Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0409974 (lupus)
 22,386 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A review of the literature regarding central nervous system side effects of the nonsteroidal anti-inflammatory drugs (NSAIDs) revealed three general categories: aseptic meningitis, psychosis, and cognitive dysfunction. Aseptic meningitis is found most commonly in patients with lupus treated with ibuprofen, but it should be considered in any patient with meningitis if the patient has used NSAIDs. Psychosis, although infrequently reported with NSAIDs, should be suspected in an elderly patient started on a regimen of indomethacin who acutely develops disorientation, paranoia, or hallucinations. Finally, there appears to be some potential for memory dysfunction and attention deficits in elderly patients treated with NSAIDs. Until further studies are available on the incidence and severity of these cognitive changes, physicians should use low doses of NSAIDs in the elderly and remain alert to the possibility of such adverse side effects.
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PMID:Central nervous system side effects of nonsteroidal anti-inflammatory drugs. Aseptic meningitis, psychosis, and cognitive dysfunction. 206 81

The authors present some literature and their own data on the antiphospholipid syndrome (venous and arterial thromboses; obstetric pathology: spontaneous abortions and intrauterine death of a fetus; CNS involvement: from headaches and disorientation to psychic disorders and cerebral infarctions; thrombocytopenia, early aseptic osteonecrosis and pulmonary hypertension). Altogether 58 patients with lupus erythematosus and 20 patients with a complicated obstetric history were examined. Lupoid anticoagulant and antibodies to cardiolipin were revealed in 27 patients. The determination of antiphospholipid antibodies was shown to be of practical value from the view-point of the knowledge of humoral immunity disorders in systemic lupus erythematosus, more detailed clinico-laboratory characterization of patients, the diagnosis and prediction of disorders of the blood coagulation system, and the development of obstetric pathology.
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PMID:[Clinical value of determining the levels of lupus anticoagulant and anti-cardiolipin antibodies]. 314 84

In summary, procainamide is a useful agent for suppressing premature depolarization frequency. Its short half-life of elimination requires a dosing frequency of every 3 hours with regular dosage forms or every 6-8 hours with a sustained action dosage. Because of the extreme unpredictability of plasma concentration, the dosage must be titrated in each patient with electrocardiographic monitoring serving as the most useful method of evaluating efficacy. Maximum and minimum plasma concentrations are helpful in monitoring the achievement of therapeutic plasma levels and adjusting the frequency of dosing, especially in the presence of impaired renal function or low cardiac output. Adverse effects of procainamide include anorexia, nausea, vomiting, fatigue, insomnia, visual hallucinations, and disorientation; these are minor and cease with discontinuation of the drug. Agranulocytosis has rarely been reported. Long-term treatment has resulted in the occurrence of a lupus-like syndrome that is reversible when the drug is stopped. Procainamide is excreted in breast milk and infants of mothers receiving procainamide should not be nursed.
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PMID:Pharmacokinetics of a sustained release procainamide preparation. 703 27

Involvement of the central nervous system associated with SLE (CNS lupus) is not rare. Two types of CNS lupus are noted clinically; one group manifesting focal neurological symptoms and another group showing mental symptoms. Though it is well known that neurological symptoms are caused by arteritis and thrombus, pathophysiological mechanisms leading to mental symptoms remain obscure and there is no established clinical evidence responsible for these symptoms. A 41-year-old woman was confused and her attention was markedly impaired. Her mental symptoms consisted of disorientation, restlessness, euphoria and emotional incontinence. There were neither focal neurological signs nor meningeal signs. Cerebrospinal fluid (CSF) examination showed increased number of polymorphonuclear cells and permeability of the blood-brain barrier, calculated based on the CSF/plasma protein ratio, was also elevated. Repeated bacteriological examinations revealed to be negative. Gd-DTPA MRI demonstrated diffuse enhancement of the cerebral leptomeninges. Methylprednisolone pulse therapy ameliorated her mental deterioration effectively, and subsequently the leptomeningeal enhancement with Gd-DTPA MRI disappeared in parallel. These radiological and laboratory findings suggest that SLE itself causes inflammation of vessels in the leptomeninges and adjacent cerebral cortex. We consider mental symptoms associated with SLE may be caused, at least in part, by this mechanism. To our knowledge, we could not find similar reports in the literature. Gd-DTPA enhanced MRI seems to be of clinical use for making diagnosis, evaluating clinical activity and understanding of CNS lupus.
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PMID:[Leptomeningeal abnormality on Gd-DTPA enhanced MRI in a case of SLE presenting diffuse organic brain syndrome]. 833 80

