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Query: UMLS:C0409974 (lupus)
 22,386 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The objective of the study was to examine the clinical features of lupus patients who present with transverse myelopathy (TM) and ascertain functional outcomes when treated early with high dose corticosteroids and/or cyclophosphamide. Case records of nine patients who developed a total of 14 episodes of TM were retrospectively studied. All the patients were female and their ages ranged from 21 to 59 years. Nine episodes of paraparesis, three of tetraparesis, one of numbness and one of neurogenic bladder were reported early in the diagnosis of SLE (median of two years). Neurogenic bowel and bladder and presence of ANA and ds-DNA were invariable. Urodynamics assessment in six patients showed abnormal detrusor behavior in all. CT scans and myelograms were uninformative and CSF studies were normal. ESR and complement levels were insensitive as markers of disease activity. The treatment regimens included pulses of methylprednisolone and/or cyclophosphamide followed by prednisolone and high dose prednisolone from onset. The functional outcomes were uniformly good-with independent ambulation in all except three (who needed assistive devices) and improvement of motor scores. Acute hospital stays were short (range of three to 45 days) whilst only two were referred for inpatient rehabilitation. Bladder abnormalities persisted despite motor recovery and would require long-term review.
Lupus 1996 Aug
PMID:Transverse myelopathy in SLE: clinical features and functional outcomes. 886 1

We report a patient with medial medullary infarction who showed deep sensory impairment as his prominent neurological manifestation. A 54-year-old man with a history of hypertension was admitted to our hospital with numbness of the bilateral upper and lower extremities, followed by dysarthria and right hemiparesis. Physical examination revealed no abnormalities except for high blood pressure. He hiccuped continuously. On neurological examination, he exhibited dysarthria, mild dysphagia and right hemiparesis without facial or lingual paresis. Sensitivity to light touch and pinprick was normal, but sensitivity to vibration and joint position was severely decreased in the bilateral upper and lower extremities, predominantly in the lower extremities and on the right side in the upper extremities. He had been treated with antiedema agents and thromboxane synthetase inhibitor. His hiccups stopped within two weeks, and his right hemiparesis gradually improved within one month. However, his deep sensory impairments remained prominent. Blood examinations disclosed positive lupus anticoagulant. MRI showed bilateral infarction at the medial portion of the upper medulla oblongata, extending to both pyramids, especially on the left. Somatosensory evoked potentials (SEP) after median nerve stimulation showed P14 and the later components with prolonged latency. No SEP were recorded after posterior tibial nerve stimulation. The latency of P14 was well correlated with the severity of deep sensory impairments in the upper extremities. Neurological manifestations of our patient are not typical of medial medullary infarction, and are informative about the functional anatomy of the deep sensory tract in the medulla oblongata. We discuss the relation of the intractable hiccups to the bilateral medial medullary lesions, and emphasize the importance of lupus anticoagulant as one of the risk factors in brainstem infarction.
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PMID:[A case of medial medullary infarction with prominent deep sensory impairment]. 892 33

The purpose of this study was to determine the clinical characteristics of chronic renal failure patients who developed hand ischemia in the limb carrying the dialysis angioaccess. A retrospective review of the charts of 352 patients who underwent 409 upper extremity arteriovenous access, and who were subsequently diagnosed as steal syndrome, was performed at the Emory University Hospital between February 1992 and January 1997. Hand ischemia occurred after 13 of 299 arteriovenous grafts (4.3%) and after 2 of 110 direct forearm arteriovenous fistulas (1.8%). Six patients developed ischemic manifestations immediately postoperatively, 2 in the first week, 4 after 1 month, and 1 after 1 year. Thirteen occurred in association with the primary access procedure. Two cases occurred following graft thrombectomy and outflow dilatation. Seven patients were mildly symptomatic with dialysis-induced pain, coldness, or numbness; 8 patients developed severe ischemic manifestations in the form of sensory loss in 3, severe intolerable pain with impalpable pulse in 3, and digital gangrene and amputation in 2, one of whom developed an unhealed amputation stump and required a higher amputation level with satisfactory healing of the revised stump. Three patients were treated conservatively, 6 by banding, 4 by ligation, 1 by embolization, and 1 by distal ligation and bypass operation. Clinical characteristics of patients with hand ischemia included long-standing insulin-dependent diabetes (10), chronic hypertension (12), peripheral arterial disease (14; 93.3%), coronary artery disease (8), and systemic lupus erythematosis (1). Severe peripheral arterial diseases are commonly found and may be markers for risk of hand ischemia after access surgery.
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PMID:Incidence and characteristics of patients with hand ischemia after a hemodialysis access procedure. 953 65

