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 36-year-old female with a history of recurrent pregnancy loss experienced sudden onset of disturbance in consciousness, with right hemiparesis and total aphasia. Computed tomography revealed a massive hemorrhage in the left frontal lobe, and angiography showed occlusion of the anterior two-thirds of the superior sagittal sinus. Laboratory investigations detected the presence of lupus anticoagulant, elevation of the anticardiolipin beta 2-glycoprotein I complex antibody level, and a decreased protein S activity level. There were no underlying conditions, such as connective tissue disorders, malignancies, infectious diseases, and drug-induced disorders, so the diagnosis was primary antiphospholipid syndrome. Primary antiphospholipid syndrome should be considered in the evaluation of patients with "idiopathic" or "primary" sinus and cerebral venous thrombosis.
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PMID:Superior sagittal sinus thrombosis associated with primary antiphospholipid syndrome--case report. 954 Mar 31

Neuropsychiatric lupus includes extremely diverse clinical manifestations, ranging from mild cognitive dysfunction to a severe, life-threatening presentation. We report a 28-year-old patient with systemic lupus erythematosus who had persistent fever for 3 months, and developed within a few hours motor and sensory aphasia, rotator nystagmus with deviation of the eyes, and severe nuchal rigidity. An extensive series of imaging and laboratory tests were interpreted as normal, except for an elevated opening pressure at lumbar puncture, cerebrospinal fluid inflammatory findings, and asymmetrical cortical perfusion on single-photon emission computed tomography. The patient received one course of high-dose intravenous immunoglobulin (IVIg) and within 5 days her condition returned to that of 3 months before admission. The mechanisms of injury, along with the management of cerebral lupus and the mechanisms of action of IVIg, are discussed.
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PMID:Successful treatment of systemic lupus erythematosus cerebritis with intravenous immunoglobulin. 1035 27

Bilateral paramedian thalamic infarction is rare. The suggested mechanism is occlusion of a central unpaired thalamic perforating artery--an anatomic variant. In the few existing reports of this condition, the diagnosis was based on computed tomography (CT) or magnetic resonance imaging (MRI) findings alone. Other causes of thalamic lesions were not ruled out, and there was no angiographic demonstration of the presumed variant artery. We present a case of a 48-year-old man with a bilateral thalamic infarction seen on CT and MRI. Initial neurological examination revealed lethargy, severe combined motor and sensory aphasia, and a mild upward gaze limitation. The patient had no focal motor deficits. After 24 hours, the patient was more alert and his speech became more fluent, but Korsakoff-type amnesia with poor attention span became apparent. The patient improved slowly over 6 months of rehabilitation. Bilateral thalamic lesions can be caused by several conditions. Among those are thiamine deficiency, cerebral lupus, toxoplasmosis, cysticercosis, cerebral syphilitic gumma, and even tumors and fungal infections. All these were ruled out in our case. Superselective digital subtraction angiography (DSA) demonstrated a single unpaired thalamic perforator. To our knowledge, this is the first time this anatomical variant has been demonstrated in vivo in association with bilateral thalamic infarction.
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PMID:Bilateral paramedian thalamic infarct in the presence of an unpaired thalamic perforating artery. 1195 45

Non convulsive status epilepticus is a heterogeneous condition consisting of very different electroclinical syndromes. It is difficult to make the diagnosis and identify common factors among patients. We report two cases with an unusual presentation. A 31 years old woman having discoid lupus presented with a prolonged exogenous psychosis that lasted two and half months, associated to echolalia. After the episode the patient remained with a severe frontal syndrome that could be the consequence of a non convulsive status epilepticus. A 60 years old woman with an epilepsy diagnosed at the age of 30, presented with recurrent episodes of aphasia. During one of these crises, the electroencephalogram showed continuous epileptiform activity.
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PMID:[Non convulsive status epilepticus: an heterogeneous disease with a difficult diagnosis. Report of 2 cases with unusual presentation]. 1463 93

Aneurysms arising from a lenticulostriate artery (LSA) are uncommon. Their causes include hypertension, moyamoya disease, infection, systemic lupus erythematosis, and flow-related saccular aneurysms. Options for treating these aneurysms are limited. The authors present a case in which an LSA aneurysm was identified in a 69-year-old woman with no significant medical history, who experienced a sudden onset of right hemiparesis and aphasia due to a basal ganglia hemorrhage. The different causes and treatment options available for these rare and difficult-to-treat aneurysms are discussed.
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PMID:Surgical treatment of a lenticulostriate artery aneurysm. Case report. 1508 44

A 44-year-old right-handed Caucasian male was initialy diagnosed in 2007 with dermatomyositis (DM) and in 2009 with systemic lupus erythematosus (SLE) (overlap syndrome). He was treated with Methylprednisolone and Hydroxychloroquine. He interrupted the treatment in the last three years. The patient presented with fever (39.8 degrees C), left zoster ophthalmicus, headache and psychomotor agitation. The cerebral CT scan showed left hemispheric hypodense lesions. Herpetic encephalitis was suspected. The patient was referred to the Institute of Infectious Diseases. The patient's neurological status worsened, he presented spastic tetraparesis and aphasia. DW-MRI, ADC, DS and AngioMRI were done, the patient proved to have an ischemic stroke due to acute thrombosis of the left internal carotid artery and multiple watershed infarctions. An infectious pathology, including HSV-1, was excluded by PLEX ID performed from CSF. Acyclovir, anti vitamin K, steroidal intravenous pulse therapy was started. The patient was referred after two weeks to the Department of Neurology. Mild inflammatory syndrome, tests for anti-double stain DNA (dsDNA), anti-Sm, anti-SSA, IgM and IgG anti-cardiolipin antibodies and lupus anticoagulant were positive. He was currently treated with Methylprednisolone (48 mg/d), anti vitamin K, statin, symptomatics. The outcome was favorable, with good laboratory response. Overlap syndrome may be associated with a significant increase in the risk of stroke. Our case presented without clinically susceptible symptoms of stroke but found to have stroke after neurological assessment associated with overlap syndrome (DM and SLE).
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PMID:Ischemic strokes in a young patient with dermatomyositis, systemic lupus erythematosus and secondary antiphospholipid syndrome mimicking herpetic encephalitis. 2550 61