A case of neuropsychiatric lupus Erythematosus characterised by the Owl’s eye signal: a case report.
BMC Neurol. 2017 Jun 29;17(1):123
Authors: Hu B, Wu P, Zhou Y, Peng Y, Tang X, Ding W, Zhang M, Qi X
BACKGROUND: Systemic lupus erythematosus (SLE) is an autoimmune inflammatory dysfunction characterised by a number of affected techniques. Greater than half of SLE sufferers will undergo from neuropsychiatric lupus erythematosus (NPSLE) in the course of the course of their illness. Though practically half of the NPSLE sufferers have regular MRI manifestations, the abnormalities discovered within the the rest might be positioned wherever within the mind, and particularly within the subcortical white matter of the frontal and temporal lobe. Nonetheless, NPSLE involving the medulla oblongata and spinal twine which presents because the “owl’s eye” signal has to our greatest information not been reported thus far.
CASE PRESENTATION: A 19-year-old lady offered at our hospital with a 7-day historical past of fever and headache since a someday’s exertion, accompanied by 2 days of weak spot. The affected person had slurred speech. Neurological examination revealed the presence of horizontal nystagmus and a limitation of bilateral eye motion when wanting up and down. On the identical time, she confirmed issue in elevating the jaw, accompanied by a weak pharyngeal reflex. Muscle power was remarkably decreased in all 4 extremities: the MRCS grade of the higher limbs was four/5, whereas within the decrease limbs it was zero/5. Hypotonia was obvious within the decrease extremities. Concerning subjective sensation, the affected person seemed to be experiencing an elevated sense of ache in the entire physique, and particularly within the cervical area, stomach, and ft. An examination of shallow reflex documented the reinforcement of the stomach reflex. Deep tendon reflexes have been symmetric: absent in decrease, regular in higher extremities. The affected person additionally had a stiff neck with a constructive Kernig’s signal. The laboratory examination confirmed elevated C – reactive protein and rheumatoid issue, in addition to complement parts three and four. Symptomatic remedies have been utilized, however she didn’t reply nicely, after which we did immunological laboratory examinations. The outcomes confirmed the presence of anti-nRNP/Sm, anti-dsDNA and anti-AMA M2 antibodies. An MRI scan and enhancement of the cervical and thoracic areas displayed irregular indicators within the medulla and bilateral anterior horn of the decrease thoracic backbone. Following the exclusion of different doable ailments, neuropsychiatric lupus was identified. Excessive-dose intravenous gamma-globulin mixed with methylprednisolone regularly improved her situation.
CONCLUSION: We report the primary case of NPSLE presenting with medulla oblongata and spinal twine involvement, manifesting because the “owl’s eye” register MRI.
PMID: 28662631 [PubMed – indexed for MEDLINE]