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Mark Peter Maskery, Paul N Cooper, Adrian Pace
Sarcoidosis is an idiopathic multisystem granulomatous disorder of unknown cause. Nervous system involvement (central and/or peripheral) is uncommon, developing in 5%-10%. The presenting symptoms are variable, reflecting the level of involvement, and frequently fluctuate and progress. Diagnosing neurosarcoidosis in people with previously confirmed systemic disease may be relatively straightforward, but diagnosing primary neurosarcoidosis is challenging. Managing neurosarcoidosis is primarily consensus based; corticosteroid is its mainstay, alongside corticosteroid-sparing agents and emerging novel therapies...
January 23, 2018: Practical Neurology
Michael A Galgano, Carlos R Goulart, Karen Chisholm, Melissa Hazen, Scellig Stone
BACKGROUND: Isolated intraspinal neurosarcoidosis is a rare clinical entity, with most reports describing intramedullary involvement in adults. CASE DESCRIPTION: We detail the case of a 9-year-old girl with rapid-onset compressive myelopathy secondary to a thoracic epidural lesion. Although pathologic diagnosis was challenging, a presumptive diagnosis of isolated extradural neurosarcoidosis was made in light of the patient's investigations and dramatic response to corticosteroids...
March 2018: World Neurosurgery
Maxime O Baud, Jeffrey R Vitt, Nathaniel M Robbins, Rafael Wabl, Michael R Wilson, Felicia C Chow, Jeffrey M Gelfand, S Andrew Josephson, Steve Miller
Objective: The mechanism of hypoglycorrhachia-low CSF glucose-in meningitis remains unknown. We sought to evaluate the relative contribution of CSF inflammation vs microorganisms (bacteria and fungi) in lowering CSF glucose levels. Methods: We retrospectively categorized CSF profiles into microbial and aseptic meningitis and analyzed CSF leukocyte count, glucose, and protein concentrations. We assessed the relationship between these markers using multivariate and stratified linear regression analysis for initial and repeated CSF sampling...
January 2018: Neurology® Neuroimmunology & Neuroinflammation
Sadanandavalli Retnaswami Chandra, Lakshminarayanapuram Gopal Viswanathan, Rahul Wahatule, Safal Shetty
Introduction: Brain as the seat of behavior is acknowledged from the times of Charaka, however where neurology ends and philosophy begins remains an enigma. It is certainly every neurologist's observation that there is loss of function either region based or domain based in progressive diseases of the nervous system making it the seat of all useful activities. However, there are references to occurrence of new skills seen during various illnesses causing progressive cognitive dysfunction...
November 2017: Indian Journal of Psychological Medicine
G Bathla, P Watal, S Gupta, P Nagpal, S Mohan, T Moritani
Involvement of the central nervous system by sarcoidosis, also referred to as neurosarcoidosis, is seen clinically in about 5% of patients with systemic disease. CNS involvement most frequently affects the leptomeninges and cranial nerves, though the ventricular system, brain parenchyma, and pachymeninges may also be involved. Even though the involvement of the intracranial vascular structures is well-known on postmortem studies, there is scant literature on imaging manifestations secondary to the vessel wall involvement, being confined mostly to isolated case reports and small series...
December 28, 2017: AJNR. American Journal of Neuroradiology
Nicholas L Zalewski, Eoin P Flanagan, B Mark Keegan
OBJECTIVE: To evaluate specific myelopathy diagnoses made in patients with suspected idiopathic transverse myelitis (ITM). METHODS: A total of 226 patients 18 years and older were referred to Mayo Clinic Neurology for suspected ITM from December 1, 2010, to December 31, 2015. Electronic medical records were reviewed for detailed clinical presentation and course, laboratory and electrophysiologic investigations, and neuroimaging to determine the etiology. Current diagnostic criteria for ITM and alternative myelopathy diagnoses were applied...
January 9, 2018: Neurology
Anna R Larici, Andor W Glaudemans, Annemilia Del Ciello, Riemer H Slart, Lucio Calandriello, Olivier Gheysens
Sarcoidosis is a multisystem chronic inflammatory disease of unknown etiology characterized by widespread growth of non-caseating granulomas. The diagnosis of sarcoidosis is based on clinical and imaging presentation, histologic confirmation and the absence of alternative diseases. Radiology and Nuclear Medicine play an essential role in the diagnostic work-up of patients with sarcoidosis to assess disease extent and activity. In addition, imaging modalities have shown their potential in managing these patients in terms of treatment response and prognostic assessment...
