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Neurosarcoidosis Differential Diagnoses

  • Author: Gabriel Bucurescu, MD, MS; Chief Editor: Tarakad S Ramachandran, MBBS, MBA, MPH, FAAN, FACP, FAHA, FRCP, FRCPC, FRS, LRCP, MRCP, MRCS  more...
 
Updated: Feb 19, 2016
 
 

Diagnostic Considerations

Sarcoidosis in general can at times be notoriously difficult to diagnose. A delay in diagnosis can result in increased morbidity and, in rare occasions, death. Patients who develop a neurologic illness consistent with neurosarcoidosis but are not known to have sarcoidosis present a diagnostic challenge. Other causes of neuropathy need to be considered, depending on the type of neuropathy, including acquired causes such as toxin exposure, metabolic abnormalities, and inflammatory disorders.

Neurosarcoidosis should not be readily diagnosed in children. This disorder is not common in children, and care must be taken to exclude other pathologies before this diagnosis is made.

Although necrotizing sarcoidosis has been reported most commonly in the lungs and rarely in other organ systems, sarcoidosis and its necrotizing variant should be considered in the differential diagnosis of an agranulomatous mass lesion involving the CNS. This is particularly true in patients with a history of systemic disease.

Other problems to be considered in the differential diagnosis of neurosarcoidosis, depending on the presentation, include the following:

  • Brain tumors
  • Porphyria
  • Toxins such as alcohol and drugs (both therapeutic and recreational)
  • Neuropathies associated with monoclonal bands and paraproteinemias
  • Myopathy of all types
  • Carcinomatous and lymphomatous meningitis with resultant polyradiculopathy
  • Berylliosis
  • Vitamin B-12 deficiency
  • Paraneoplastic neuropathy
  • Paraproteinemic neuropathy

Differential Diagnoses

 
 
Contributor Information and Disclosures
Author

Gabriel Bucurescu, MD, MS Staff Neurologist, Neurology Service, Philadelphia Veterans Affairs Medical Center

Gabriel Bucurescu, MD, MS is a member of the following medical societies: American Academy of Neurology

Disclosure: Nothing to disclose.

Coauthor(s)

Amer Suleman, MD Private Practice

Amer Suleman, MD is a member of the following medical societies: American College of Physicians, Society for Cardiovascular Angiography and Interventions, American Heart Association, American Institute of Stress, American Society of Hypertension, Federation of American Societies for Experimental Biology, Royal Society of Medicine

Disclosure: Nothing to disclose.

Chief Editor

Tarakad S Ramachandran, MBBS, MBA, MPH, FAAN, FACP, FAHA, FRCP, FRCPC, FRS, LRCP, MRCP, MRCS Professor Emeritus of Neurology and Psychiatry, Clinical Professor of Medicine, Clinical Professor of Family Medicine, Clinical Professor of Neurosurgery, State University of New York Upstate Medical University; Neuroscience Director, Department of Neurology, Crouse Irving Memorial Hospital

Tarakad S Ramachandran, MBBS, MBA, MPH, FAAN, FACP, FAHA, FRCP, FRCPC, FRS, LRCP, MRCP, MRCS is a member of the following medical societies: American College of International Physicians, American Heart Association, American Stroke Association, American Academy of Neurology, American Academy of Pain Medicine, American College of Forensic Examiners Institute, National Association of Managed Care Physicians, American College of Physicians, Royal College of Physicians, Royal College of Physicians and Surgeons of Canada, Royal College of Surgeons of England, Royal Society of Medicine

Disclosure: Nothing to disclose.

Acknowledgements

Paul E Barkhaus, MD Professor, Department of Neurology, Medical College of Wisconsin; Director of Neuromuscular Diseases, Milwaukee Veterans Affairs Medical Center

Paul E Barkhaus, MD is a member of the following medical societies: American Academy of Neurology, American Association of Neuromuscular and Electrodiagnostic Medicine, and American Neurological Association

Disclosure: Nothing to disclose.

