: In a retrospective study in 2 tertiary referral centers in the Netherlands, we analyzed clinical characteristics, complications, and outcome of patients with neurosarcoidosis treated with infliximab. RESULTS: Twenty-eight patients were identified with a mean age of 42 years. Neurosarcoidosis presented with a cerebral parenchymal localization in 16 (59%), pituitary gland/hypothalamic sarcoidosis in 15 (54%), peripheral nerve involvement in 12 (43%), and chronic meningitis in 11 patients (41%)...

BACKGROUND: Nervous system is affected in 25% of patients with sarcoidosis. Current literature is largely limited to case reports with disproportionate Caucasian population. We aim to evaluate differences in presentation, management and outcomes by race in neurosarcoidosis. METHODS: Clinical and demographic data on consecutive patients fulfilling Zajicek criteria for neurosarcoidosis from 1995 to 2016 at Henry Ford Hospital were extracted. Disparities in clinical presentation, laboratory values, radiological features, treatment and outcomes, were compared between two groups: African Americans (AA) and non-AA using chi-squared tests, two sample t-test for age and Wilcoxon two sample tests...