Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0018681 (headache)
 56,091 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Lymphomas, both within and outside the central nervous system, are uncommon among patients with systemic lupus erythematosus (SLE). We describe a 58-year old Korean woman with SLE who presented with acute headache and confusion in the setting of prednisone and mycophenolate mofetil (MMF) therapy used to treat focal proliferative and membranous lupus nephritis. Three-dimensional brain magnetic resonance imaging (MRI) showed two peripherally ('ring') enhancing lesions within the basal ganglia, bilaterally, with associated mass effect and subfalcine herniation. A brain biopsy revealed an Epstein-Barr virus (EBV)-positive diffuse large B cell lymphoma. This is the first description of CNS lymphoma in a patient treated with MMF for lupus nephritis. While intracerebral lymphoma in the immunocompromised patient with lupus is rare, this disorder should be considered in the differential diagnosis of new-onset neurological symptoms among such patients.
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PMID:Central nervous system lymphoma associated with mycophenolate mofetil in lupus nephritis. 1633 85

A 33-year-old female who had been on a steroid treatment for the past 14 years due to systemic lupus erythematosus (SLE) visited our hospital complaining of mild headache. No neurological deficit and no positive serologic tests for lupus anticoagulants (LAC) and anticardiolipin antibodies (aCL) were noted. Only a mild inflammatory change was observed on routine hematological examination. On neuroradiological examination, MRI revealed thickened falx cerebri and tentorium cerebelli, and an empty delta sign. These findings were suggestive of sinus thrombosis of superior sagittal sinus (SSS). Angiograms clearly demonstrated occlusion of the posterior part of superior sagittal sinus and transeverse sinus (TS). Conservative treatment was chosen because of no evidence of intracranial hypertension. There was no deterioration in her general and neurological status during her hospital stay and she was discharged. Longstanding vasculitis and pachymeningitis related to lupus erythematosus might be the probable cause of the sinus thrombosis in this case.
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PMID:[Superior sagittal sinus thrombosis in a case of longstanding systemic lupus erythematosus]. 1648 23

Hydralazine was one of the first orally active antihypertensive drugs developed. Currently, it is used principally to treat pregnancy-associated hypertension. Hydralazine causes two types of side effects. The first type is an extension of the pharmacologic effect of the drug and includes headache, nausea, flushing, hypotension, palpitation, tachycardia, dizziness, and salt retention. The second type of side effects is caused by immunologic reactions, of which the drug-induced lupus-like syndrome is the most common, and provides clues to underscoring hydralazine's DNA demethylating property in connection with studies demonstrating the participation of DNA methylation disorders in immune diseases. Abnormalities in DNA methylation have long been associated with cancer. Despite the fact that malignant tumors show global DNA hypomethylation, regional hypermethylation as a means to silence tumor suppressor gene expression has attracted the greatest attention. Reversibility of methylation-induced gene silencing by pharmacologic means, which in turns leads to antitumor effects in experimental and clinical scenarios, has directed efforts toward developing clinically useful demethylating agents. Among these, the most widely used comprise the nucleosides 5-azacytidine and 2'deoxy-5-azacytidine; however, these agents, like current cytotoxic chemotherapy, causes myelosuppression among other side effects that could limit exploitation of their demethylating properties. Among non-nucleoside DNA demethylating drugs currently under development, the oral drug hydralazine possess the ability to reactivate tumor suppressor gene expression, which is silenced by promoter hypermethylation in vitro and in vivo. Decades of extensive hydralazine use for hypertensive disorders that demonstrated hydralazine's clinical safety and tolerability supported its testing in a phase I trial in patients with cancer, confirming its DNA demethylating activity. Hydralazine is currently being evaluated, along with histone deacetylase inhibitors either alone or as adjuncts to chemotherapy and radiation, for hematologic and solid tumors in phase II studies.
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PMID:Hydralazine target: from blood vessels to the epigenome. 1650

