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The bm12 Inducible Model of Systemic Lupus Erythematosus (SLE) in C57BL/6 Mice
Published on: November 1, 2015
A severe case of systemic lupus erythematosus with cerebral involvement
M Wiedmann1, W Seidel, L Mende
1Department of Internal Medicine II (Gastroenterology), Center of Internal Medicine, University of Leipzig, Leipzig, Germany. wiedm@medizin.uni-leipzig.de
Insights
This case study details a severe systemic lupus erythematosus case in an 18-year-old female, complicated by bleeding and neurological issues. The patient experienced a challenging recovery despite intensive treatment for lupus and its complications.
Area of Science:
- Neurology
- Rheumatology
- Neurosurgery
Background:
- Systemic lupus erythematosus (SLE) can present with severe multi-organ involvement.
- Neurological complications, including seizures and intracranial hemorrhage, are serious manifestations of SLE.
Observation:
- An 18-year-old female with severe SLE developed intracerebral bleeding requiring neurosurgical intervention.
- Postoperative complications included rebleeding, disseminated intravascular coagulation, platelet dysfunction, and hydrocephalus requiring a ventriculo-peritoneal (VP) shunt.
- Catastrophic antiphospholipid syndrome was suspected but unconfirmed.
Findings:
- Treatment involved plasmapheresis, cyclophosphamide, corticosteroids, and VP shunt placement.
- The patient had a slow, incomplete recovery with persistent cachexia and amaurosis.
- A complication of an intraperitoneal VP shunt cyst necessitated surgical revision.
Implications:
- This case highlights the complex management challenges in severe SLE with neurological and bleeding complications.
- Aggressive immunosuppressive therapy and neurosurgical interventions may be required.
- Long-term sequelae and management of complications like VP shunt issues are critical considerations.
Abstract:
We describe an 18-year-old girl who presented with severe systemic lupus erythematosus with multiple organ involvement. The disease was further complicated by recurrent seizures and intracerebral left parieto-occipital bleeding that required neurosurgical treatment. Postoperative rebleeding occurred due to disseminated intravascular coagulation and platelet dysfunction. Catastrophic antiphospholipid syndrome was suspected, but could not be confirmed during follow-up. Additional treatment with plasmapheresis and intravenous pulse cyclophosphamide in combination with corticosteroids was started. Liquor drainage via a ventriculo-peritoneal (vp)-shunt was necessary because of a hydrocephalus malresorptivus. The patient's recovery was slow and incomplete (cachexia and amaurosis persisted). Follow-up was further complicated by an intraperitoneal vp-shunt cyst, which was initially treated conservatively, but finally had to be revised operatively.
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