Intracranial hypertension in systemic lupus erythematosus.
ABSTRACT Intracranial hypertension (IH) mimicking pseudotumor cerebri (i.e., idiopathic IH) has been reported in individuals with systemic lupus erythematosus (SLE) since the 1960s. Although various mechanisms have been proposed (e.g., venous thrombosis, medication side effect, and immunologic or inflammatory disease) none have been proven to be causal.
Retrospective chart review of patients with IH and SLE at a single tertiary care institution.
In a cohort of IIH patients the percentage of patients in our study with SLE was 1% (4 in 410). Three out of the four patients had serologic evidence for a hypercoagulable state but only one had cerebral venous sinus thrombosis. Two patients had onset or worsening of IH in close temporal relationship to steroid withdrawal. The course, treatment, and prognosis of our patients with SLE and intracranial hypertension did not differ significantly from our clinical experience with IIH or with the published natural history data for IIH.
The association of SLE and IH may occur more than by chance alone. Steroid withdrawal in the treatment of the SLE may be a predisposing or precipitating factor in the development of IH in these patients rather than the inflammatory effects of SLE per se. The hypercoagulable state in some patients with SLE may also produce cerebral venous sinus thrombosis as an additional potential mechanism of IH.
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ABSTRACT: Idiopathic intracranial hypertension is a common disorder affecting mainly healthy, young, overweight women. The pathogenesis of this condition is unknown, but it has been shown to follow treatment with several compounds including corticosteroids and vitamin A derivatives. This paper will offer a novel hypothesis and insight on the pathogenesis of drug induced intracranial hypertension following a review and analysis of the literature. Both corticosteroids and vitamin A derivatives have been shown to upregulate the expression of aquaporin 1, a water channel protein. Aquaporin 1 is widely distributed in the human brain and is associated with water secretion into the subarachnoid space. Aquaporin 1 was also shown to participate in the regulation of weight. Agents used for treating idiopathic intracranial hypertension reduce aquaporin 1 expression. Based on these observations, we propose that aquaporin 1 has a pathogenetic role in drug induced idiopathic intracranial hypertension. Over expression of this gene causes increased intracranial pressure, and downregulation reduces pressure and alleviates the symptomatology and complications of idiopathic intracranial hypertension.Medical Hypotheses 10/2013; 81(6). DOI:10.1016/j.mehy.2013.10.002 · 1.15 Impact Factor