We report a case of a 31-year-old woman with common variable immunodeficiency (CVID) complicated with intractable diarrhea and the functional disorder of renal tubules. The patient became hypogammaglobulinemic after she suffered from measles at 6 years of age. She also suffered from lupus-like syndrome at 7 years of age. The complete remission was obtained by glucocorticosteroid treatment. An intravenous immunoglobulin replacement therapy was introduced at 11 years of age, since then her general condition was stable for more than 20 years. When she was 29 years old, she suffered from generalized malaise, anorexia with body weight loss, and numbness of face. The intractable diarrhea as protein loosing syndrome, and the severe abnormality of electrolyte balance with metabolic acidosis as the functional disorder of renal tubules were found. Her condition was not improved by the electrolytes or alkali replacement therapy. She was admitted for further evaluation and treatment. The intractable diarrhea and the functional disorder of renal tubules were dramatically improved after absolute restriction of food intake under hyperalimentation. When she began to take food, the symptom and sign became worse again. The interstitial nephritis and nonspecific inflammation of intestine were found by the tissue biopsy. The most characteristic finding was the infiltration of lymphocytes (predominantly CD 8 + T lymphocytes) in both intestinal mucosa and renal interstitium. The introduction of glucocorticosteroids improved her general condition and biochemical findings. This CVID case is complicated with intractable diarrhea and the functional disorder of renal tubules which is associated with the infiltration of CD 8 + lymphocytes in intestine and kidney. We consider that such case is very rare and valuable to report.
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PMID:[A case of common variable immunodeficiency with intractable diarrhea and the functional disorder of renal tubules]. 1086 33

An 87-year-old woman with antiphospholipid syndrome accompanied by cortical blindness and thalamic syndrome resulting from infarction of the posterior cerebral artery is reported. She was hospitalized because of laceration of the head. Two months later, she complained of loss of visual acuity, sharp pain and numbness involving the left half of the body except her face. New right posterior lobe infarction and the existence of old left infarctions were confirmed by serial CT scans. Helical CT scan revealed embolization of the posterior cerebral artery with atherosclerotic stenosis. Serological examination showed biologically false-positive and positive findings for lupus anticoagulant. She was treated with warfarin potassium and clonazepam.
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PMID:Antiphospholipid syndrome with cortical blindness resulting from infarction around the posterior cerebral artery in an elderly woman. 1088 6

Lupus-related transverse myelitis is a rare but serious complication. A 25-year-old Japanese woman with systemic lupus erythematosus (SLE) was admitted because of numbness of the face and left upper extremity, headache, and intermittent fever. Six days later, she developed tetraplegia. MRI of the spinal cord showed longitudinal high intensity signals from medulla oblongata to C5, and from Th12 to conus medullaris on T2-weighted image. These MRI findings were consistent with acute catastrophic neurological abnormalities. Despite administration of the combination of methylprednisolone and cyclophosphamide pulse therapies, as well as plasmapheresis, her condition did not improve. Any vasculopathy in addition to the autoimmune pathogenesis, and narrow therapeutic window may relate to the present refractory case.
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PMID:Systemic lupus erythematosus related transverse myelitis presenting longitudinal involvement of the spinal cord. 1186 6

Carpal tunnel syndrome is caused by the pressure of the medianus nerve in the carpal canal. This syndrome is characterized by the following symptoms: numbness of the thumb and fingers, hand pain, particularly at night, and handicap of the hand efficiency. The examination of the perception, provocation tests, electromyography, carpal radiography, ultrasonography and magnetic resonance imaging are very helpful in the diagnosis of carpal tunnel syndrome. Pathological changes in flexor's theca are the most frequent causes of the syndrome. It may also occur in patients with lupus erythematosus, tuberculosis, endocrinopathy, rheumatic diseases and during pregnancy. Carpal tunnel syndrome is one of the most common occupational neuropathy. It is associated with the recurrence of carpal movements and observed in seamstresses, butchers, persons testing bar codes in supermarkets and meat packers.
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PMID:[Occupational and non-occupational aspects of carpal tunnel syndrome]. 1257 11