March 2018: Quarterly Journal of Nuclear Medicine and Molecular Imaging
Jacqueline J Greene, Ilka C Naumann, Janet M Poulik, Kevin T Nella, Lindsay Weberling, Jeffrey P Harris, Akihiro J Matsuoka
BACKGROUND: A rare subset of sarcoidosis, neurosarcoidosis, is reported to occur in 5-7% of sarcoid patients and can manifest in a variety of ways. The most common are facial paralysis and optic neuritis, less commonly causing cochleovestibulopathy, blindness, anosmia, and other cranial nerve (CN) palsies. The sensory deficit may be severe and psychiatric symptoms may result from the effects of the disease or steroid treatment. Although MRI-compatible cochlear implants are now available, concerns about the feasibility of recoverable hearing with cochlear implantation in these patients as well as the practical difficulty of disease monitoring due to implant artifact must be considered...
November 23, 2017: Audiology & Neuro-otology
Jonas Graf, Marius Ringelstein, Orhan Aktas, Mike P Wattjes, Hans-Peter Hartung
Neurosarcoidosis is a rare disease with various clinical phenotypes. Thus, case reports and series broaden the understanding of this entity (Ibitoye et al., 2017). We present a case of isolated intramedullary spinal cord sarcoidosis, an exceedingly rare phenotype, which needs to be distinguished especially from neuromyelitis optica spectrum disorders (NMOSD). This report exemplifies the need for a systematic approach to diagnosis and management of spinal cord sarcoidosis.
November 2017: Multiple Sclerosis and related Disorders
Bharat Bajantri, Sindhaghatta Venkatram, Masooma Niazi, Tushi Singh, Gilda Diaz-Fuentes
RATIONALE: Neurosarcoidosis (NS) is an uncommon manifestation of systemic sarcoidosis, with a propensity for middle-agedwomen. Often discovered only at autopsy, rates of neurologic involvement (5%-10%) reported in the literature underscore a lack of sensitivity and specificity in current diagnostic methods. PATIENT CONCERNS: Herein, we describe a 53-year-old woman who presented with gait imbalance and distal extremity muscular weakness. She was known to harbor a brain mass (4 years in duration) that was monitored and recently seemed to enlarge...
November 2017: Medicine (Baltimore)
Patompong Ungprasert, Eric L Matteson
Neurosarcoidosis occurs in 3% to 10% of patients with sarcoidosis. Cranial neuropathy and meningeal involvement are the most common manifestations, but any part of the nervous system can be affected. Definite diagnosis requires the presence of noncaseating granuloma in the nervous system, although histopathologic confirmation is often not obtainable. Moderate to high dose of glucocorticoids is the main therapy for neurosarcoidosis. Relapse often occurs after the dose of glucocorticoids is tapered down, often necessitating the use of steroid-sparing immunosuppressive agents...
November 2017: Rheumatic Diseases Clinics of North America
Tali Drori, Gili Givaty, Joab Chapman, Merav Lidar, Pnina Langevitz, Yehuda Shoenfeld, Oren S Cohen
Specific inflammatory pathways and specifically Tumor Necrosis Factor alpha (TNF-α) have been associated with the neurodegeneration in Parkinson's disease (PD). TNFα is also known to play an important role in the pathogenesis of sarcoidosis and TNF blockers can ameliorate the disease. In contrast, multiple sclerosis (MS) is clearly exacerbated by anti- TNF-α medications. We have therefore hypothesized that Parkinson-like disease would be more common in neurosarcoidosis (NS) compared to MS. The aim of this case-control study was therefore to assess the frequency of extrapyramidal signs in patients with NS compared to MS patients...
October 16, 2017: Immunobiology
Jeffrey M Gelfand
No abstract text is available yet for this article.
November 1, 2017: JAMA Neurology
Bastien Joubert, Catherine Chapelon-Abric, Lucie Biard, David Saadoun, Sophie Demeret, Didier Dormont, Matthieu Resche-Rigon, Patrice Cacoub
Importance: Prognostic factors are lacking in neurosarcoidosis (NS), and the association of immunosuppressive treatments with outcomes are unclear. Objectives: To identify prognostic factors of and analyze the association of immunosuppressive treatment with relapse of NS. Design, Setting, and Participants: In this retrospective study, a cohort of 234 patients fulfilled the diagnostic criteria for NS in a tertiary referral center in Paris, France, from January 1, 1990, through December 31, 2015...
