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Brain Nerve 2022 May;74(5):455-461

Department of Neurology, Yamaguchi Prefectural Grand Medical Center.

Sarcoidosis is a granulomatous multiorgan disease of unknown etiology that commonly affects the respiratory system, eyes, and skin, and less commonly affects the nervous system. Because of its rarity, a standard treatment for central nervous system (CNS) sarcoidosis has not yet been established. Corticosteroids remain the cornerstone of CNS sarcoidosis treatment. Read More

Brain Nerve 2022 May;74(5):449-454

Department of Neurology, Tohoku University Graduate School of Medicine.

Sarcoidosis is a systemic non-caseating granulomatous disease of unknown origin, and involvement of the nervous system may result in irreversible neurological deficits. Corticosteroids (CSs) are commonly used as first-line agents for neurosarcoidosis. In steroid-refractory patients, immunosuppressants (ISs) have been used as second-line agents, and tumor necrosis factor-alpha (TNF-alpha) inhibitors as third-line agents. Read More

J Neurol 2022 May 18. Epub 2022 May 18.

Department of Neurology, Emory University School of Medicine, 12 Executive Park Drive NE, Atlanta, GA, 30329, USA.

Background: Facial nerve palsy is a cardinal manifestation of neurosarcoidosis, but dedicated studies of this disease feature have not been conducted. We sought to clarify the impact of facial palsy on the diagnosis of neurosarcoidosis, its subsequent clinicoradiographic evolution, and eventual treatment decisions.

Methods: A single-center retrospective analysis of patients with neurosarcoidosis and facial palsy was conducted over the preceding 10 years (01/01/2011-08/12/2021). Read More

BMJ Case Rep 2022 May 17;15(5). Epub 2022 May 17.

Neurology, Meitra Hospital, Calicut, India.

We present a unique case of biopsy-proven necrotising sarcoidosis involving the central nervous system in a man in his 40s. The patient presented with a 2-week history of right-sided headache and diplopia. He had right trochlear and abducens nerve palsy, sensory blunting over V1 and V2 segment of right trigeminal sensory nerve and right sensory neural hearing loss. Read More

J Neuroimmunol 2022 Apr 18;368:577871. Epub 2022 Apr 18.

Johns Hopkins University, Department of Neurology, Baltimore, MD, United States of America. Electronic address:

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. Read More

Respir Med Case Rep 2022 4;37:101647. Epub 2022 Apr 4.

Maimonides Medical Center, USA.

Sarcoidosis is a systemic inflammatory disease of unknown etiology with a myriad of clinical presentations depending on the organ systems involved. Neurosarcoidosis is an uncommon entity which is characterized by non-caseating granuloma infiltration of the central nervous system. Dysphagia in sarcoidosis is even more uncommon, and can involve one or more pathophysiological mechanisms: central nervous system involvement (cranial nerves associated with swallowing), lower motor neuron involvement (invasion of the enteric nervous plexus), direct muscle infiltration (invasion of the skeletal muscle portion of the esophagus and posterior pharynx), or mechanical obstruction (extrinsic compression by mediastinal lymph nodes). Read More

Neurol Neuroimmunol Neuroinflamm 2022 Jul 29;9(4). Epub 2022 Apr 29.

From the Neurology Residency Program (G.A.B.), and Division of Neuromuscular Medicine (R.G.-S.), Department of Neurology, Division of Neuroradiology (A.J.C.), Department of Radiology, and Division of Hospital Neurology (S.K.H.), Department of Neurology, Emory University School of Medicine, Atlanta, GA.

Background And Objectives: Sarcoidosis is a multisystem granulomatous disease affecting the nervous system in 3%-5% of cases. It can affect almost any component of the nervous system. Involvement of the cauda equina is an understudied phenotype, and questions remain regarding its natural history and optimal approach to management. Read More

J Neuroophthalmol 2022 Apr 27. Epub 2022 Apr 27.

Department of Neurology, NYU Grossman School of Medicine, New York, New York.

NMC Case Rep J 2022 4;9:31-35. Epub 2022 Mar 4.

Department of Neurosurgery, Graduate School of Biomedical and Health Sciences, Hiroshima University, Hiroshima, Hiroshima, Japan.