Cerebellar vasculopathy is an uncommon but clinically important neuropsychiatric syndrome of systemic lupus erythematosus (NP-SLE) for its ominous outcome and need for prompt interventions. We described a young Chinese lady with systemic lupus erythematosus and normal tension glaucoma, who had sudden headache, nausea and vomiting coupled with rapid neurological deterioration leading to comatose status. Diagnosis of lupus cerebellar vasculopathy was made and intense immunosuppressive therapy consisting of prednisolone and cyclophosphamide was commenced. Clinical condition was salvaged with marked improvement of both sensorium and general well-being.
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PMID:Cerebellum vasculopathy and normal tension glaucoma in systemic lupus erythematosus: report of a case and review of the literature. 1687 11

There are potential concerns regarding serotonin receptor agonists in SLE patients with migraine, particularly patients with concomitant Raynaud's syndrome. We estimated the prevalence of lupus-related headache and Raynaud's syndrome in the Montreal General Hospital SLE clinic cohort and evaluated the relationship between these two variables in multivariable logistic regression models, controlling for age, sex, race, SLE duration and the presence of lupus anticoagulant and antibodies to cardiolipin and beta2 glycoprotein I. We also assessed, through chart review in those individuals with both Raynaud's syndrome and migraine, a history of serotonin receptor agonist use, and any associated worsening vasospasm. Based on Systemic Lupus Activity Measure (SLAM) scores, the cumulative incidence of lupus-related headache in our sample (n = 391) was 46.1%; the prevalence of Raynaud's syndrome was 49.4%. The adjusted odds ratio (OR) for lupus-related headache and Raynaud's syndrome was 1.7 (95% CI 1.1, 2.5). In addition, there was a strong independent relationship between headache and anti-beta2 glycoprotein I antibodies (adjusted OR 5.6 [95% CI 1.8, 17.0]). The data from our chart review suggest that careful use of serotonin receptor agonists in patients with both Raynaud's syndrome and migraines may be undertaken, although caution would necessitate that these agents not be used in individuals with very severe Raynaud's (eg, digital ulcerations, and so on).
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PMID:Headache, Raynaud's syndrome and serotonin receptor agonists in systemic lupus erythematosus. 1712 May 94

We describe a 13-year-old systemic lupus erythematous (SLE) patient who presented with severe headache. The diagnosis of pseudotumor cerebri (PTC) was confirmed by an increased intracranial pressure and normal neuroimaging studies of the brain, including magnetic resonance (MR) venography. She later developed a Coombs positive anemia, lymphopenia, positive tests for antinuclear antibody (ANA) and anti-dsDNA and a migratory polyarthritis confirming the diagnosis of SLE. IgM type anticardiolipin antibodies were positive in low titer. Since she did not have a demonstrable thromboembolic phenomenon in neuroimaging studies, a diagnosis of antiphospholipid antibody syndrome could not be made and anticoagulant treatment was not given. Treatment with pulse i.v. methylprednisolone followed by oral treatment along with azathioprine produced a rapid and dramatic resolution of the clinical symptoms. PTC may also be a neurological manifestation of childhood SLE and should be considered in the differential diagnosis. We suggest that pulse steroids and azathioprine is an effective treatment for this feature.
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PMID:Systemic lupus erythematosus presenting with pseudotumor cerebri: a rare association. 1747 54

A male adolescent developed a sinovenous thrombosis 4 weeks following a Henoch-Schonlein purpura episode. A hypercoagulation evaluation revealed a positive lupus anticoagulant. This suggests an association between Henoch-Schonlein purpura and antiphospholipid antibody syndrome and is the first report of sinovenous thrombosis after Henoch-Schonlein purpura that was likely due to elevated antiphospholipids. Children who develop Henoch-Schonlein purpura with neurologic features including headache should be evaluated for sinovenous thrombosis and a hypercoagulable state.
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PMID:Lupus anticoagulant and thrombosis following Henoch-Schonlein purpura. 1750 70

