title: Challenges in the diagnosis and treatment of neurosarcoidosis.
abstract: The diagnosis and treatment of neurosarcoidosis can be very challenging for several reasons. It affects clinically 5%-10% of sarcoidosis patients, but can be found in up to 25% of autopsies. These data reveal that a high percentage of asymptomatic or misdiagnosed cases can be missed at an initial diagnostic approach. Clinical and imaging findings are often non-specific since they can be found in a large number of neurological disorders. Histopathology can also be confounding if not performed by an expert pathologist and not placed in an appropriate clinical context. In this review, we discuss clinical features, laboratory findings, imaging, and histology of neurosarcoidosis, and we report current evidence regarding drug therapy. We conclude that a correct diagnostic approach should include a multidisciplinary evaluation involving clinicians, radiologists, and pathologists and that future studies should evaluate the genetic signature of neurosarcoidosis as they could be helpful in the assessment of this uncommon disease. With head-to-head comparisons of medical treatment for neurosarcoidosis still lacking due to the rarity of the disease and an increasing number of immunomodulating therapies at hand, novel therapeutic approaches are to be expected within the next few years.
authors.affiliation: a Internal Medicine Unit , Guastalla Hospital, AUSL Reggio Emilia , Reggio Emilia , Italy.
authors.forename: Claudio
authors.initials: C
authors.lastname: Tana
authors.affiliation: b Department of Neurology , University Hospital Zurich and University of Zurich , Zurich , Switzerland.
authors.forename: Susanne
authors.initials: S
authors.lastname: Wegener
authors.affiliation: c Department of Pathology , Loyola University Medical Center and Stritch School of Medicine, Loyola University Chicago , Maywood , Illinois , USA.
authors.forename: Ewa
authors.initials: E
authors.lastname: Borys
authors.affiliation: c Department of Pathology , Loyola University Medical Center and Stritch School of Medicine, Loyola University Chicago , Maywood , Illinois , USA.
authors.forename: Stefan
authors.initials: S
authors.lastname: Pambuccian
authors.affiliation: d Polyclinic for Dermatology and Venereology and Medical Faculty , University Hospital Lozenetz and Sofia University , Sofia , Bulgaria.
authors.forename: Georgi
authors.initials: G
authors.lastname: Tchernev
authors.affiliation: e Department of Medicine and Science of Aging , "G. d'Annunzio" University , Chieti , Italy.
authors.forename: Marco
authors.initials: M
authors.lastname: Tana
authors.affiliation: e Department of Medicine and Science of Aging , "G. d'Annunzio" University , Chieti , Italy.
authors.forename: Maria Adele
authors.initials: MA
authors.lastname: Giamberardino
authors.affiliation: a Internal Medicine Unit , Guastalla Hospital, AUSL Reggio Emilia , Reggio Emilia , Italy.
authors.forename: Mauro
authors.initials: M
authors.lastname: Silingardi
mesh_terms: D002493:Central Nervous System Diseases / Q000175:diagnosis* / Q000188:drug therapy / Q000503:physiopathology; D015195:Drug Design; D006801:Humans; D007155:Immunologic Factors / Q000627:therapeutic use*; D033183:Interdisciplinary Communication; D012507:Sarcoidosis / Q000175:diagnosis* / Q000188:drug therapy / Q000503:physiopathology
chemical_list: D007155:Immunologic Factors
keywords: Biopsy; magnetic resonance imaging; nervous system; sarcoidosis; spinal cord