AB1275 HYDROCEPHALUS IN AUTOIMMUNE DISEASES: A SERIES OF CHALLENGING CASES
Authors: N. El Ghobashy
Journal: Annals of the Rheumatic Diseases (2020)

Background: Hydrocephalus can be a rare neurological manifestation but lethal complication of various SAIDs, including systemic lupus erythematosus (SLE), rheumatoid arthritis (RA), sarcoidosis, and primary vasculitis. The commonly used medical management programs based on the etiology of hydrocephalus are anti-inflammatory or anti infectious therapies, while surgical management such as ventriculoperitoneal shunts is effective most of the time (Wei Jetal.,2019). Objectives: To analyze the diagnosis and managment of hydrocephalus associated with autoimmune diseases Methods: A retrospective case series study was conducted at Rheumatology department.Cairo university.Files were retrieved from the hospital archives by screening records from Jan 2014to Jan2019. Medical records were screened for data regarding the clinical manifestation and outcomeof hydrocephalus associated with autoimmune disease. Results: Case (1)Male patient 28 yrs old diagnosed a systemic lupus erythematosus The patient was controlled on low dose steroid and azathioprine. In 2019: the patient presented with headache and blurring of vision. Fundus examination showed grade 2 to 3 papilledema. MRI brain showed: Non obustrcutive hydrocephalus. MRA &amp; MRV: normal laboratory investigation was done and showed consumed c3 &amp; c4. ESR: 35 mm/hour. Hb: 12.3g/dl. WBC:6.38 cells/cm3. normal liver and kidney function. Neurosurgery consultation was done and recommended urgent ventriculo –peritoneal shunt. follow up fundus showed improvement of papilledema. The patient was discharged on steroids 30 mg and mycophenolate mofetyl: 2gram with improvement of his condition.Case (2)Female patient 22 yrs old diagnosed as systemic lupus erythematosus onset 2010. In 2018 she started to develop quadriparesis started in left side followed by Right side associated with attacks of Tonic colonic convulsions. MRI brain showed: Marked communicating hydrocephalus with markedly reduced cerebral hemisphere white matter. MRV &amp; MRA: normal. Her laboratory investigation showed ESR: 49 mm/h. HB: 10.3 mg/dl, WBC: 5.9 cells/ mm3. Normal platelet count. normal liver &amp; kidney function. Normal c3 &amp; c4. neurosurgery consultation was done. Urgent ventriculoperitoneal shunt was done with improvement of her condition. The patient was discharged on solupred 20 mg along with azathioprine. Conclusion: VPS along with medical treatment with steroids and immunosuppresion represent an effective treatment protocol. References: [1]Wei J, Yin H, Wang L, Cui L, Wang R. Systemic autoimmune diseases complicated with hydrocephalus: pathogenesis and management. Neurosurg Rev. 2019 Jun;42(2):255-261. [2]Wei J, Yin H, Wang L, Cui L, Wang R. Systemic autoimmune diseases complicated with hydrocephalus: pathogenesis and management. Neurosurg Rev. 2019 Jun;42(2):255-261. [3]Tang SC, Lee CF, Lee CW, Jeng JS. Systemic lupus erythematosus flare up manifestations as cerebral and subarachnoid hemorrhage. Lupus 2011; 20: 1211-13 [4]Kitching GB, Thompson JR, Hasso AN, Hirst AE. Angiographic demonstration of lupus cerebral phlebitis with communicating hydrocephalus. Neuroradiology 1977; 14: 59-63. [5]Mortifee PRS, Bebb RA, Stein H. Communicating hydrocephalus in systemic lupus erythematosus with antiphospholipid antibody syndrome. J Rheumatol 1992; 19: 1299-302. [6]Honda K, Matsumoto M, Kaneko T, KAmei I, Tatsumi H, Murai N, et al. Linear deposition of immunoglobulins and complement components on the dura in normal pressure hydrocephalus complicating systemic lupus erythematosus. J Clin Neurosci 2004; 11: 561-63. [7]Koga H, Ooch IN, Osato S, Ishida I, Hirakata H, Okuda S, Fujishima M (1994) Case report: Wegener’s granulomatosis accompanied by communicating hydrocephalus. Am J Med Sci 307:278–281 [8]Bertken RD, Cooper VR (1997) Wegener granulomatosis causingsellarmass,hydrocephalus,andglobalpituitaryfailure.WestJMed 1997; 167:44–47 [9]Scarrow AM, Segal R, Medsger TAJ, Wasko MC (1998) Communicating hydrocephalus secondary to diffuse meningeal spread of Wegener’s granulomatosis: case report and literature review. Neurosurgery 1998;43:1470–1473 Disclosure of Interests: None declared