Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UNIPROT:P50583 (asymmetrical)
 12,197 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Cerebral infarction is an uncommon complication in multiple myeloma with hyperviscosity. Serum hyperviscosity may cause a variety of clinical manifestations including bleeding from mucosal membranes, congestive heart failure, retinopathy, and various neurologic deficits. These manifestations have been attributed to the presence of large quantities of asymmetrical molecules of high molecular weight in the serum. We recently experienced a case of multiple myeloma with acute cerebral infarction, which caused by hyperviscosity, as an initial manifestation in IgG multiple myeloma, and reviewed the relevant literature of myeloma presenting with the stroke. A 68-yr-old woman abruptly developed hypesthesia and monoplegia in the left leg. The stroke confirmed by the brain MRI and MR angiography, which revealed acute infarction at the right anterior cerebral artery territory. On admission, routine blood tests showed a slight decrease in hemoglobin and a marked increase in erythrocyte sedimentation rate. Peripheral blood smear, serum protein electrophoresis, serum visocity, and bone marrow aspiration showed that she had IgG multiple myeloma with hyperviscosity. She was treated by chemotherapy with cyclophosphamide and discharged with the improved clinical condition.
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PMID:Cerebral infarction in IgG multiple myeloma with hyperviscosity. 1610 Apr 71

A 9-year-old Japanese girl received a cadaveric dura mater graft during surgery following a head injury with brain contusion. She continued to do well, but when she became 19-years-old, she gradually showed a violent character and was treated in a psychiatric hospital. Another 6 years later, 200 months after the procedure, she developed a progressive gait ataxia, which subsequently led to her death within 10 months of onset. An autopsy showed she had CJD. This patient represents an atypical case of dura-associated CJD (dCJD) with unusual clinicopathological features including the late occurrence of myoclonus, an absence of periodic synchronous discharges in the electroencephalogram, and the presence of widespread florid plaques. However, our detection of an asymmetrical increase in the MRI-derived images of pulvinar nuclei has not been previously observed in other atypical cases of dCJD. Because atypical dCJD cases share several clinicopathological features with those of vCJD, and because asymmetrical hyperintense signals in the pulvinar have been observed in some neuropathologically confirmed vCJD cases, we had some difficulty in a differential diagnosis between atypical dCJD and vCJD. This is the first atypical dCJD case showing a pulvinar high signal compared with all other basal ganglia on MRI.
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PMID:Increased asymmetric pulvinar magnetic resonance imaging signals in Creutzfeldt-Jakob disease with florid plaques following a cadaveric dura mater graft. 1652 84

Cardiac amyloidosis typically presents with diastolic heart failure, but asymmetrical septal hypertrophy with outflow tract obstruction has been described. We illustrate the case of a 71-year-old woman with biopsy-proven cardiac amyloidosis and severe medical comorbidities with refractory severe heart failure who had asymmetric septal hypertrophy, systolic anterior motion (SAM) of the mitral valve, and a resting left ventricular outflow tract gradient of 86 mm Hg, increasing to 102 mm Hg on Valsalva maneuver. She underwent percutaneous transluminal septal myocardial ablation (PTSMA) with a dramatic resolution of her SAM and outflow tract obstruction, confirmed by intracavitary pressure wire measurements. PTSMA is technically feasible in this context, and correction of outflow tract obstruction may represent a new therapeutic target in cardiac amyloidosis.
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PMID:Use of percutaneous transluminal septal myocardial ablation for relief of outflow tract obstruction in cardiac amyloidosis: a novel therapeutic target. 1696 77

A 14-year-old girl with aortitis syndrome in the early pre-pulseless phase was admitted to our hospital because of slight fever, neck bruit, asymmetrical blood pressure, stenosis or dilatation of the main branch arteries in aorta on chest computed tomography. Laboratory examination revealed a high level of C-reactive protein and an elevated erythrocyte sedimentation rate, as well as hypergammaglobulinemia, and 18F-FDG-PET revealed an accumulation of 18 fluorodeoxyglucose in the great vessels. She was first given pulse therapy with a combination of methylprednisolone and intravenous cyclophosphamide, and then maintenance therapy with oral prednisolone and azathioprine. All the abnormal laboratory parameters improved to normal levels within a month. We suggest that early diagnosis of aortitis syndrome may permit early treatment in the early pre-pulseless phase and could possibly prevent progression to the occlusive phase.
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PMID:[A case of aortitis syndrome diagnosed with 18F-fluorodeoxyglucose-positron tomography (18F-FDG-PET) in the early pre-pulseless phase]. 1760 61