Because of nonspecific clinical and radiological findings, it is difficult to diagnose isolated neurosarcoidosis without histological examination. Distinguishing neurosarcoidosis from neoplasm, infectious disease, or granulomatous disease can be challenging. In this study, we present a case of a 61-year-old female who presented with unilateral blindness. Read More

Neurohospitalist 2022 Apr 7;12(2):290-294. Epub 2022 Feb 7.

Department of Internal Medicine and Clinical Immunology, CHU Bordeaux (Groupe Hospitalier Saint-André), University of Bordeaux, Bordeaux, France.

Neurological involvement occurs in 5 to 15% of patients with sarcoidosis. It rarely represents the sole manifestation of the disease, a condition called isolated neurosarcoidosis.

Objectives: To describe patients with definite isolated central neurosarcoidosis. Read More

J Neuroimmunol 2022 06 1;367:577860. Epub 2022 Apr 1.

Department of Medicine, Washington University School of Medicine, St. Louis, MO 63110, United States of America. Electronic address:

The tissue-specific drivers of neurosarcoidosis remain poorly defined. To identify cerebrospinal fluid (CSF) specific, antigen-driven T and B cell responses, we performed single-cell RNA sequencing of CSF and blood cells from neurosarcoid participants coupled to T and B cell receptor sequencing. In contrast to pulmonary sarcoidosis, which is driven by CD4 T cells, we found CD8 T cell clonal expansion enriched in the neurosarcoid CSF. Read More

Neurology 2022 Apr 4. Epub 2022 Apr 4.

Department of Neurology, University Hospital Carl Gustav Carus, Technische Universität Dresden, Dresden, Germany.

A 37-year-old patient with biopsy-proven neurosarcoidosis was admitted for a follow-up cranial MRI. Because of obstructive hydrocephalus at initial presentation, a ventriculoperitoneal shunt system had previously been implanted. Read More

J Neuroimmunol 2022 06 26;367:577849. Epub 2022 Mar 26.

Department of Neurology, Odense University Hospital, J.B. Winsloewsvej 4, 5000 Odense, Denmark; BRIDGE-Brain Research-Inter-Disciplinary Guided Excellence, Department of Clinical Research, University of Southern Denmark, J.B. Winsloewsvej 19, 5000 Odense, Denmark; Department of Neurobiology Research, Institute of Molecular Medicine, University of Southern Denmark, J.B. Winsloewsvej 21, 5000 Odense, Denmark.

Methods: Cerebrospinal fluid (CSF) and plasma levels of 38 biomarkers from 20 neurosarcoidosis (NS) patients were compared to healthy controls (HC).

Results: In CSF, 25 biomarkers were significantly elevated compared to HC: IFNγ, TNFα, TNFβ, IL-2, IL-6, IL-10, IL-12B, IL-15, IL-16, CCL2, CCL3, CCL4, CCL11, CCL13, CCL17, CCL22, CCL26, CXCL8, CXCL10, TNFR2, VEGF-A, PIGF, SAA, VCAM1, and ICAM1. In plasma, 12 biomarkers were significantly elevated compared to HC: IFNγ, TNFα, CCL2, CCL3, CCL4, CCL17, CXCL10, VEGFR1, PIGF, SAA, VCAM1, and ICAM1. Read More

Radiologe 2022 Apr 17;62(4):302-307. Epub 2022 Mar 17.

Klinik für Diagnostische und Interventionelle Neuroradiologie, Universitätsklinikum des Saarlandes, Kirrberger Str., Gebäude 90, 66421, Homburg/Saar, Deutschland.

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. Read More

Cureus 2022 Jan 28;14(1):e21687. Epub 2022 Jan 28.

Internal Medicine, Beaumont Health System, Royal Oak, USA.

Neurosarcoidosis is a rare manifestation of sarcoidosis that can exhibit a variety of neuropsychiatric symptoms and can present independently of pulmonary or other systemic symptoms. This is the case of a 51-year-old African American male who presented with recurrent episodes of auditory and visual hallucinations, confusion, seizures that did not respond to antiepileptics, and recent-onset primary polydipsia. In the emergency department, he did not have meningeal signs, focal neurologic deficits, or a fever. Read More

Eur J Neurol 2022 Jun 3;29(6):1763-1770. Epub 2022 Mar 3.

Department of Neurology, Amsterdam University Medical Center, University of Amsterdam, Amsterdam Neuroscience, Amsterdam, The Netherlands.

