Neurosarcoidosis

https://doi.org/10.1016/j.rdc.2017.06.008Get rights and content

Section snippets

Key points

  • Neurosarcoidosis occurs in 3% to 10% of patients with sarcoidosis.

  • Any part of the nervous system can be affected with cranial neuropathy and meningeal involvement being the most common manifestations.

  • Glucocorticoids are the main therapy, although immunosuppressive agents are also often required because of the high rate of relapse.

Clinical features

Any part of the nervous system can be affected by sarcoidosis, and multiple lesions are often noted.11, 12, 13 The frequency of each neurologic manifestation is summarized in Table 1. Neurologic abnormalities are one of the first clinical manifestations that lead to the diagnosis of sarcoidosis in 70% to 80% of patients with neurosarcoidosis.13, 14, 15 Isolated neurosarcoidosis is uncommon, because more than 90% of patients also have sarcoidosis in other organs, especially the lungs and

Diagnostic tests

Similar to systemic sarcoidosis, diagnosis of neurosarcoidosis requires the presence of noncaseating granuloma and compatible clinical presentations after exclusion of alternative diagnoses.50 The presence of noncaseating granuloma itself is not sufficient to make a definite diagnosis because it could be seen in other conditions, such as foreign body reaction, tuberculosis, and fungal infection.11, 25

Given the invasive nature of biopsy of the nervous system, histopathologic confirmation is

Treatments

Unlike pulmonary sarcoidosis, spontaneous resolution of neurosarcoidosis is uncommon (except for facial nerve palsy), and treatment is generally indicated to minimize morbidity and mortality.6 Unfortunately, there have been no randomized, placebo-controlled trials in neurosarcoidosis to guide treatment. Therefore, all recommendations are based on clinical experience and limited data from observation studies. Doses, adverse reactions, and monitoring of the commonly used medications in

Summary

Neurosarcoidosis is an uncommon manifestation of sarcoidosis that is associated with significant morbidity and mortality. Diagnosis of neurosarcoidosis is often challenging, because clinical manifestations and findings on imaging studies can be mimicked by several other diseases. Glucocorticoids are the cornerstone for the treatment of neurosarcoidosis; immunosuppressive agents are also often required. Clinical trials are needed to establish the efficacy of these treatments.

First page preview

First page preview
Click to open first page preview

References (81)

  • I. Adamec et al.

    Progressive meningoencephalitis due to neurosarcoidosis

    Clin Neurol Neurosurg

    (2013)
  • J. Youssef et al.

    Infection risk and safety of corticosteroid use

    Rheum Dis Clin North Am

    (2016)
  • J.D. Doty et al.

    Treatment of corticosteroid-resistant neurosarcoidosis with a short-course cyclophosphamide regimen

    Chest

    (2003)
  • A. Chaussenot et al.

    Neurosarcoidosis treated with mycophenilate mofetil: two cases

    Rev Neurol

    (2007)
  • M. Sodhi et al.

    Infliximab therapy rescues cyclophosphamide failure in severe central nervous system sarcoidosis

    Respir Med

    (2009)
  • P. Brito-Zeron et al.

    Epidemiologic patterns of disease expression in sarcoidosis: age, gender and ethnicity-related differences

    Clin Exp Rheumatol

    (2016)
  • T. Morimoto et al.

    Epidemiology of sarcoidosis in Japan

    Eur Respir J

    (2008)
  • M.A. Judson et al.

    The clinical course of sarcoidosis: presentation, diagnosis, and treatment in a large white and black cohort in the United States

    Sarcoidosis Vasc Diffuse Lung Dis

    (2012)
  • R.P. Baughman et al.

    Clinical characteristics of patients in a case control study of sarcoidosis

    Am J Respir Crit Care Med

    (2001)
  • S.K. Sharma et al.

    Clinical characteristics, pulmonary function abnormalities and outcome of prednisolone treatment in 106 patients with sarcoidosis

    J Assoc Physicians India

    (2001)
  • K. Iwai et al.

    Pathological studies on sarcoidosis autopsy

    Acta Pathol Jpn

    (1993)
  • W. Ricker et al.

    Sarcoidosis; a clinicopathological review of 300 cases, including 22 autopsies

    Am J Clin Pathol

    (1949)
  • R.K.A. Allen et al.

    A prospective study of 32 patients with neurosarcoidosis

    Sarcoidosis Vasc Diffuse Lung Dis

    (2003)
  • D. Ferriby et al.

    Long-term follow-up of neurosarcoidosis

    Neurology

    (2001)
  • F.G. Joseph et al.

    Neurosarcoidosis: a study of 30 new cases

    J Neurol Neurosurg Psychiatry

    (2009)
  • B.J. Stern et al.

    Sarcoidosis and its neurological manifestations

    Arch Neurol

    (1985)
  • M.L. Carlson et al.

    Cranial base manifestation of neurosarcoidosis: a review of 305 patients

    Otol Neurotol

    (2014)
  • S. Pawate et al.

    Presentations and outcomes of neurosarcoidosis: a study of 54 cases

    QJM

    (2009)
  • A. Dua et al.

    Images in clinical medicine. Heerfordt’s syndrome, or uveoparotid fever

    N Engl J Med

    (2013)
  • P. Ungprasert et al.

    Clinical characteristics of parotid gland sarcoidosis: a population-based study

    JAMA Otolaryngol Head Neck Surg

    (2016)
  • E.E. Lower et al.

    Diagnosis and management of neurological sarcoidosis

    Arch Intern Med

    (1997)
  • J.P. Zajicek et al.

    Central nervous system sarcoidosis – diagnosis and management

    QJM

    (1999)
  • K. Kane

    Deafness in sarcoidosis

    J Laryngol Otol

    (1976)
  • S. Marangoni et al.

    Neurosarcoidosis. Clinical description of 7 cases with proposal for a new diagnostic strategy

    J Neurol

    (2006)
  • D.A. Nowak et al.

    Neurosarcoidosis: a review of its intracranial manifestation

    J Neurol

    (2001)
  • V. Terushkin et al.

    Neurosarcoidosis. Presentations and management

    Neurologist

    (2010)
  • A. Krumholz et al.

    Clinical implications of seizures in neurosarcoidosis

    Arch Neurol

    (1991)
  • B. Chang et al.

    Depression in sarcoidosis

    Am J Respir Crit Care Med

    (2001)
  • J. Brook et al.

    Computed tomography changes in neurosarcoidosis clearing with steroid treatment

    J Comput Assist Tomogr

    (1979)
  • S.U. Ho et al.

    Sarcoid encephalopathy with diffuse inflammation and focal hydrocephalus shown by sequential CT

    Neurology

    (1979)
  • Cited by (50)

    • Sarcoidosis

      2023, Immunology and Allergy Clinics of North America
    • Rare autoimmune and autoinflammatory neurologic disorders

      2023, Translational Neuroimmunology: Neuroinflammation: Volume 7
    • Pediatric neurosarcoidosis of the internal auditory canal: A case report

      2022, Otolaryngology Case Reports
      Citation Excerpt :

      It can affect any organ but is primarily seen in the lungs. It can rarely present as neurosarcoidosis in the central or peripheral nervous system with a reported prevalence of 3% and 10% of adult patients [1]. A 5-year-old African American female initially presented to the pediatric neurology clinic for evaluation of sudden onset right sided unilateral facial palsy with incomplete eye closure.

    View all citing articles on Scopus

    Disclosure Statement: The authors have no financial or nonfinancial potential conflicts of interest to declare.

    View full text