The neurological manifestations of antiphospholipid syndrome (APS) are diverse. Transverse myelitis (TM) is an uncommon, but well-known neurological complication of systemic lupus erythematosus (SLE). On the other hand, the reported cases associated with primary APS are extremely rare. To our knowledge, this is the first report of TM in a patient with primary APS in Korea. A 32-year-old male patient was admitted with the sudden onset of numbness, a tingling sensation, and weakness in both lower extremities. He had a 19 months history of external iliac and femoral arterial thromboses prior to admission. The laboratory results indicated the presence of anticardiolipin antibodies of the IgG class and lupus anticoagulant. No other autoantibodies were detected and there were no apparent clinical manifestations of SLE or multiple sclerosis. A T2-weighted magnetic resonance (MR) image showed swelling and increased intensity of the cervical and thoracic spinal cord between C6 and T7 with slight enhancement by contrast medium. After steroid pulse therapy, the patient's symptoms were gradually relieved and the abnormal findings on MR imaging disappeared.
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PMID:Transverse myelitis in a patient with primary antiphospholipid syndrome. 1272 75

Leprosy, a rare chronic granulomatous communicable disease caused by Mycobacterium leprae, is classically known to have cutaneous and neurologic sequelae. As a result of immigration, the disease, endemic in Brazil, India, Nepal, Madagascar, Myanmar, and Indonesia, has been recognized to be present in North America and the Caribbean. We describe a case of a woman presenting with a long history of a recurrent rash and leg numbness, initially diagnosed with systemic lupus, who was later proven to have lepromatous leprosy. It is a reminder that this underappreciated disease should still be considered in the differential diagnosis of skin rash and neuropathy, even in nonendemic regions.
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PMID:A case of recurrent rash and leg numbness mimicking systemic rheumatic disease: the occurrence of leprosy in a nonendemic area. 1755 81

Current diagnostic classification criteria recommend elevated titres of anti-cardiolipin (aCL) and/or anti-beta(2)GPI antibody by ELISA IgG or IgM and/or lupus anticoagulant (LA) to confirm antiphospholipid syndrome (APS). Although IgA aPL antibodies have been shown to be pathogenic in animal models of APS, their clinical significance has remained elusive. We report four cases of exclusive IgA anti-beta(2)GPI antibody sero-positivity with concomitant clinical manifestations associated with APS. Four of the five patients were LA negative. 1) Thirty-eight-year-old African-American female with SLE presented with resolving digital ulcers. Serum IgA anti-beta(2)GPI antibody titres were 118.5 SAU (normal range: 0-20 SAU). 2) Twenty-seven-year-old African-American woman with SLE was evaluated for recent onset of severe headaches, unresponsive to analgesics and anti-migraine medications. MRI of the brain revealed hyper-intensities in the white matter in the frontal lobes. Serum IgA anti-beta(2)GPI antibody titres were 29.1 Standard A Units (SAU). 3) Thirty-two-year-old Hispanic female with history of two unexplained miscarriages and negative serologies for SLE. Serum IgA anti-beta(2)GPI antibody titres were 102.0 SAU. 4) Twenty-five-year-old white female with history of recent unexplained miscarriage in the 11th week of gestation and associated complaints of numbness and tingling in her hands. Her IgA anti-beta(2)GPI antibody titre was 62.0 SAU. 5) Twenty-five-year-old African-American woman with SLE, positive for anti-Ro antibodies with a history of ischemic fingers, a pregnancy loss and recent pregnancy complicated due to pre-eclampsia. Her LA was positive and her IgA anti-beta(2)GPI antibody titer was 186.0 SAU. This case series supports that elevated IgA anti-beta(2)GPI antibody titres may identify additional patients who have clinical features of APS but who do not meet current diagnostic criteria.
Lupus 2009 Oct
PMID:Isolated elevation of IgA anti-beta2glycoprotein I antibodies with manifestations of antiphospholipid syndrome: a case series of five patients. 1976 4


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