CNS Lupus Differential Diagnoses

Updated: Aug 22, 2017
  • Author: Tarakad S Ramachandran, MBBS, MBA, MPH, FAAN, FACP, FAHA, FRCP, FRCPC, FRS, LRCP, MRCP, MRCS; Chief Editor: Niranjan N Singh, MD, DM  more...
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DDx

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 or thyroid deficiency, or opportunistic infection in the immunosuppressed patient, 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 with known to have SLE with neurologic complications, especially myelopathy or cerebrovascular events, whether embolic, thrombotic, or hemorrhagic. Concomitant SLE and antiphospholipid syndrome has 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 of 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: