Normal pressure hydrocephalus in the spectrum of neurological complications of systemic lupus erythematosus.

ABSTRACT

Normal pressure hydrocephalus is an unusual manifestation of systemic lupus erythematosus and its pathogenesis is still unclear. We report the case of a 39-year-old white woman with systemic lupus erythematosus who developed magnetic gait, speech difficulties, progressive memory impairment, urinary incontinence and episodes of involuntary closure of the eyelids. Signs and symptoms, associated with ventriculomegaly and normal cerebrospinal fluid pressure, suggested normal pressure hydrocephalus, which as a complication of systemic lupus erythematosus believably develops due to the insidious inflammatory process that occurs in the meningeal tissues or to the vasculitis itself. Normal pressure hydrocephalus tends to develop secondary to trauma, infection or subarachnoid haemorrhage, but in 50 % of patients no aetiology is found. Shunt surgery is the only effective treatment, specifically for the gait disorder, which usually improves more than the cognitive symptoms. Since the tap-test showed a strongly positive result, a medium pressure ventriculoperitoneal shunt was inserted, further replaced by a high pressure one in view of the complications, with less than expected improvement. Subdural hematomas and empyemas developed, requiring surgery and antibiotic therapy. A new tap-test was positive, and the patient improved only after a programmable valve was finally placed. However, pressure regulation shall be continuously required, and shunt dysfunction might still develop in the long term. The few similar case reports in the literature are reviewed, confirming the rarity of this neurological complication of systemic lupus erythematosus.