Radiological features of patients with headache as a presenting symptom of neurosarcoidosis.

OBJECTIVE: To describe the radiological features of patients with headache as a presenting symptom of neurosarcoidosis. BACKGROUND: Neurologic complications occur in approximately 5%-10% of patients with sarcoidosis, and approximately 50% of these patients have neurologic deficits at the time sarcoidosis is first diagnosed. A wide spectrum of central and peripheral nervous system clinical manifestations may be observed, including cranial nerve palsies, sensory and/or motor deficits, and headache. Magnetic resonance imaging (MRI) results in patients with neurosarcoidosis may include abnormal contrast enhancement, structural masses, and demyelinating lesions. METHODS: This single-center retrospective cohort study assessed patients who were diagnosed with neurosarcoidosis in an urban tertiary care center between 1995 and 2016. We included patients who had MRI results at the time of diagnosis. Patients were divided into two groups based on the presence or absence of headache as a presenting symptom. The MRI result of meningeal contrast enhancement was reviewed. RESULTS: Of the 110 patients analyzed, 30 (27.3%) had an initial presenting symptom of headache while 80 (72.7%) did not. Patients with headache had a higher proportion of meningeal contrast enhancement on MRI (66.7% [20/30] vs. 25.0% [20/80]; p < 0.001) and leptomeningeal involvement (53.3% [16/30] vs. 7.5% [6/80], p < 0.001) compared to patients with no headache. However, those with headache had a lower proportion of spinal cord localization (13.8% [4/29] vs. 34.2% [26/76], p = 0.038) and intraparenchymal central nervous system involvement (16.7% [5/30] vs. 51.3% [41/80], p = 0.001) compared to patients with no headache. CONCLUSION: Patients with neurosarcoidosis who presented with headache as an initial symptom had a higher proportion of meningeal contrast enhancement seen by MRI than patients who presented with other neurological symptoms. This suggests a clinico-radiologic link between headache and meningeal disruption in patients with neurosarcoidosis.

Neurosarcoidosis involving cervical nerve root with unusual MRI findings: a case report and systematic literature review.

BACKGROUND: Neurosarcoidosis is rare, and among its manifestations, nerve root involvement has been reported in only a few cases. Therefore, magnetic resonance imaging (MRI) findings of neurosarcoidosis, particularly those involving nerve roots, are scarce in the literature. METHODS: We presented the case of neurosarcoidosis involving cervical nerve roots and cranial nerves, alongside a systematic literature review. RESULTS: A 28-year-old female suddenly developed right facial numbness as well as left upper extremity and left hand pain. Initial brain and spine MRI showed a bulging mass of T2 iso-to-high signal intensity in the left Meckel's cave/trigeminal nerve, as well as diffuse enlargement of the right C6 and C7 nerve roots. Follow-up MRI at 2 months revealed a reduction in the size of the initial lesion and the appearance of new similar lesions on the contralateral side (right Meckel's cave, left C3-C8 nerve roots). In particular, the lesions involving the nerve roots demonstrated central enlargement along the nerve roots, without involvement of the adjacent spinal cord. All these lesions exhibited enhancement, leading to the differentiation between sarcoidosis and lymphoma. Sarcoidosis was subsequently confirmed through biopsy of a hilar lymph node. CONCLUSIONS: This report presents a distinctive MRI feature of neurosarcoidosis involving spinal nerve roots, representing the first of its kind, and describes the evolution of MRI findings throughout the clinical course.

Specific Cutaneous Lesions in Patients With Neurosarcoidosis. / Lesiones cutáneas específicas en pacientes con neurosarcoidosis.

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). Twenty types of specific cutaneous lesions were observed: maculopapular lesions (n=6), plaques (n=9), lupus pernio (n=1), and scar sarcoidosis (n=4). These last lesions coexisted with maculopapular lesions in 2 patients and plaques in another 2. Specific cutaneous lesions were present at diagnosis of neurosarcoidosis in 13 patients. Recognition of specific cutaneous lesions in a patient with suspected neurosarcoidosis is important as biopsy can accelerate diagnosis.