A 83-year-old woman presented with a 25-year history of hypertension which was long-standing, uncontrolled, severe hypertension because of irregular oral administration of antihypertensive drug underwent an echocardiographic examination as part of an evaluation of hypertension. She described chest distress associated with activity, syncope for three times in the past one year. On physical examination, she was in no acute distress, with a regular pulse rate and blood pressure of 185/115 mmHg. On auscultation, her lung fields were clear. There was a III/VI late peaking crescendo/decrescendo systolic murmur along the left sternal border radiating to the apex, which increased with standing and Valsalva's maneuver and decreased with squatting. There was no report of provocative maneuvers performed during auscultation. There was no edema. Transthoracic echocardiography showed a hyperdynamic left ventricle with markedly increased left ventricular wall thicknesses and asymmetrical septal hypertrophy. M-mode echocardiography showed systolic anterior motion of the mitral valve apparatus and midsystolic closure of the aortic valve. A dynamic left ventricular outflow tract obstruction was present, with a resting maximal instantaneous gradient of 55 mmHg. With the Valsalva maneuver, the gradient increased to 114 mmHg. No any factors that could cause hypertension were found at kidney, adrenal gland and renal artery etc. by ultrasound and multislice compute tomography.
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PMID:Valsalva maneuver, uncontrolled hypertension, asymmetric septal hypertrophy and dynamic outflow obstruction: a case report. 1915 78

Polymicrogyria (PMG) is a relatively common malformation of the cortex for which the pathogenesis remains poorly understood. Both acquired and genetic causes are known, and to date more than 70 cases of PMG have been associated with chromosomal abnormalities. Here we report on a 12-year-old girl presenting with asymmetrical PMG predominantly affecting the right occipital lobe. She was the only child of consanguineous parents. At 7 years of age she was referred for mental retardation with speech delay and seizures. Cytogenetic studies of the patient revealed an inverted 9p duplication/deletion and bacterial artificial chromosomes (BACs)-array also showed a 22q11.2 microduplication confirmed by quantitative PCR. This case is of interest in the search for candidate genes and emphasizes the importance of the 22q11 region in PMG. It also highlights the efficiency of BACs-array in detecting complex rearrangements.
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PMID:Polymicrogyria in a child with inv dup del(9p) and 22q11.2 microduplication. 1921 26

A 21-year-old woman was admitted to our unit with suspected infective endocarditis. Transthoracic and transoesophageal echocardiogram demonstrated vegetation in a parachute-like asymmetrical mitral valve with severe mitral regurgitation. She was completely asymptomatic before this presentation. Though there was no evidence of mitral stenosis, this deformity is associated with transvalvular turbulence, which would account for the increased likelihood of infective endocarditis. She underwent a prosthetic mitral valve replacement with a 21 mm ATS mechanical valve.
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PMID:Infective endocarditis in a parachute-like asymmetrical mitral valve. 1926 90