Glenn Lopate, MD Associate Professor, Department of Neurology, Division of Neuromuscular Diseases, Washington University School of Medicine; Director of Neurology Clinic, St Louis ConnectCare; Consulting Staff, Department of Neurology, Barnes-Jewish Hospital

Glenn Lopate, MD is a member of the following medical societies: American Academy of Neurology, American Association of Neuromuscular and Electrodiagnostic Medicine, and Phi Beta Kappa

Disclosure: Baxter Grant/research funds Other; Amgen Grant/research funds None

Nicholas Lorenzo, MD Chief Editor, Medscape Reference Neurology; Consulting Staff, Neurology Specialists and Consultants

Nicholas Lorenzo, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Neurology, and American College of Physician Executives

Disclosure: Nothing to disclose.

Haresh Mani, MD Assistant Professor, Department of Pathology, Milton S Hershey Medical Center, Pennsylvania State University College of Medicine

Disclosure: Nothing to disclose.

N K Nikhar, MD , MRCP Private Practice

N K Nikhar, MD is a member of the following medical societies: American Academy of Neurology

Disclosure: Nothing to disclose.

Amy A Pruitt, MD Associate Professor of Neurology, University of Pennsylvania; Attending Neurologist, Hospital of the University of Pennsylvania

Amy A Pruitt, MD is a member of the following medical societies: American Academy of Neurology

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Salary Employment

Florian P Thomas, MD, MA, PhD, Drmed Director, Spinal Cord Injury Unit, St Louis Veterans Affairs Medical Center; Director, National MS Society Multiple Sclerosis Center; Professor, Department of Neurology and Psychiatry, Associate Professor, Institute for Molecular Virology, and Department of Molecular Microbiology and Immunology, St Louis University

Florian P Thomas, MD, MA, PhD, Drmed is a member of the following medical societies: American Academy of Neurology, American Paraplegia Society, and National Multiple Sclerosis Society

Disclosure: Nothing to disclose.

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Atrophic right optic disc of a 37-year-old man with neurosarcoidosis and involvement of both optic nerves. Vision was lost. The disc is pale with sharp borders.
Atrophic left optic disc of a 37-year-old patient with neurosarcoidosis and involvement of both optic nerves. The disc is pale with sharp borders. Vision was largely preserved.
MRI of the brain in a 37-year-old man with neurosarcoidosis who had complete loss of vision in the right eye for 2 months and occasional blurry vision in the left. T1-weighted sagittal image shows intact optic nerves.
MRI of the brain in a 37-year-old man with neurosarcoidosis who had complete loss of vision in the right eye and mild left eye blurriness. This fluid-attenuated inversion recovery (FLAIR) axial image shows a wedge-shaped area of infarction in the right temporo-occipital area. The optic nerves exhibit abnormal signal.
MRI of the brain in a 37-year-old patient with sarcoidosis who had right eye blindness and mild blurry vision in the left eye. This postgadolinium, T1-weighted axial image shows right optic nerve enhancement along almost the entire intraorbital portion and a small amount in the prechiasmatic portion. The left optic nerve enhances from the level of the optic chiasm to the distal intraorbital portion. The right temporo-occipital infarct is seen as a faint hypodensity; it does not enhance after gadolinium administration.
MRI of the brain in a 37-year-old man with sarcoidosis who had loss of vision in the right eye and blurry vision in the left eye. This scan was taken 6 months after the scan shown in Pictures 3, 4, and 5. Both the right and left optic nerves are enlarged and show abnormal signal on this T1-weighted axial image. The patient remained on oral prednisone from the time of the first scan and did not exhibit any further loss of vision in the left eye. Vision in the right eye never returned.
MRI of the brain in a 37-year-old man with sarcoidosis who had loss of vision in the right eye and blurry vision in the left. This postgadolinium, T1-weighted axial image shows abnormal enhancement of both optic nerves, with the left optic nerve appearing worse on this study than in the study shown as Picture 5, which was done 6 months earlier. The right temporo-occipital hypodensity represents the old infarction.
Early chest radiograph findings in sarcoidosis.
Advanced chest radiograph findings in sarcoidosis.
Noncaseating granuloma surrounded by epithelioid cells, from the medulla oblongata. Also shown are nodular inflammatory infiltrates consisting of multinucleated giant cells, macrophages, and lymphocytes (hematoxylin and eosin, 40x).
Noncaseating granuloma in medulla oblongata showing the granuloma surrounded by epithelioid cells and nodular inflammatory infiltrates (hematoxylin and eosin, 20x).
 
 
 
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