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- W2038454911 abstract "To the Editor, Spontaneous intracranial hypotension (SIH) is typically characterized by traction headaches. The most common pathological mechanism is cerebrospinal fluid (CSF) leakage after lumbar puncture, spine/spinal cord trauma, or an unidentified cause [1]. We present a case of SIH with systemic lupus erythematosus (SLE), where SIH symptoms synchronized with SLE activity. A 32-year-old woman was admitted to a hospital with gait disturbance and cognitive dysfunction in September 2012. In October 2004, she was diagnosed with SLE because of facial erythema, photosensitivity, oral ulcers, positive serum antinuclear antibody, elevated anti-double-stranded DNA antibody, polyarthritis, and leukocytopenia. Her symptoms receded after prednisolone treatment. However, proteinuria and hematuria first appeared in March 2009 after she became pregnant. Artificial abortion and renal biopsy were performed in February 2009. The histological diagnosis revealed lupus nephritis Class III +V (WHO classification). She was treated with prednisolone, immunosuppressants, and immunoabsorption; however, she presented with orthostatic headache and nausea in October 2011, and SLE exacerbation, such as increase of proteinuria, positive serum antinuclear antibody, elevation of anti-double-stranded DNA antibody, and leukocytopenia, was found to be associated with her poor treatment adherence. Magnetic resonance imaging (MRI) revealed dural thickening and subdural hygroma, suggesting SIH. However, she had no previous history of lumbar puncture or trauma, and MR myelography did not reveal the location of CSF leakage. After 1 year of follow-up, gait disturbance and cognitive dysfunction appeared in August 2012. MRI re-examination revealed worsening subdural hygroma and lateral ventricle narrowing (Figure 1a). MR myelography suggested CSF leakage at C1–3 levels (Figure 1b). After admission, prednisolone was increased from 20 to 30 mg/day, combined with cyclosporine 100 mg/day and mizoribine 150 mg/day following one course of methylprednisolone pulse therapy (500 mg/day) for three consecutive days. Immunoabsorption was performed weekly. These therapies attenuated serological SLE indicators. Although her headache and cognitive dysfunction improved, she transferred to another hospital's neurosurgical department to determine the requirement of autologous blood injection into the epidural space in October 2012. Her CSF pressure decreased to 10 mm H2O, fulfilling the criteria for intracranial hypotension. However, CSF leakage was not confirmed using CT myelography or radioisotope cisternography. Then, she did not undergo autologous blood injection into the epidural space. CSF leakage possibly stopped before she was transferred. CSF leakage was not confirmed using MR myelography in December 2012 (Figure 1c), and MRI in July 2013 revealed improvements of subdural hygroma and lateral ventricle narrowing (Figure 1d)." @default.
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- W2038454911 date "2014-06-16" @default.
- W2038454911 modified "2023-09-23" @default.
- W2038454911 title "Spontaneous intracranial hypotension in a case of systemic lupus erythematosus" @default.
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- W2038454911 doi "https://doi.org/10.3109/14397595.2014.924469" @default.
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