Eosinophilic meningoencephalitis (EME) has been described in various species of animals and in humans. In dogs it has been associated with protozoal infections, cuterebral myiasis and various other aetiologies. Ten cases of idiopathic eosinophilic meningoencephalitis have been reported in dogs and one in a cat where the origin was uncertain or unknown. The dogs were all males, of various breeds but with a predominance of Golden Retrievers and Rottweilers; they generally had a young age of onset. Two cases with no apparent underlying aetiology were diagnosed on post mortem examination. The 18-month-old, male Boerboel presented with sudden onset of cerebellar ataxia, as well as various asymmetrical cranial nerve deficits of 2 weeks' duration and without progression. Haematology revealed a peripheral eosinophilia. Necropsy showed extreme generalised congestion especially of the meninges and blood smear and histological sections of various tissues showed intravascular erythrocyte fragmentation with the formation of microcytes. Histopathology revealed severe diffuse cerebrocortical subarachnoidal meningitis and submeningeal encephalitis, the exudate containing variable numbers of eosinophils together with neutrophils and mononuclear cells. There was also deeper white matter and hippocampal multifocal perivascular mononuclear encephalitis and multifocal periventricular malacia, gliosis and phagocytosis of white matter. The cerebellum, brain stem and spinal c showed only mild multifocal oedema or scattered occasional axon and myelin degeneration respectively, with no inflammation. Immunohistochemical staining of central nervous tissue for Toxoplasma gondii failed to show any antigen in the central nervous tissue. Ultrastructure of a single submeningeal suspected parasitic cyst showed it to be chromatin clumping within a neuron nucleus indicating karyorrhexis. Gram stain provided no evidence of an aetiological agent. The 3-year-old Beagle bitch had a Caesarian section after developing a non-responsive inertia 8 days prior to presentation. This animal's clinical signs included status epilepticus seizures unrelated to hypocalcaemia and warranted induction of a barbiturate coma. She died 4 hours later. Post mortem and histopathological findings in the brain were almost identical to those of the Boerboel and she also showed histological evidence of recent active intravascular haemolysis with microcyte formation. Rabies, distemper and Neospora caninum immunohistochemical stains were negative in the brains of both dogs. Immunohistochemical staining of the cerebral and meningeal exudates of the Beagle for T- and B-lymphocyte (CD3 and CD79a) markers showed a predominance of T-lymphocytes with fewer scattered B lymphocytes. A possible allergic response to amoxicillin/clavulanate is considered, as this appeared to be the only feature common to the recent history of both animals. An overview of EME in humans, dogs and cats is given and the previously published cases of idiopathic EME in dogs and the single published cat case are briefly reviewed.
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PMID:Review of idiopathic eosinophilic meningitis in dogs and cats, with a detailed description of two recent cases in dogs. 1949 20

Churg-Strauss syndrome (CSS), also known as allergic granulomatous angiitis, is a rare disorder characterized by the presence of asthma, eosinophilia and small-to-medium sized vessels vasculitis. Vasculitis commonly affects lung, heart, skin and peripheral nerves. The authors report a case of a 47-year-old woman that was admitted with a 1-week history of painful purpuric lesions affecting extensor surfaces of lower extremities. She had a 7 years-long medical history of asthma, paranasal sinusitis and allergic rhinitis. Laboratorial tests revealed leucocytosis, peripheral blood eosinophilia and elevated inflammatory parameters. Antineutrophil cytoplasmic antibodies were negative. Skin biopsy was consistent with necrotizing vasculitis with perivascular eosinophil-rich infiltrate. Electromyogram and nerve conduction studies showed asymmetrical peripheral polyneuropathy. Churg-Strauss syndrome diagnosis was supported by clinical and histopathological findings. High-dose oral steroid therapy led to a dramatic clinical improvement. Skin manifestations can be the presenting feature of Churg-Strauss syndrome. Early recognition is essential for appropriate treatment and prevention of irreversible organ damage.
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PMID:[Churg-Strauss syndrome presenting with cutaneous vasculitis]. 1956 84

We report on a 12-year-old patient from Congo who presented acute chorea following cardiac surgery for poststreptococcal mitral valvulopathy. She showed severe and asymmetrical chorea, associated with motor impersistence and agitation. Biological investigations disclosed inflammatory signs and brain MRI was normal. Due to the negative results of the biological and morphological investigations, the diagnosis of Sydenham chorea was suspected. High doses of oral steroids resulted in a dramatic improvement of the chorea as well as the behavior disturbance within 1 month. Sydenham chorea is not an unusual complication of rheumatic fever. Usually, patients develop chorea a few weeks after beta-hemolytic streptococcal pharyngitis. Details on its pathophysiology remain to be determined. Our case highlights its possible onset in the postoperative period if alternative etiologies of infantile chorea have been excluded.
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PMID:[Acute poststreptoccocal chorea: an atypical postoperative reaction following cardiac surgery for mitral valvulopathy]. 1957 9


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