We report the case of a 26-year-old woman who developed thrombophlebitis in her left leg in 2002, followed by fever, asthenia and headache in 2004. Antinuclear antibodies, antimitochondrial antibodies, anti-liver kidney microsome, anti-Smith, antiphospholipid (aPL) and antineutrophil cytoplasmic antibodies, as well as lupus- anticoagulant activity were positive. Systemic lupus erythematosus (SLE) with aPL syndrome was diagnosed and the patient was treated with azathioprine and heparin. Symptoms persisted and itching arose in the following months. The patient was admitted to our department in January 2005 for jaundice and skin rash. Elevated levels of acute phase proteins and cholestasis and liver necrosis indexes were present. Antinuclear antibodies, aPL and antimitochondrial antibodies (M5) antibodies were positive. Liver histology showed minimal focal hepatocyte necrosis, intrahepatic biliary stasis and intralobular inflammatory cell infiltrate. The absence of clinical signs that are characteristic of SLE as well as the failure to confirm antiSmith antibody positivity led us to rule out a diagnosis of SLE. On the basis of clinical, immunological and histological data, autoimmune intrahepatic cholangiopathy associated with primary aPL syndrome was diagnosed. The patient was treated with intravenous methylprednisolone followed by oral prednisone, warfarin and ursodeoxycholic acid. Liver necrosis and cholestasis indexes rapidly improved within 1 month and progressively reached the normal range. To our knowledge, this is the first description of a patient with an association of intrahepatic cholangiopathy and aPL, thus suggesting that autoimmune liver disease might associate with aPL syndrome.
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PMID:Autoimmune intrahepatic cholangiopathy associated with antiphospholipid antibody syndrome. 1787 18

We report a case of Rhupus with secondary anti-phospholipid syndrome who presented with headache and papilloedema due to cerebral venous thrombosis. We propose that an increased awareness about the condition and meticulous investigation of headaches in lupus can avert catastrophic outcomes.
Headache 2008 Jan
PMID:Headache in a case of Rhupus syndrome. 1818 99

Neuropsychiatric (NP) events are severe manifestations of systemic lupus erythematosus (SLE) and relate to poor outcome. The aims of this study are to investigate the NP manifestations of SLE and to identify the predictive factors for clinical outcome. There was a retrospective review of 240 hospital patients with primary NP events of SLE (NPSLE) from 1990 to 2004. Neuropsychiatric manifestations, SLE disease activity index (SLEDAI) score, System lupus International Collaborating Clinic/American College of Rheumatology Damage Index (SLICC/ACR-DI) score, magnetic resonance imaging (MRI) findings, treatment and mortality rate were included for analysis. From this group of patients, 15 NP syndromes were identified. The most frequent manifestation was headache, followed by seizure. The mean SLEDAI and SLICC/ACR-DI scores were 19.9 +/- 6.9 and 3.5 +/- 1.6, respectively. Abnormal MRI features were found in 67% (61/91) patients. At least one intrathecal (IT) injection of methotrexate (MTX) plus dexamethasone (DXM) was administered to 109 (45.4%) patients. High dose (1 g) intravenous methylprednisolone pulse therapy (IVMP) was administered to 167 (69.5%) patients. Multifactor analysis revealed that high SLICC/ACR-DI scores and sets of concurrent NP symptoms were independently associated with poor outcome, whereas pulse IVMP and IT injection of MTX plus DXM were protective factors against poor outcome. From our data, NPSLE is heterogeneous and is usually associated with high disease activity and organ damage scores. High SLICC/ACR-DI score and having more than two sets of NP symptoms are the predictors for poor outcome, whereas pulse IVMP and IT injection of MTX plus DXM can improve the prognosis.
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PMID:Clinical features and outcome of neuropsychiatric lupus in Chinese: analysis of 240 hospitalized patients. 1825 Jan 31


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