Primary perineuritis, a rare but treatable neuropathy: Review of perineurial anatomy, clinicopathological features, and differential diagnosis.

The perineurium surrounds each fascicle in peripheral nerves, forming part of the blood-nerve barrier. We describe its normal anatomy and function. "Perineuritis" refers to both a nonspecific histopathological finding and more specific clinicopathological entity, primary perineuritis (PP). Patients with PP are often assumed to have nonsystemic vasculitic neuropathy until nerve biopsy is performed. We systematically reviewed the literature on PP and developed a differential diagnosis for histopathologically defined perineuritis. We searched PubMed, Embase, Scopus, and Web of Science for "perineuritis." We identified 20 cases (11 M/9F) of PP: progressive, unexplained neuropathy with biopsy showing perineuritis without vasculitis or other known predisposing condition. Patients ranged in age from 18 to 75 (mean 53.7) y and had symptoms 2-24 (median 4.5) mo before diagnosis. Neuropathy was usually sensory-motor (15/20), painful (18/19), multifocal (16/20), and distal-predominant (16/17) with legs more affected than arms. Truncal numbness occurred in 6/17; 10/18 had elevated cerebrospinal fluid (CSF) protein. Electromyography (EMG) and nerve conduction studies (NCS) demonstrated primarily axonal changes. Nerve biopsies showed T-cell-predominant inflammation, widening, and fibrosis of perineurium; infiltrates in epineurium in 10/20 and endoneurium in 7/20; and non-uniform axonal degeneration. Six had epithelioid cells. 19/20 received corticosteroids, 8 with additional immunomodulators; 18/19 improved. Two patients did not respond to intravenous immunoglobulin (IVIg). At final follow-up, 13/16 patients had mild and 2/16 moderate disability; 1/16 died. Secondary causes of perineuritis include leprosy, vasculitis, neurosarcoidosis, neuroborreliosis, neurolymphomatosis, toxic oil syndrome, eosinophilia-myalgia syndrome, and rarer conditions. PP appears to be an immune-mediated, corticosteroid-responsive disorder. It mimics nonsystemic vasculitic neuropathy. Cases with epithelioid cells might represent peripheral nervous system (PNS)-restricted forms of sarcoidosis.

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. RECENT FINDINGS: Although new biomarkers are being studied, there are no reliable tests for neurosarcoidosis. Advances in serum testing and imaging have improved the diagnosis for key mimics of neurosarcoidosis in certain clinical scenarios, but biopsy remains an important method of differentiation. Key mimics of neurosarcoidosis in all cases include infections (tuberculosis, fungal), autoimmune disease (vasculitis, IgG4-related disease), and lymphoma. As neurosarcoidosis can affect any part of the nervous system, patients should have a unique differential diagnosis tailored to their clinical presentation. Although biopsy can assist with excluding mimics, diagnosis is ultimately clinical.

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. 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. Clinical presentations unlikely to be compatible with neurosarcoidosis were excluded. Positive cases of neurosarcoidosis were defined as definite, probable, and possible cases, according to the latest international neurosarcoidosis diagnostic criteria from the Neurosarcoidosis Consortium Consensus Group. RESULTS: A total of 529 patients underwent a MSGB for clinical manifestations compatible with neurosarcoidosis. Among the 13 who fulfilled the criteria for neurosarcoidosis, only one had a positive MSGB. The sensitivity of MSGB was 7.7% (95% CI [0.2-36.0%]) and the specificity was 100.0% (95% CI [99.3-100%]). CONCLUSION: Considering the low sensitivity of MSGB for the diagnosis of NS, MSGB should be performed in selected indications, including a suspicion of spinal cord sarcoidosis, or when there is a strong clinical, laboratory, and radiological suspicion of NS. MSGB should rather not be performed when the chest CT-scan does not show signs of pulmonary or lymph node sarcoidosis.

Coexistence of open-angle glaucoma and sarcoidosis-associated optic neuropathy.