The patient was a 33 year female. In 2001, she was diagnosed with systemic lupus erythematosus (SLE) and treated with prednisolone and ciclosporin. In May 2006, she noticed slight muscle weakness in the bilateral lower limbs. In July of the same year, she experienced gait difficulty and was admitted to our hospital because of fatigue, appetite loss, fever and disorientation. Soon afterwards, she had a fit of general convulsion and suffered from urinary retention and fecal incontinence. A brain magnetic resonance image revealed atrophy of the thoracic cord in T2 weighted images, and cerebrospinal fluid examination showed high total protein and interleukin-6 concentration, indicating complication of lupus myelitis as well as cerebral involvement. Steroid pulse and oral prednisolone treatment resulted in ameriolation of cerebral complications such as disorientation and convulsion, but muscle weakness and paresthesia in the lower limbs and urinary retention persisted. Cyclophosphamide pulse therapy was started and resulted in a marked recovery from muscle weakness, paresthesia and urinary retention, and she could discharge. We conclude that steroid and cyclophosphamide pulse therapy for a SLE patient with CNS lupus and lupus myelitisis is effective for ameriolation of symptoms such as disorientation, convulsion, urinary retension, fecal incontinence, muscle weakness and paresthesia in the lower limbs as well as elevated level of serum anti-ribosomal P antibody.
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PMID:[Successful therapy with steroid and cyclophosphamide pulse for CNS lupus and lupus myelitis]. 1798 83

We describe a case of systemic lupus erythematosus (SLE) complicated with multifocal leukoencephalopathy (PML). A 57-year old woman, who had a five-year history of SLE, was admitted to our hospital because of fever and multiple subcutaneous nodules. Diagnosis of disseminated cryptococcosis was made based on histological and bacteriological examinations, and she was successfully treated with anti-fungal drugs. Corticoteroids were increased for persistent lupus activities. One month later, however, she gradually developed disorientation and short-term memory loss. A brain magnetic resonance image (MRI) showed a focal lesion in the white matter of the right frontal lobe. Brain biopsy demonstrated demyelinating lesions with the presence of JC viral antigen. Polymerase chain reaction also revealed JC virus DNA in the cerebrospinal fluid. Her condition gradually progressed, and she died a year later due to pneumonia. Although acquired immunodeficiency syndrome is currently the most common disease associated with PML, patients with autoimmune diseases receiving immunosuppressive therapy also have risks for developing PML. In patients with SLE presenting with subacute neurological abnormalities and white matter lesions in the brain, PML should be considered in the differential diagnosis.
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PMID:[A case of progressive multifocal leukoencephalopathy associated with systemic lupus erythematosus]. 1912 78

Dementia comprises several neurodegenerative disorders with similar neuropsychiatric features and Alzheimer's disease (AD) is the most common of them. Genetic factors are strongly implicated into its etiology especially for early-onset cases (EOAD) occuring before the age of 65. About 10% of these are inherited in autosomal dominant fashion via pathogenic polymorphisms in three genes- APP, PSEN-1, and PSEN-2. Despite genotypic clarity, however, phenotypic variability exists with different symptom constellations observed in patients with identical mutations. Below, we present a case of a 39-year-old male with a family history for early onset dementia who was referred to our department with anamnesis for abrupt behavioral change 7 months prior to hospitalization-noticeable slowing of speech and reactivity, impaired occupational functioning and irritability, followed by aphasic symptoms and transient episodes of disorientation. He was followed up for 2 years and manifested rapidly progressing cognitive decline with further deterioration of speech, apraxia, acalculia, ataxia, and subsequently bradykinesia and tremor. Based on the clinical and neuroimaging findings (severe cortical atrophy), familial EOAD was suspected and a whole exome sequence (WES) analysis was performed. It identified a heterozygous missense variant Leu424Val (g.71074C > G) in PSEN-1 gene considered to be pathogenic, and only reported once until now in a Spanish patient in 2009. Despite genotype identity however, distinct phenotypic presentations were observed in the two affected subjects, with different neuroimaging findings, and the presence and absence of seizures in the Spanish and Bulgarian case, respectively. Besides, myoclonus and spastic paraparesis considered "typical" EOAD clinical features were absent. Age of symptom onset was consistent with two of the reported mutations affecting 424 codon of PSEN-1 gene and significantly earlier than the other two implying that factors influencing activity of PSEN-1 pathological forms are yet to be clarified. Furthermore, our patient had co-occurring lupus erythematosus (LE) and we suggest that this condition might be etiologically linked to the PSEN-1 mutation. In addition to illustrating the symptomatic heterogeneity of PSEN-1 caused EOAD, our study confirms that in patients presenting with early cognitive deterioration and family history for dementia, WES can be especially informative and should be considered as a first-line examination.
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PMID:A Clinical Case of Patient Carrying Rare Pathological PSEN1 Gene Mutation (L424V) Demonstrates the Phenotypic Heterogenity of Early Onset Familial AD. 3192 Jul 35