Abstract
BackgroundSystemic lupus erythematosus (SLE) is an autoimmune inflammatory disorder characterized by multiple affected systems. More than half of SLE patients will suffer from neuropsychiatric lupus erythematosus (NPSLE) during the course of their disease. Although nearly half of the NPSLE patients have normal MRI manifestations, the abnormalities found in the remainder can be located anywhere in the brain, and especially in the subcortical white matter of the frontal and temporal lobe. However, NPSLE involving the medulla oblongata and spinal cord which presents as the “owl’s eye” sign has to our best knowledge not been reported to date.Case presentationA 19-year-old girl presented at our hospital with a 7-day history of fever and headache since a one day’s exertion, accompanied by 2 days of weakness. The patient had slurred speech. Neurological examination revealed the presence of horizontal nystagmus and a limitation of bilateral eye movement when looking up and down. At the same time, she showed difficulty in raising the jaw, accompanied by a weak pharyngeal reflex. Muscle strength was remarkably decreased in all four extremities: the MRCS grade of the upper limbs was 4/5, while in the lower limbs it was 0/5. Hypotonia was apparent in the lower extremities. Regarding subjective sensation, the patient appeared to be experiencing an increased sense of pain in the whole body, and especially in the cervical region, abdomen, and feet. An examination of shallow reflex documented the reinforcement of the abdominal reflex. Deep tendon reflexes were symmetric: absent in lower, normal in upper extremities. The patient also had a stiff neck with a positive Kernig’s sign. The laboratory examination showed elevated C - reactive protein and rheumatoid factor, as well as complement components 3 and 4. Symptomatic treatments were applied, but she did not respond well, after which we did immunological laboratory examinations. The results showed the presence of anti-nRNP/Sm, anti-dsDNA and anti-AMA M2 antibodies. An MRI scan and enhancement of the cervical and thoracic regions displayed abnormal signs in the medulla and bilateral anterior horn of the lower thoracic spine. Following the exclusion of other possible diseases, neuropsychiatric lupus was diagnosed. High-dose intravenous gamma-globulin combined with methylprednisolone gradually improved her condition.ConclusionWe report the first case of NPSLE presenting with medulla oblongata and spinal cord involvement, manifesting as the “owl’s eye” sign in MRI.
Highlights
Systemic lupus erythematosus (SLE) is an autoimmune inflammatory disorder characterized by multiple affected systems
We report the first case of neuropsychiatric lupus erythematosus (NPSLE) presenting with medulla oblongata and spinal cord involvement, manifesting as the “owl’s eye” sign in Magnetic resonance imaging (MRI)
It has been reported in a number of diseases, such as complex cervical spondylosis, Hirayama disease, poliomyelitis, encephalitis caused by HIV infection, spinal cord infarction, neuromyelitis optica, paraneoplastic neurological syndrome and flail arm syndrome [20, 21]
Summary
Systemic lupus erythematosus (SLE) is an autoimmune inflammatory disorder characterized by multiple affected systems [1]. Seven days before clinical presentation, the girl had developed a headache, sore throat, bucking, vomiting and fever after one day’s hard work She went to the local hospital, and after several days of symptomatic treatment, only the temperature dropped slightly. Her personal history was unremarkable, and no similar symptoms were found in her family When she was transferred to our hospital, she was in a state of sleepiness, dysarthria, dysphagia and fever, with a body temperature of 37.8 °C, while an examination of the heart, lungs and abdomen revealed that they were normal. Axial T2 weighted imaging and the magnified image of the thoracic spinal cord revealed a small rough high signal in the bilateral anterior horn showing an“owl’s eye” sign At the 3-month follow-up, the patient’s neurological deficit was completely resolved with weekly i.v. pulsed cyclophosphamide (400 mg/week)
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