BACKGROUND: In cases with advanced glaucomatous disc changes, further changes associated with other optic neuropathies cannot be easily identified. We present a case of preexisting open-angle glaucoma and concurrent involvement of sarcoidosis-associated optic neuropathy. CASE PRESENTATION: A 53-year-old man presented with gradual visual loss in his left eye, which began 1 year ago and accelerated 3 months ago. The best-corrected visual acuity in the right eye was 20/20 and counting fingers in the left. Intraocular pressures (IOP) were 12 mmHg in the right eye and 34 mmHg in the left. We diagnosed him with advanced open-angle glaucoma in the left eye based on the advanced glaucomatous cupping of the left optic disc. The IOP in the left eye dropped to 10 mmHg and was well controlled with antiglaucomatous medication; however, his left optic disc developed pallor 3 months after the treatment. The patient was revealed to be diagnosed with sarcoidosis a month ago and had been treated with systemic corticosteroids thereafter by a pulmonologist. Orbital magnetic resonance imaging revealed sarcoidosis-associated optic neuropathy in the left eye. Subsequently, optic neuropathy occurred in his right eye. CONCLUSIONS: In eyes with advanced glaucomatous disc change, detecting the coexistence of other optic neuropathies can be difficult. This report highlights the importance of careful ophthalmic examinations and investigation for etiologies of other optic neuropathies if non-glaucomatous changes are suspected even in eyes with advanced glaucoma.

Insights into neurosarcoidosis: an imaging perspective.

Neurosarcoidosis is a complex and multifaceted inflammatory disorder affecting the nervous system. The disease, characterized by non-caseating granulomas, primarily involves the central and peripheral nervous systems. Neuro-logical manifestations vary widely and can include cranial nerve palsies, meningeal involvement, parenchymal lesions, hydrocephalus, and more. Diagnosis remains challenging due to the lack of specific imaging features, necessitating a combination of clinical evaluation, cerebrospinal fluid analysis, imaging studies, and sometimes tissue biopsy. This review article underscores the importance of the identification of various imaging features to mitigate the mortality and morbidity associated with neurosarcoidosis.

Clinical characteristics and outcome of neurosarcoidosis-associated myelitis: A retrospective cohort study and review of the literature.

BACKGROUND AND PURPOSE: Neurosarcoidosis can affect all parts of the nervous system of which myelitis is relatively frequent. The aim of this study was to describe clinical characteristics, treatment and prognosis of patients with myelitis attributable to neurosarcoidosis. METHODS: We performed a retrospective cohort study and a systematic review and meta-analysis of neurosarcoidosis-associated myelitis. RESULTS: Myelitis was identified in 41 of 153 (27%) neurosarcoidosis patients seen at our clinic from 2015 to 2020. Classification of neurosarcoidosis was definite in three (7%), probable in 29 (71%) and possible in nine patients (22%). The median (interquartile range) age at onset was 49 (41-53) years and 20 of the patients were female (49%). The presenting symptoms included muscle weakness in 31 of 41 patients (78%), sensory loss in 35 (88%) and micturition abnormalities in 30 (75%). Spinal magnetic resonance imaging showed longitudinally extensive myelitis in 27 of 36 patients (75%) and cerebrospinal fluid examination showed an elevated leukocyte count in 21 patients (81%). Initial treatment consisted of glucocorticoids in 38 of 41 patients (93%), with additional methotrexate or azathioprine in 21 of 41 patients (51%) and infliximab in 10 of 41 patients (24%). Treatment led to remission, improvement or stabilization of disease in 37 of 39 patients (95%). Despite treatment, 18 of 30 patients (60%) could not walk independently at the end of follow-up (median 36 months). A review of the literature published between 2000 and 2020 identified 215 patients with comparable clinical characteristics and results of ancillary investigations. CONCLUSION: Sarcoidosis-associated myelitis is observed in 27% of neurosarcoidosis patients. Although treatment often led to a decrease in disease activity, residual neurological deficits leading to loss of ambulation occurred frequently.

Neurosarcoidosis: Phenotypes, Approach to Diagnosis and Treatment.

