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Background: Systemic 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 Presentation: A 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.Conclusion: We report the first case of NPSLE presenting with medulla oblongata and spinal cord involvement, manifesting as the "owl's eye" sign in MRI. [ABSTRACT FROM AUTHOR]

The treatment of clival chordoma remains highly challenging. This difficulty is enhanced by the very small likelihood of a successful complete surgical resection or nonsurgical treatment of chordoma. Additionally, no effective means of interdisciplinary treatment for chordoma have been identified. With this background, we analyzed data of patients who underwent multidisciplinary treatment for clival chordoma at our institution during the last 25 years.This retrospective study evaluated patients treated at a single center from 1992 to 2017.During the study period, 24 patients underwent 24 surgeries. Twenty-two surgical resections (including 1 initial surgery and 1 surgery for recurrence) were deemed maximally safe cyto-reductive resections (92%); the remaining 2 surgeries were deemed incomplete (8%), which were histologically confirmed in all but in 1 case (which involved radionecrosis). The complications were divided into endocrinologic, neurologic, and other complications. In 1 case (4%), surgery led to immediate dyspnea followed by death on the following day; in another case (4%), ischemic infarction led to sudden death. In 3 cases (13%), patients exhibited improvements of neurologic (visual or oculomotor) deficits that had been observed prior to surgery. The following new postoperative neurologic deficits were observed: oculomotor deficits in 4 cases, dizziness in 2 cases, and cranial nerve-attributed dysphagia in 3 cases. About 19 patients underwent adjuvant postoperative radiotherapy following the initial surgery (dose: 54.5 Gy in all cases). The mean and median follow-up durations were 50 ± 53 and 48.5 months, respectively. A Kaplan-Meier analysis estimated a median survival duration of 50.2 months (95% confidence interval 27.9-72.4 months).These findings highlight the importance of interdisciplinary treatment strategies, particularly those combining maximally safe cyto-reductive tumor resection and adjusted radiotherapy and other treatment options, for patients with relatively good conditions.