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. Read More

Front Immunol 2021 4;12:811351. Epub 2022 Feb 4.

Department of Pharmacology, University of Oxford, Oxford, United Kingdom.

Background: Inclusion of cerebrospinal fluid (CSF) oligoclonal IgG bands (OCGB) in the revised McDonald criteria increases the sensitivity of diagnosis when dissemination in time (DIT) cannot be proven. While OCGB negative patients are unlikely to develop clinically definite (CD) MS, OCGB positivity may lead to an erroneous diagnosis in conditions that present similarly, such as neuromyelitis optica spectrum disorders (NMOSD) or neurosarcoidosis.

Objective: To identify specific, OCGB-complementary, biomarkers to improve diagnostic accuracy in OCGB positive patients. Read More

Ugeskr Laeger 2022 02;184(7)

Neurologisk Afdeling, Aalborg Universitetshospital.

Neurosarcoidosis (NS) affects 5-10% of sarcoidosis patients and can involve any part of the nervous system. The prognosis can be serious and relapse is common. The diagnostic work-up is difficult with many differential diagnoses in inflammatory, infective and malignant neurological diseases. Read More

Neurology 2022 04 10;98(14):e1479-e1488. Epub 2022 Feb 10.

From the Service de Neurologie, Sclérose en Plaques, Pathologies de la Myéline et Neuro-inflammation (A.G., R.M.) and Service de Neuro-Oncologie (B.J.), Hôpital Neurologique Pierre-Wertheimer, Hospices Civils de Lyon, Bron; Service de Biostatistique-Bioinformatique (A.G.), Département de Médecine Interne et Immunologie Clinique, Hôpital Édouard Herriot (C.-A.D.), and Département de Médecine Interne, Hôpital de la Croix Rousse (P.S.), Hospices Civils de Lyon; Université Lyon 1 (A.G., B.J., R.M.), Université de Lyon, Villeurbanne; Pitié-Salpêtrière University Hospital (A.-C.D., C.C.-A., D.S.), Sorbonne Universités; Department of Internal Medicine and Clinical Immunology (A.-C.D., C.C.-A., D.S.), AP-HP; Centre de Référence des Maladies Auto-Immunes Systémiques Rares (A.-C.D., C.C.-A., D.S.), Centre de Référence des Maladies Auto-Inflammatoires et de l'Amylose Inflammatoire, Paris; Département de Médecine Interne et Maladies Systémiques (C.A., A.T., B.B.) and Département de Neurologie (T.M.), CHU Dijon; Département de Médecine Interne (E.B.), CH Saint Luc Saint Joseph, Lyon; Département de Médecine Interne (T.D.), CH Montluçon; Département de Médecine Interne (J.F.D.), Centre Hospitalier Fleyriat, Bourg-en-Bresse; Département de Médecine Interne, CHU Estaing (G.L.G., M.R.), and Service de Médecine Interne, Hôpital Gabriel Montpied (M.A.), CHU de Clermont-Ferrand; Département de Neurologie (J.-P.C., J.-C.G.A.), Hôpital Nord, CHU de Saint-Etienne; and Research on Healthcare Performance (RESHAPE) (P.S.), INSERM U1290, Université Claude Bernard Lyon 1, France.

Background And Objectives: Spinal cord sarcoidosis is a rare manifestation of sarcoidosis with a consequent risk of neurologic sequelae for the patient. We investigated prognostic factors and efficacy of immunosuppressive treatments in a longitudinal cohort.

Methods: We retrospectively studied patients with spinal cord sarcoidosis followed between 1995 and 2021 in 7 centers in France. Read More

Curr Neurol Neurosci Rep 2022 01 9;22(1):33-45. Epub 2022 Feb 9.

Department of Neurology, Stritch School of Medicine, Loyola University Chicago, 2160 South First Avenue Maguire Building Suite 2700, Maywood, IL, 60153, USA.

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. Read More

Neurol Sci 2022 04 6;43(4):2875-2877. Epub 2022 Feb 6.

Department of Neurological Surgery, University of Pittsburgh Medical Center, 200 Lothrop Street, Suite B-400, Pittsburgh, PA, 15213-2582, USA.

