Diagnostic Considerations

Chronic organic encephalopathy may mimic degenerative dementia. In any patient with systemic lupus erythematosus (SLE) with slowly progressive cognitive loss, a search for other clinical evidence of SLE activity, electrolyte disturbance, medication effect, vitamin B-12 deficiency, hypothyroidism, or opportunistic infections is indicated.

Mechanistic similarities between SLE and multiple sclerosis might have lead to the term lupoid sclerosis. Although at times, neuro-SLE might mimic multiple sclerosis very closely, pathologic studies clearly show them to be very distinct disorders.^[23]

Antiphospholipid syndrome was first described in association with SLE but also may occur independently. This should be searched for in patients known to have SLE with neurologic complications, especially myelopathy or cerebrovascular events, whether embolic, thrombotic, or hemorrhagic. Concomitant SLE and antiphospholipid syndrome have been shown to increase the risk of nervous system involvement.^[1]

Complement studies (C3, C4, CH50) may be useful to determine disease activity in patients known or thought to have SLE.

Other conditions that should be considered when evaluating a patient with suspected central nervous system (CNS) lupus include neuromuscular diseases, aseptic meningitis, Devic syndrome, Lambert-Eaton myasthenic syndrome, abducens (cranial nerve VI) nerve palsy, granulomatous angiitis of the CNS, acute and chronic inflammatory demyelinating polyradiculoneuropathy.

Differential Diagnoses

The following are considered in the differential diagnosis of CNS lupus:

Workup

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Media Gallery

This axial, T2-weighted brain magnetic resonance image (MRI) demonstrates an area of ischemia in the right periventricular white matter of a 41-year-old woman with longstanding systemic lupus erythematosus (SLE). She presented with headache and subtle cognitive impairments but no motor deficits. Faintly increased signal intensity was also seen on T1-weighted images, with a trace of enhancement following gadolinium that is too subtle to show on reproduced images. The distribution of the abnormality is consistent with occlusion of deep penetrating branches, such as may result from local vasculopathy, with no clinical or laboratory evidence of lupus anticoagulant or anticardiolipin antibody. Cardiac embolus from covert Libman-Sacks endocarditis remains less likely due to the distribution.

of 1

Author

Pradeep C Bollu, MD Associate Professor of Neurology, Vice Chair of Quality, Department of Neurology, Prisma Health, University of South Carolina School of Medicine, Columbia

Pradeep C Bollu, MD is a member of the following medical societies: American Academy of Neurology, American Academy of Sleep Medicine, Boone County Medical Society, Indian Medical Association, Indian Red Cross Society, International Parkinson and Movement Disorder Society, Missouri State Medical Association

Disclosure: Nothing to disclose.

Coauthor(s)

Sireesha Murala, MBBS Postdoctoral Research Associate, Division of Medical Genetics, Department of Pediatrics, Duke University Medical Center

Sireesha Murala, MBBS is a member of the following medical societies: Indian Medical Association, Medical Council of India

Disclosure: Nothing to disclose.

Specialty Editor Board

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

Disclosure: Received salary from Medscape for employment. for: Medscape.

Florian P Thomas, MD, PhD, MA, MS Chair, Neuroscience Institute and Department of Neurology, Director, Hereditary Neuropathy Center, Co-Director, Center for Memory Loss and Brain Health, Co-Director, ALS Center, Hackensack University Medical Center; Associate Dean of Faculty, Founding Chair and Professor, Department of Neurology, Hackensack Meridian School of Medicine

Florian P Thomas, MD, PhD, MA, MS is a member of the following medical societies: Academy of Spinal Cord Injury Professionals, American Academy of Neurology, American Neurological Association, Consortium of Multiple Sclerosis Centers, National Multiple Sclerosis Society, Sigma Xi, The Scientific Research Honor Society

Disclosure: Nothing to disclose.

Chief Editor

Niranjan N Singh, MBBS, MD, DM, FAHS, FAANEM Adjunct Associate Professor of Neurology, University of Missouri-Columbia School of Medicine; Medical Director of St Mary's Stroke Program, SSM Neurosciences Institute, SSM Health

Niranjan N Singh, MBBS, MD, DM, FAHS, FAANEM is a member of the following medical societies: American Academy of Neurology, American Association of Neuromuscular and Electrodiagnostic Medicine, American Headache Society

Disclosure: Nothing to disclose.

Additional Contributors

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 Academy of Neurology, American Academy of Pain Medicine, American College of Forensic Examiners Institute, American College of International Physicians, American College of Physicians, American Heart Association, American Stroke Association, National Association of Managed Care 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.

Thomas A Kent, MD Welch Chair in Chemistry Professor, Center for Genomic and Precision Medicine, Institute for Biosciences and Technology, Texas A&M Health Science Center; Adjunct Professor, Stanley H Appel Department of Neurology, Institute for Academic Medicine, Houston Methodist Hospital

Thomas A Kent, MD is a member of the following medical societies: American Academy of Neurology, American Neurological Association, New York Academy of Sciences, Royal Society of Medicine, Sigma Xi, The Scientific Research Honor Society, Stroke Council of the American Heart Association

Disclosure: Nothing to disclose.

CNS Lupus Differential Diagnoses

Diagnostic Considerations

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