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Query: UMLS:C0018991 (hemiplegia)
 3,997 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The authors report a new case of necrosing angeitis similar to those individualised by Churg and Strauss in 1951. After a persistent rhinitis, the patient developed serious asthma, left hemiplegia 8 months later (secondary to an intracerebral hemorrhage) polyneuritis and extensive necrosis of the small intestine. There was no renal or cardiac involvement. Anatomopathological examination of the small intestine revealed an arteriolitis with periarterial granuloma and severe inflammation. Complementary investigations revealed a hypereosinophilia, an inflammatory syndrome, a reduction in the hemolytic 50 complement and of the C4 fraction. Corticosteroid therapy associated with cyclophosphamide formed the basis of treatment. Plasmapheresis have been a useful aid. The follow-up is too short to assess the long term prognosis. A familial immune deficiency, disseminated lupus erythematosis in a sister, and rheumatoid purpura in one of the patient's children are interesting features of this text.
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PMID:[Severe visceral necrotizing angiitis with hereditary immune disorders: a case]. 613 87

We reported a case of malignant rheumatoid arthritis (MRA) with cerebral infarction associated with a possible cause of lupus anticoagulant. The patient was a 68-year-old woman who had received treatment for rheumatoid arthritis (RA) from 15 to 16 years ago. She consulted to our hospital with a major complaint of right hemiplegia. Brain CT revealed a low density area in the left hemisphere. She was diagnosed as cerebral infarction and hospitalized. Since she was noted to have hypocomplementemia, interstitial pneumonia and pericarditis, she was diagnosed as MRA. Coagulation test disclosed positive lupus anticoagulant (LA). Generally, CNS disorders in MRA are uncommon. Cerebral infarction was complicated in the present case, suggesting the involvement of antiphospholipid antibodies as its pathogenesis.
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PMID:[A case of malignant rheumatoid arthritis with lupus anticoagulant and cerebral infarction]. 777 8

Records of 108 patients with lupus erythematosus beginning in childhood (1953-1990) were reviewed; 25 had recorded neurologic findings. This is the largest group of childhood lupus erythematosus patients with neurologic disease that has been reported. The average age of children at the time of diagnosis of lupus was 154 months. There were 22 girls and 3 boys in the group. All patients met at least four of the 1982 American Rheumatism Association criteria for the classification of systemic lupus erythematosus. Average age at onset of neurologic difficulties was 168 months. In 4 patients, the neurologic symptoms preceded the diagnosis: 1 month (spastic diplegia), 1 month (bilateral weakness and spasticity), 24 months (chorea), and 26 months (chorea), respectively. Four patients had neurologic symptoms coincident with the diagnosis of lupus erythematosus. In those patients whose symptoms followed the diagnosis of lupus erythematosus, the average elapsed time until symptoms appeared was 33 months; the single lowest and highest outliers were discounted. Most frequent findings were headache (16/25) and behavioral aberrations (10/25). All behavioral manifestations were depression except in 1 patient. Other prevalent findings included hemichorea or chorea (7/25), cerebrovascular accident with hemiplegia or diplegia (7/25), seizures (5/25), visual loss (3/25), and cranial neuropathy (2/25). Vertigo and myelopathy occurred in 1 patient each. All patients were treated primarily with corticosteroids and azathioprine; in the presence of active disease, the drug dosages were increased with significant improvement in neurologic symptoms. Resolution usually occurred from days to months; most improved in a few days to a few weeks; 3-4 months was the longest period until symptoms subsided.
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PMID:Neurologic characteristics of childhood lupus erythematosus. 855 56

The significance of antiphospholipid antibodies was examined in patients with childhood strokes. Eight patients, aged 2-13 years, who presented with acute hemiplegia were studied. On the basis of magnetic resonance imaging, magnetic resonance angiography, single photon emission computed tomography, and cerebral angiographic findings, 3 children were diagnosed as having infarctions due to moyamoya disease, and the others as having idiopathic infarctions. Serologic studies revealed elevated anticardiolipin antibody (ACA) IgG in 3 of the 5 patients with idiopathic infarction; no such elevation was revealed in patients with moyamoya disease. Values for all other tests, including ACA IgM and lupus anticoagulant, were within normal limits or negative in all patients. ACA IgG, therefore, may be a more important causative agent of childhood strokes than was previously considered.
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PMID:Antiphospholipid antibody syndrome in childhood strokes. 877 Nov 68

A young male patient who presented with steroid-responsive parkinsonism, hemiplegia, thrombocytopaenia and systemic illness who was subsequently diagnosed to have systemic lupus erythematosus (SLE) is described. He later developed post-hemiplegic dystonia. Thalamic lesions on magnetic resonance imaging (MRI) are demonstrated. Clinical features and neuropathology of central nervous system lupus are discussed. This is the first report of SLE presenting with Parkinsonism, and the first to demonstrate anatomically-relevant MRI lesions in Parkinsonism associated with SLE.Copyright Lippincott-Raven Publishers
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PMID:Systemic lupus erythematosus presenting with steroid-responsive parkinsonism and post-hemiplegic dystonia. 1021 Aug 49