Am J Dermatopathol 2022 Feb;44(2):e19-e22

Department of Pathology and Medical Biology, Division of Pathology, University of Groningen, University Medical Center Groningen, Groningen, the Netherlands; and.

Abstract: Immunodeficiency-associated lymphoproliferative disorders are associated with latent infection by Epstein-Barr virus (EBV). Most cases of EBV-positive immunodeficiency-associated lymphoproliferative disorders arise from B cells, although some are of T-cell or natural killer origin. Cutaneous involvement is unusual and sporadically reported in the literature. Read More

Rheumatology (Oxford) 2022 Jan 18. Epub 2022 Jan 18.

Department of Pathology, Tama-Nambu Chiiki Hospital, Tokyo, Japan.

Breathe (Sheff) 2021 Dec;17(4):210069

Dept of Pulmonology, University Hospital Dubrava, Zagreb, Croatia.

https://bit.ly/3oMSBap. Read More

Cells 2021 12 29;11(1). Epub 2021 Dec 29.

Department of Ophthalmology, Charité-Universitätsmedizin Berlin, Augustenburger Platz 1, 13353 Berlin, Germany.

Ocular involvement is present in up to 79% of sarcoid patients. Uveitis is the main ocular manifestation and presents as a chronic intraocular inflammatory condition with potentially detrimental effects on visual acuity and quality of life. This retrospective study was conducted to explore the incidence and characteristics of ocular sarcoidosis in a single tertiary ophthalmology center. Read More

Brain Commun 2022 Feb 16;4(1):fcab292. Epub 2021 Dec 16.

Institute of Neuroimmunology and Multiple Sclerosis, University Medical Center Hamburg-Eppendorf, Germany.

Although most of the progressive multifocal leukoencephalopathy cases in sarcoidosis patients are explained by the treatment with immunosuppressive drugs, it is also reported in treatment-naive sarcoidosis patients, which implies a general predisposition of sarcoidosis patients for progressive multifocal leukoencephalopathy. Indeed, it was shown that active sarcoidosis patients have increased regulatory T cell frequencies which could lead to a subsequent systemic immunosuppression. However, if sarcoidosis with systemic changes of T cell subsets frequencies constitute a risk factor for the development of progressive multifocal leukoencephalopathy, which could then be counteracted by sarcoidosis treatment, is not known. Read More

Can J Neurol Sci 2022 Jan 3:1-2. Epub 2022 Jan 3.

Department of Radiology, McMaster University, Hamilton, Ontario, Canada.

Acta Gastroenterol Belg 2021 Oct-Dec;84(4):672-674

Dpt. of pneumology, AZ St Dimpna, Geel, Belgium.

We present the case of a 40-year-old male with recent history of moderately differentiated invasive adenocarcinoma of the sigmoid in whom both respiratory and neurological disease developed simultaneously, mimicking diffuse metastatic disease. The broad differential diagnosis and pitfalls (both diagnostic and therapeutic) are described. Pulmonary sarcoidosis as well as neurosarcoidosis occur very rarely after solid cancers. Read More

Neurohospitalist 2022 Jan 30;12(1):162-166. Epub 2021 Jun 30.

Department of Neurology, MedStar Georgetown University Hospital, Washington, DC, USA.

This report explores the case of a 49-year-old African American male with a six-month history of multifocal neurological deficits who presented to an outside hospital after a generalized seizure. Patient was transferred to our tertiary medical center after brain imaging showed multiple bilateral supratentorial intraparenchymal hemorrhages (IPH). A brain biopsy confirmed parenchymal and perivascular non-caseating granulomas with vasculitis. Read More

Neuropathol Appl Neurobiol 2022 Jun 13;48(4):e12789. Epub 2022 Jan 13.

Division of Neuroscience and Experimental Psychology, School of Biological Sciences, Faculty of Biology, Medicine and Health, The University of Manchester, Manchester, UK.

The brain is protected by the endothelial blood-brain barrier (BBB) that limits the access of micro-organisms, tumour cells, immune cells and autoantibodies to the parenchyma. However, the classic model of disease spread across a disrupted BBB does not explain the focal distribution of lesions seen in a variety of neurological diseases and why lesions are frequently adjacent to the cerebrospinal fluid (CSF) spaces. We have critically reviewed the possible role of a blood-CSF-brain route as a disease entry pathway into the brain parenchyma. Read More