PURPOSE OF REVIEW: The aim of this review is to provide an update of clinical presentation, diagnosis, differential diagnoses, and treatment according to recent evidence. RECENT FINDINGS: Neurosarcoidosis remains a diagnosis of exclusion, with infectious and malignant etiologies recognized as important mimickers. Corticosteroids remain as first-line therapy. In recent years, however, studies have demonstrated the effectiveness of anti-tumor necrosis factor (anti-TNF) therapy in the treatment of neurosarcoidosis, leading to improved outcomes. Neurosarcoidosis is a granulomatous disease with protean manifestations that may affect any part of the central and peripheral nervous system. It has many mimickers, and potentially devastating complications necessitating long-term follow-up. Early initiation of treatment, particularly with anti-TNF therapy, may lead to better outcomes and fewer relapses. There is an unmet need for randomized controlled trials that provide robust data to guide therapy and the long-term management of neurosarcoidosis patients.

Granulomatous Diseases of the Central Nervous System.

PURPOSE OF REVIEW: To discuss the pathophysiology, key clinical features, necessary diagnostic evaluation, and current treatment regimens for granulomatous diseases of the central nervous system. RECENT FINDINGS: The diagnosis and management of granulomatous disease of the central nervous system has been revolutionized by advances in diagnostic imaging. Nevertheless, tissue and/or cerebrospinal fluid (CSF) sampling remains necessary to establish the diagnosis in most cases. Establishing a specific diagnosis is critical because treatment selection needs to focus on the granulomatous process centering on either antibiotic or immunosuppressive agents. Particular for non-infectious granulomatous disease more aggressive immunotherapies may help in clinical outcome. There are multiple non-infectious and infectious etiologies for granulomatous disease of the central nervous system. Clinical manifestations result from local structural invasion of granulomas or granulomatous inflammation of the blood vessels and meninges. Rapid diagnosis and specific treatment is essential.

Discussing Challenges in Diagnosis of Tuberculous Meningitis and Neurosarcoidosis.

Isolated chronic granulomatous meningitis remains a diagnostic challenge for the physician. Symptoms are often nonspecific and ancillary tests have low-sensitivity rates, which may delay targeted treatment and lead to increased morbidity and mortality. Here, we discuss the challenges in diagnosing and treating patients with chronic meningitis by reporting two cases of previously healthy patients who presented with granulomatous meningitis on brain biopsy.

[Unusual forms of inflammatory diseases of the central nervous system]. / Seltene Formen der entzündlichen Erkrankungen des Zentralnervensystems.

BACKGROUND: White matter lesions of the central nervous system (CNS) are frequently encountered on magnetic resonance imaging (MRI) exams. If the morphologic findings, clinical symptoms and laboratory results are not typical for one of the more common inflammatory CNS diseases, the diagnosis may become challenging, which also means that interesting and sometime emotional discussions may arise. OBJECTIVE: While frequent causes of inflammatory CNS diseases were already discussed in a previous article, we now focus on more seldom forms and place attention on morphologic characteristics which may help to find the correct diagnosis.

PATHOLOGICALLY-CONFIRMED ISOLATED HYPOTHALAMO-PITUITARY SARCOIDOSIS REFRACTORY TO PULSE-DOSE GLUCOCORTICOIDS AND SUCCESSFULLY TREATED WITH METHOTREXATE.

Isolated sarcoidosis of the hypothalamic-pituitary system is a very rare form of neurosarcoidosis. A high index of suspicion is required for diagnosis and the choice of therapy embodies another challenge due to lack of standardized protocols. Glucocorticoids are the mainstay of initial treatment, whereas the second and third-line therapy include immunomodulators and cytotoxic drugs, in addition to monoclonal antibodies. This report presents an unusual case of panhypopituitarism in a 32-year-old previously healthy male patient due to isolated hypothalamo-pituitary sarcoidosis confirmed histologically, refractory to pulse-dose glucocorticoids and then successfully treated by methotrexate. Based on our report, in patients requiring additional therapy usage of the methotrexate as the second line agent should be considered, however the time frame and the dosing schedule of methotrexate are still unknown and deserve further investigation.

Immune-Mediated Disorders Affecting the Spinal Cord and the Spine.

