Pseudotumor cerebri and leukoencephalopathy in childhood lupus
Department of Neurosciences, King Faisal Specialist Hospital and Research Centre, Riyadh, Kingdom of Saudi Arabia.Lupus (Impact Factor: 2.2). 02/1999; 8(1):81-4. DOI: 10.1191/096120399678847317
We describe an adolescent with systemic lupus erythematosus (SLE) and pseudotumor cerebri (PTC) associated with diffuse white matter lesions (leukoencephalopathy) on neuroimaging studies. Although the association between SLE and PTC has been reported previously in 21 cases, the findings of leukoencephalopathy is known in only one other patient.
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ABSTRACT: Systemic lupus erythematosus (SLE) remains a challenging autoimmune disease in term of its etiology, pathogenesis, and management. Much progress has been made in the past year in searching for the SLE susceptibility genes, particularly by several genome-wide screening groups. Cumulative evidence about the association of infections and hormones with SLE has been gathered. Researchers believe that childhood SLE involves more severe organ involvement than adult SLE. Central nervous system complicated lupus continues to be problematic because functional imaging can be abnormal in otherwise asymptomatic lupus individuals. Whether these abnormalities result from subclinical central nervous system involvement or from false positives remains to be determined. With the wide use of corticosteroids as a cornerstone therapy for major organ involvement in childhood SLE, potential complications, especially those involving the growing bone or osteoporosis, are a cause of concern. Evidence suggests that regular exercise, as well as calcium and vitamin D supplementation, may help alleviate bone complications. Researchers have also updated information about pediatric antiphospholipid antibody syndrome. Follow-up studies on neonatal lupus and its pathogenesis have progressed, leading to a better understanding of its natural history and, in turn, to proper counseling of mothers of infants with neonatal lupus and of women with positive anti-Ro or anti-La antibodies. Drug-induced lupus in children is not uncommon. Minocycline and zafirlukast have been increasingly used, and were reported to induce lupus in children.
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ABSTRACT: The diagnosis of PTC is usually not difficult. Provided that the history, examination, neuroimaging studies, and lumbar puncture are all consistent with PTC, the diagnosis can be made easily. The elimination of "look-alikes" should be made with appropriate testing. After the diagnosis is made, patients should be observed closely in terms of their visual field and visual acuity until it is apparent that these are stable. Patients who have progressive loss of visual acuity or visual field should be treated promptly. An ophthalmologist should be involved in the care of these patients along with a neurologist or neurosurgeon, because visual loss may occur either early or late in the disease process and may be insidious. The most effective treatments are weight loss, carbonic anhydrase inhibitors, diuretics, and, if necessary, a short course of steroids. Blood pressure and IOP should be followed closely. If visual loss occurs despite these maneuvers, some type of shunt procedure, preferably optic nerve sheath decompression, is recommended. There seems to be no reason to treat the asymptomatic patient with PTC.
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ABSTRACT: To identify the pathogenetic mechanisms of central nervous system (CNS) syndromes of systemic lupus erythematosus (SLE) as described in the literature. Using PUBMED, we performed a systematic search of publications from 1980 onwards. Studies were eligible if they had been performed on patients or material from patients with CNS manifestations and definite SLE and when the CNS manifestations were not secondary. Criteria were formulated for the identification of pathogenetic mechanisms. The single most important cause of the CNS syndromes of SLE is ischaemia due to narrowing or occlusion of small vessels, arteries and veins. Antiphospholipid antibodies and premature atherosclerosis play roles in these processes, but they have not been delineated definitely. Intracranial and intraspinal haemorrhages are much less frequent than ischaemia and are presumably in part due directly to SLE. Vasculitis may cause ischaemia or haemorrhage in the CNS and is involved occasionally, as shown by imaging and histological findings. White matter damage is heterogeneous and ill-understood. It includes white matter degeneration and myelin vacuolation of the spinal cord, and reversible leucoencephalopathy due to oedema. Antibody-induced neuronal dysfunction in the CNS is a realistic hypothesis and may involve anti-ribosomal P antibodies and several other antibodies. Deficiency of psychological reactions forms a separate and entirely different category of mechanisms. Causes have been identified or possible causes have been suggested for most of the CNS syndromes of SLE, thus offering rationales for different forms of prevention and therapy.