Cerebral venous thrombosis presenting with subdural haematoma as first presentation for systemic lupus erythematosus with negative antiphospholipid antibodies

Abstract

We report the case of a 30-year-old woman, without any previous comorbidities presenting with acute onset headache, altered sensorium and unsteadiness of gait. Neurological evaluation revealed a drowsy patient with papilloedema, bilateral lateral rectus palsy, generalised hyper-reflexia and up going plantar responses. Urgent imaging performed showed extensive cortical venous sinus thrombosis. Workup for secondary causes of cortical venous sinus thrombosis revealed very high titres of antinuclear antibody and anti-dsDNA, but negative antiphospholipid antibodies (APLA). In hospital she started developing other complications of systemic lupus erythematosus (SLE). Urine evaluation revealed proteinuria and granular casts suggestive of glomerulonephritis. Cardiac evaluation revealed moderate pericardial effusion. We have discussed neurolupus as initial presentation of SLE and the rare occurrence of major neurovascular complications without secondary APLA syndrome.

Cerebral venous sinus thrombosis and subdural hematoma in a female patient with systemic lupus erythematosus: a case report and literature review

“…However, CVST with SDH in SLE is even rarer. There is only one case report of a patient with SLE who had CVST and SDH reported in literature (8). Both the previous patient and this patient had high SLE activity and reported negative for anticardiolipin antibodies.…”

Atypical Presentations of Systemic Lupus Erythematosus by Cerebral Venous Thrombosis and Generalized Lymphadenopathy without Antiphospholipid Antibodies: Case Report

“…Of reported cases of CVT as the initial presentation of SLE, complaints of persistent headache were frequently noted up to 73.3-95% of the time [7,13]. Other neurological ndings including seizures (20-75%) [7,13], visual eld defects (40%) [13], altered level of consciousness (26.7-57.5%) [7,13], nausea and/or vomiting (20-82.5%) [7,13], focal weakness (52.5%) [7], and papilledema [7,9,17] presented in variable frequencies [7][8][9][10][13][14][15][16][17]. Our patient presented with similar complaints of progressive, throbbing headaches and visual prodrome.…”

“…When CVT does occur as a neurocomplication of SLE, it tends to manifest years to decades after diagnosis of SLE [11,14], possibly related to a developing hypercoagulable state or concomitant antiphospholipid antibody (APLA) syndrome [6]. CVT as the initial presentation of SLE is even more rare, with limited case reports published describing this occurrence [7][8][9][10][13][14][15][16][17][18][19][20]. Out of these documented cases, CVT presentations were mixed, the occurrence of concomitant APLA syndrome was varied, and "classic" clinical SLE manifestations of arthritis, serositis, or other cutaneous features are often secondarily elucidated to the patient's primary neurological manifestations [7][8][9][10][13][14][15][16][17][18][19][20].…”

“…CVT as the initial presentation of SLE is even more rare, with limited case reports published describing this occurrence [7][8][9][10][13][14][15][16][17][18][19][20]. Out of these documented cases, CVT presentations were mixed, the occurrence of concomitant APLA syndrome was varied, and "classic" clinical SLE manifestations of arthritis, serositis, or other cutaneous features are often secondarily elucidated to the patient's primary neurological manifestations [7][8][9][10][13][14][15][16][17][18][19][20]. Although the association between CVT and SLE is becoming better understood, it is not currently part of SLICC nor ACR neurological criteria [1][2].…”