Cerebral venous thrombosis (CVT) is a disease with multiple known etiologies that present with a remarkably wide spectrum of clinical signs and symptoms. We present a case of a 34-year-old man with a history of meningeal symptoms for 1 week after receiving a lumbar injection for lower back pain. He subsequently developed dense right hemiplegia and global aphasia. Head magnetic resonance imaging revealed superior sagittal sinus thrombosis. The patient was started on intravenous heparin but deteriorated neurologically. Urokinase infusion directly into the superior sagittal sinus was performed, with striking functional and neurologic improvement. Lupus anticoagulant was positive. We also present the case of a 24-year-old pregnant woman who developed an acute onset of meningeal symptoms and resultant left hemiparesis. Head magnetic resonance angiography revealed thrombosis of right transverse and sigmoid sinuses. Protein S deficiency was found. She was started on intravenous heparin, then enoxaparin, with improvement in symptoms. These cases demonstrate that CVT can be a cause of stroke in young patients with hypercoagability disorders, and a heightened awareness of CVT will promote optimal medical care and functional outcomes. Excellent functional recovery is likely with early recognition and treatment of the underlying etiology, as well as successful lysis of the clot.
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PMID:Cerebral venous thrombosis in young adults: 2 Case reports. 1134 48

A-38-year-old man suddenly developed nausea, vomiting and vertigo during chiropractic neck manipulation. This was followed by right hemiplegia, right deep sensory disturbance and left hypoglossal nerve palsy, consistent with the medial medullary infarction (Dejerine syndrome). The MRI revealed infarction at left medial part of the medulla. The vertebral angiogram and MRA showed marked narrowing of the left vertebral artery. X-rays of the cervical spine showed no spondylosis, dislocation nor osteolysis of the odontoid process. The serological studies, including lupus anticoagulant, protein C, and protein S gave normal results. Although vascular accidents involving the brain stem after chiropractic neck manipulation have been reported since Pratt-Thomas and Berger, previous reports are still rare. In them lateral medullary infarction (Wallenberg syndrome) is probably the most common case. On the other hand, medial medullary syndrome (Dejerine syndrome) is absolutely rare. To our knowledge, the only one report has been made by Watanabe and his colleagues before our present case. The mechanism was suggested that rotation and tilting of the neck stretches and compresses the vertebral artery at the cervical joint causing injury to the vessel, with an intimal tearing, dissection, and pseudoaneurysm formation. Consequently, the present case may be caused by injury to the left vertebral artery with an intimal tearing during neck manipulation sufficient to cause disection and subsequent infarction of the brain stem.
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PMID:[The medial medullary infarction (Dejerine syndrome) following chiropractic neck manipulation]. 1268 91

A 43-year-old female, who had been treated for systemic lupus erythematosus (SLE), presented with a subarachnoid hemorrhage (SAH) induced by rupture of cortical venous thrombosis, and be followed by medial medullary infarction during the acute stage of the SAH. The patient initially manifested a SAH. Angiography demonstrated no evidence of any aneurysms or arteriovenous malformations, but revealed cortical venous thrombosis. She suddenly developed left hemiplegia caused by medial medullary infarction on the 6th day. An active anticoagulant therapy was thought to be inappropriate because of initial symptoms as a hemorrhage. Since she had been accompanied by the medullary infarction, then, initially started by antiplatelet therapy. After the confirmation of no saccular or dissecting aneurysms with 2nd angiography, her treatment could be changed to anticoagulant therapy. Because of the sustained negative reactions of anti-cardiolipin beta 2 glycoprotein I antibody and lupus anticoagulant during the course of SLE, the definite diagnosis of antiphospholipid syndrome (APS) could not be made. However, this case is pathogenically thought to be cerebrovascular disease based upon APS, considering that this syndrome may be related to various antigen/antibody systems.
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PMID:[Systemic lupus erythematosus manifesting as subarachnoid hemorrhage induced by cortical venous thrombosis and followed by medial medullary infarction]. 1457 41

The role of immunity and inflammation in epilepsy have long been suggested by the anticonvulsant activity of steroids in some infancy and childhood epilepsies. The role of fever and infection in exacerbating seizures due to possible proinflammatory molecules, the increased frequency of seizures in systemic autoimmune diseases like systemic lupus erythematous, and, recently, the detection of autoantibodies in some unexplained epilepsies reinforced the causal place of immunity and inflammation in epilepsies with unknown etiology. In this article, we summarize epilepsies where clinical and biologic data strongly support the pathogenic role of autoantibodies (e.g., limbic encephalitides, N-methyl-d-aspartate [NMDA] encephalitis) and epilepsies where immune-mediated inflammation occurs, but the full pathogenic cascade is either not clear (e.g., Rasmussen's encephalitis) or only strongly hypothesized (idiopathic hemiconvulsion-hemiplegia syndrome [IHHS] and fever-induced refractory epilepsy in school-aged children [FIRES]). We emphasize the electroclinical features that would help to diagnose these conditions, allowing early immunomodulating therapy. Finally, we raise some questions that remain unclear regarding diagnosis, mechanisms, and future therapies.
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PMID:Autoimmune and inflammatory epilepsies. 2294 22

Moyamoya syndrome (MMS) is a rare, chronic progressive cerebrovascular occlusive disease that is characterized by a stenosis or occlusion of the bilateral internal carotid arteries and the circle of Willis arteries leading to the development of collateral vessels as visualized by cerebral angiography. We report a case of a 24-year-old woman with nephrotic syndrome whose biopsy showed membranous nephropathy. Ten months after the diagnosis she suffered sudden right hemiplegia and seizure. She was diagnosed with MMS by angiogram seven months ago and received decompressive craniotomy. The patient was admitted to our hospital and a diagnosis of systemic lupus erythematosus (SLE) was made. Glucocorticoids and tacrolimus were used to control the symptoms of SLE. Following one month of immunosuppressant treatment, the patient died of brain hemorrhage. This case alongside another six reviewed cases shows that an underlying cerebrovascular lesion of moyamoya in the vessels of patients with SLE is susceptible to cerebrovascular accidents.
Lupus 2013 May
PMID:Systemic lupus erythematosus associated with Moyamoya syndrome: a case report and literature review. 2357 43


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