November 1, 2017: JAMA Neurology
Jeffrey M Gelfand, Michael J Bradshaw, Barney J Stern, David B Clifford, Yunxia Wang, Tracey A Cho, Laura L Koth, Stephen L Hauser, Jason Dierkhising, NgocHanh Vu, Subramaniam Sriram, Harold Moses, Francesca Bagnato, Jeffrey A Kaufmann, Deidre J Ammah, Tsion H Yohannes, Mark J Hamblin, Nagagopal Venna, Ari J Green, Siddharama Pawate
OBJECTIVE: To describe clinical and imaging responses in neurosarcoidosis to infliximab, a monoclonal antibody against tumor necrosis factor-α. METHODS: Investigators at 6 US centers retrospectively identified patients with CNS sarcoidosis treated with infliximab, including only patients with definite or probable neurosarcoidosis following rigorous exclusion of other causes. RESULTS: Of 66 patients with CNS sarcoidosis (27 definite, 39 probable) treated with infliximab for a median of 1...
November 14, 2017: Neurology
J David Avila, Robert C Bucelli
No abstract text is available yet for this article.
October 2017: Neurohospitalist
Matteo Gastaldi, Enrico Marchioni, Paola Banfi, Valeria Mariani, Laura Di Lodovico, Roberto Bergamaschi, Enrico Alfonsi, Paola Borrelli, Ottavia Eleonora Ferraro, Elisabetta Zardini, Anna Pichiecchio, Andrea Cortese, Patrick Waters, Mark Woodhall, Mauro Ceroni, Marco Mauri, Diego Franciotta
BACKGROUND: Transverse myelitis (TM) is an inflammatory disorder that can be idiopathic or associated with central nervous system autoimmune/dysimmune inflammatory diseases, connective tissue autoimmune diseases, or post-infectious neurological syndromes. Prognosis of initial TM presentations is uncertain. OBJECTIVE: To identify outcome predictors in TM. METHODS: Retrospective study on isolated TM at onset. Scores ⩾3 on the modified Rankin scale (mRS) marked high disability...
September 1, 2017: Multiple Sclerosis: Clinical and Laboratory Research
Guang Yang, Yoshinobu Eishi, Anwar Raza, Heather Rojas, Adina Achiriloaie, Kenneth De Los Reyes, Ravi Raghavan
A 56-year-old woman with a 3-year history of hydrocephalus and ventriculo-peritoneal shunt placement, presented with worsening altered level of consciousness for 2 days. Imaging studies showed severe ventriculomegaly involving the lateral and third ventricles with multiple septated cysts noted in the lateral ventricles predominantly near the frontal horns. Histopathologic examination of the excised brain lesion revealed choroid plexus tissue and adjacent cerebral parenchyma with several non-caseating granulomas...
August 30, 2017: Neuropathology: Official Journal of the Japanese Society of Neuropathology
Michael Thambuswamy, Ashish H Shah, Mai Tran, Joanne T Thambuswamy, Amade Bregy, Michael D Norenberg, Ricardo J Komotar
Isolated neurosarcoidosis without prior systemic symptoms is extremely rare, occurring in approximately 2% of patients with neurosarcoidosis. Due to its rarity, mistakes in diagnosis and treatment occur commonly. We present a case of a 47-year-old female who was found to have an incidentally discovered solitary intracranial lesion that mimicked a high-grade neoplasm, but was later confirmed to be neurosarcoidosis. Incidental solitary neurosarcoid granulomas are difficult to diagnose due to its nonspecific clinical and imaging presentations...
July 2017: Asian Journal of Neurosurgery
Haben Kefella, Daniel Luther, Clotilde Hainline
PURPOSE OF REVIEW: Sarcoidosis is a multisystemic inflammatory disease that commonly affects the eye and less often the neuro-ophthalmic pathways. The manifestations can be quite variable but can have characteristic signs and clinical features. This review provides a comprehensive overview of the various ocular and neuro-ophthalmic manifestations of sarcoidosis, emerging diagnostic measures and approach to treatment. Particular focus is given to recent advances in diagnostic approach and available treatment options...
November 2017: Current Opinion in Ophthalmology
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