Unusual Presentations for Lupus: From Occular Oddities to Cauda Equina Nerve Root Enhancement
Khan MS, and Memon A. Unusual Presentations for Lupus: From Occular Oddities to Cauda Equina Nerve Root Enhancement. Neurology 2019; 92(15).
Objective: Atypical neurological manifestation of systemic lupus erythematosus (SLE) with multiple cranial neuropathies and cauda equina nerve root enhancement, mimicking acute inflammatory demyelinating polyneuropathy (AIDP). Background: SLE can present with neurological manifestation, including the central and peripheral nervous systems and muscles. Its relatively easier to diagnose SLE when it presents with systemic manifestations. 10%-20% of patients with systemic lupus erythematosus show peripheral nervous system involvement. SLE may affect the visual system in up to a third of patients. Case 1: 28-year-old woman admitted with multiorgan failure and severe encephalopathy preceded by flue like symptoms. Upon arousal from coma patient reported visual loss and severe lower extremity weakness. Neurological examination revealed no light perception in both eyes, severe flaccid lower extremity weakness with areflexia. Patient had an extensive neurological work up done which showed positive ANA, Anti-RNP and anti-Smith antibodies with low C3, very high CSF protein, multiple cranial neuropathies and cauda equina nerve root enhancement. Patient failed IVIG and PLEX. Patient improved with high doses of steroids followed by oral prednisone and Plaquenil. Case 2: 29-year-old woman presented with transient visual loss and bilateral lower extremity paresthesia preceded by viral prodrome. On exam she had very mild optic disc edema, lower extremity weakness with areflexia. CSF showed elevated protein and white count. She had positive ANA, anti-RNP and anti-smith antibodies. Patient later developed a malar rash and cerebral venous sinus thrombosis (CVT). Treatment for lupus was initiated with pulse steroids, followed by oral prednisone and Plaquenil. Anticoagulation was initiated for CVT. Patient symptoms improved significantly with treatment. Results: N/A Conclusions: We present two interesting cases of SLE with ocular manifestation and vasculitis polyradiculopathy mimicking AIDP. Lupus should be considered as a differential for AIDP in patients with cauda equina nerve root enhancement with positive autoimmune panel not responsive to IVIG or PLEX.