PURPOSE OF REVIEW: To review the most recent advances and provide a description of the most common autoimmune diseases causing myelitis and selective spine disorders. The ultimate goal of this article is to facilitate the prompt recognition of these diseases. RECENT FINDINGS: The recent discovery of biomarkers such as aquaporin 4 (AQP4) and myelin oligodendrocyte glycoprotein (MOG) antibodies has changed our understanding of autoimmune diseases affecting the spinal cord as well as their treatment and outcomes. Autoimmune neurology is an increasingly evolving field that encompasses a broad spectrum of autoimmune-inflammatory diseases of the central nervous system (CNS) and peripheral nervous system (PNS). Autoimmune disorders of the spinal cord are a heterogeneous group of myelopathies with a broad differential diagnosis and many of them have been recently identified. Prompt recognition of these myelopathies is important as some of them are treatable, which could improve patient outcomes and prevent disability.

UNUSUAL CLINICAL COURSE OF NEUROSARCOIDOSIS MANIFESTED WITH ACUTE HYDROCEPHALUS.

Approximately 5% to 15% of patients with systemic sarcoidosis develop neurological complications. However, the actual prevalence of subclinical disease may be higher. Symptoms are not specific, and may resemble those of other neurological diseases. Hydrocephalus occurs in 6% of patients with neurosarcoidosis. Acute hydrocephalus is extremely rare and when it occurs, it is usually difficult to diagnose, thus leading to possible complications. We present a patient who developed acute hydrocephalus due to neurosarcoidosis, for which he had to be operated on; soon after the operation, cranial infection developed that required definitive drainage system and ventriculoperitoneal shunt had to be implanted. In further complicated clinical course, after four years on corticosteroid therapy (corticosteroid dependent sarcoidosis), he had to be urgently operated on because of significant ventricular catheter adhesions, but several days after the operation he died in coma because of progressive brain edema not responding to treatment. As hydrocephalus due to neurosarcoidosis has high morbidity and mortality, early diagnosis and proper treatment are of utmost importance.

Sarcoidosis of the pineal region, dorsal mesencephalon, and periaqueductal region: Case report and systematic review.

BACKGROUND: The central nervous system is an unusual location of sarcoidosis, which commonly affects the cranial nerves, meninges, hypothalamus, and pituitary gland. Involvement of the pineal region is extremely rare. This systematic review focused on the diagnosis and management of pineal region sarcoidosis, dorsal mesencephalon, and periaqueductal region. OBJECTIVES: This study aimed to discuss diagnostic modalities and best management tools of the aforementioned pathology. METHODS: ScienceDirect, PubMed, and Google Scholar databases were searched for English or French articles about sarcoidosis of the pineal region, dorsal mesencephalon, and periaqueductal region. The clinical case of a patient managed at our department that we believe is directly relevant to this review is also presented. Patients' demographics, clinical presentations, presence of hydrocephalus, other sarcoidosis locations in the central nervous system, and medical treatment were collected. Surgical management, surgical approach, and outcomes and complications of each procedure were also obtained. This study was conducted in agreement with the Preferred Reporting Items for Systematic Reviews and Meta-Analyses statement. RESULTS: Fifteen cases were examined. The study sample consisted of nine (60%) male and six (40%) female, and the mean age was 32 years. Eight (53%) patients had hydrocephalus, and the predominant clinical presentations were signs of increasing intracranial pressure (headaches, vomiting, and papilledema). Six (40%) patients had diplopia, and convergence-retraction nystagmus was noted in three (20%) patients. Argyll Robertson sign was present in one patient and suspected in another patient (13%). Medical treatment consisted mainly of steroids (93% of cases). Open surgery on the pineal region was performed in five patients, and four of them reported to have serious complications (such as ophthalmoplegia, hemianopsia, hemiparesis, bilateral third cranial nerve paresis, and cerebellar syndrome). Endoscopic management was performed in two patients without complications. CONCLUSION: To treat hydrocephalus, brain imaging is mandatory in patients with sarcoidosis if intracranial hypertension is suspected. In pineal region sarcoidosis, management of hydrocephalus is the priority, followed by medical treatment of the lesion. Open surgery of any approach presents a high risk of complications; thus, an endoscopic approach is the preferred management, as it treats hydrocephalus and makes biopsy possible with minimal risk.

Neurosarcoidosis presenting as CRVO combined CRAO: a biopsy-proven case report of a Chinese patient.

BACKGROUND: Neurosarcoidosis is a rare systemic disorder that can affect the eye and other organs, including the central nervous system. Neurosarcoidosis infiltrating the optic nerve presenting as central retinal vein occlusion combined with artery ischaemia has not been reported in the literature previously. We describe a Chinese patient presenting with acute monocular vision loss, in whom an optic nerve biopsy confirmed the diagnosis of neurosarcoidosis. CASE PRESENTATION: A 47-year-old woman complained of acute decreased vision in her left eye over the course of 1 month. She reported that her vision deteriorated quickly within first 3 days of consulting an ophthalmologist at a local hospital. She was diagnosed with central retinal vein occlusion after funduscopic examination and fundus fluorescein angiography, and the vision in her left eye further deteriorated to no light perception. An orbital magnetic resonance imaging showed an abnormal T1-weighted image of the optic nerve after contrast enhancement. She was referred to a neuro-ophthalmologist for further evaluation. After routine blood tests ruled out infectious and metastatic diseases, she was prescribed 500 mg/d methylprednisolone for 5 days, but her vision did not improve. As she could still not perceive light, an optic nerve biopsy was performed, and the histopathology revealed non-necrotising granuloma that was consistent with neurosarcoidosis. CONCLUSIONS: Isolated optic nerve infiltration by neurosarcoidosis without the involvement of the central nervous system or other systemic organs is challenging to diagnose. Biopsy of the optic nerve sheath is crucial for the final diagnosis of neurosarcoidosis. Therefore, a comprehensive ophthalmologic and systemic examination and work-up for inflammation of the eye, chest, and central nervous system should be conducted for atypical cases.

The eclipse sign as a radiological presentation of neurosarcoidosis.

Neurosarcoidosis is a rare inflammatory neurological condition. We describe a challenging radiological presentation of neurosarcoidosis. The eclipse sign refers to hypo T1-weighted parenchymal or leptomeningeal images surrounded by circular gadolinium enhancement. The eclipse sign was identified in 3 out of 46 patients with histologically-proven neurosarcoidosis. The eclipse sign may correspond to necrotizing parenchymal or leptomeningeal granuloma. This sign expands the spectrum of radiological presentations of neurosarcoidosis.

Neurosarcoidosis As a Rare Differential Diagnosis for Single Or Multiple Lesions of the Nervous System.

Objective: Sarcoidosis is a multisystemic granulomatous disease of unknown cause which affects the lung or bilateral hilar lymphadenopathy in over 90% of the cases. Neurosarcoidosis (NS) is rare and accounts for approximately 5 - 15% of the cases. Involvement of all parts of the central and peripheral nervous system is possible with various clinical symptoms, e. g. seizures, hydrocephalus, optic/facial nerve palsy or hearing loss.Methods: We screened the neuropathological data bases and the medical records of two neurosurgical university hospitals for cases of NS. All these cases had been verified by surgical biopsy. We retrospectively evaluated the patient's records with special regard to the histopathology reports and specific clinical symptoms.Results: We identified 9 cases of NS between 1994 and 2014 (3 female, 6 male patients). The average age at the time of diagnosis of NS was 41,4 years. Various clinical symptoms like hydrocephalus (n = 3), seizures (n = 1), meningitis (n = 1), optical nerve involvment with vision disorder (n = 1), myelitis with paraplegia (n = 1), mastoiditis with hearing loss (n = 1), back pain syndrome (n = 2) were present. 7 patients were treated with corticosteroids, 1 patient with cyclophosphamide and 1 with a combination of corticosteroids and methotrexate.Conclusion: NS is a rare but potentially life-threatening disease. It is difficult to distinguish sarcoidosis from other granulomatous diseases, infectious diseases like tuberculosis, multiple sclerosis or neoplasm. For a definite diagnosis, a neurosurgical biopsy with histological evidence of noncaseating epithelioid cell granulomas is required, followed by multidisciplinary treatment.