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ABSTRACT: Determine if the MRL/lpr mouse develops neurological deficits and, if so, the pathologic basis for these deficits. Antiphospholipid antibodies (aPL) are associated with ischemic stroke, multiinfarct dementia, chorea, and cardiac valvular abnormalities. The MRL/lpr mouse develops high titer anticardioplin antibodies (aCL) suggesting that it may be used as a model for the neurological complications of aPL.
We undertook a prospective clinicopathologic study comparing the MRL/lpr mouse against its congenic strain, the MRL/+ mouse. We studied 15 MRL/pr and 15 MRL/lpr and 15 MRL/+ mice at 16 to 20 weeks and a group of 16 mice of each strain at 8 to 10 weeks. aCL and anti-DNA antibodies were measured by ELISA: Cognitive and neurological deficits were assessed by a water maze and a standardized rodent neurological examination. The brains and cardiac valves of the mice were then examined pathologically.
The MRL/lpr mice had significantly elevated aCL at both ages. Cognitive and sensorimotor deficits were apparent at 16 weeks but no correlation could be found with aCL or anti-DNA titer. Even at 8 weeks the MRL/lpr mice performed poorer on the water maze when compared to their age matched congenic strain. No evidence of cerebral infarction was found but mononuclear infiltrates were found in the choroid plexus of all the MRL/lpr mice at both 10 and 20 weeks. No evidence of cardiac valve pathology was seen at 20 weeks.
(1) The MRL/lpr mouse develops cognitive and neurologic deficits. The etiology of these deficits is not clear but may be related to early infiltration of the central nervous system with mononuclear cells. (2) Despite the elevated aCL, evidence of cerebral infarction or mitral valve abnormalities could not be found.
The Journal of Rheumatology 05/1993; 20(4):610-7. · 3.69 Impact Factor
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ABSTRACT: We describe 2 patients with systemic lupus erythematosus (SLE) with neurological complications and thrombocytopenia, who, upon further investigation, were found to have thrombotic thrombocytopenic purpura. While neither patient responded to treatment with corticosteroids, both responded to plasma exchange and intravenous cyclophosphamide. This highlights the need to carefully consider this diagnosis in patients with SLE presenting with neurological complications and thrombocytopenia.
The Journal of Rheumatology 10/1992; 19(9):1474-8. · 3.69 Impact Factor
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ABSTRACT: We report successful treatment of paroxysmal dystonia (tonic seizures) in three patients with central demyelinating disease (CDD) using acetazolamide alone or in combination with carbamazepine. Acetazolamide is a useful alternative, or an adjunct, to carbamazepine in the treatment of paroxysmal dystonia in CDD.
Neurology 05/1992; 42(4):919-21. · 8.31 Impact Factor
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D C Hess
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ABSTRACT: There is a strong link between antiphospholipid antibodies and stroke. Retrospective studies indicate that these patients are generally young, often have had prior thrombotic events including stroke, frequently complain of headaches, and often experience amaurosis fugax. Cardiac valvular lesions, particularly involving the mitral valve, are frequently found. The limited pathological studies show a noninflammatory thrombotic occlusion of both large and small cerebral vessels and a "thrombotic endocarditis." The underlying pathogenesis involves an immune-mediated prothrombotic state.
Stroke 03/1992; 23(2 Suppl):I23-8. · 5.73 Impact Factor
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ABSTRACT: Pulmonary endothelial aminopeptidase P (AmP) may be an important contributor to the inactivation of circulating bradykinin in certain species. To examine this possibility, we measured AMP activity in vivo and in vitro using Arg-Pro-Pro-[3H]benzylamide (3H-APPB) as substrate under conditions of first order enzyme kinetics. Utilizing multiple indicator dilution techniques, metabolism of 3H-APPB to Arg and Pro-Pro-[3H]benzylamide by AmP was not detectable during a single transpulmonary passage in anesthetized rabbits (n = 4), cats (n = 3) and pigs (n = 4). However, percent metabolism of 3H-APPB ranged from 54 to 63% in anesthetized rats (n = 6). In all experiments, the substrate remained within the vascular space and was thus accessible to endothelial and blood AmP only. At the same time, single-pass transpulmonary percent metabolism of [14C]benzoyl-Ala-Gly-Pro by endothelial-bound angiotensin converting enzyme was remarkably similar among rabbits, cats, rats and pigs (60-65%). In culture, Vmax/Km of AmP was 3 to 10 x 10(-4) min-1 for human basal arterial and rabbit and bovine pulmonary arterial endothelial cell monolayers (2 x 10(5) cells). AmP activity in the supernatant of lung and kidney tissue (homogenized in saline containing 1-o-n-octyl-beta-glucopyranoside) from rabbit, cat, pig and rat expressed as Vmax/Km(min-1) per (g wet tissue/ml) was 0.74, 2.25, 3.91 and 185.8 (lung), and 1.0, 3.7, 8.4 and 438.3 (kidney), respectively. Similarly, Vmax/Km values of AmP in plasmas of cat, dog, rabbit, pig, calf (serum), human and rat were 0, 0.016, 0.025, 0.068, 0.191, 0.237 and 3.53 min-1. These results suggest that 1) there are large interspecies variations in AmP activities of plasma, lung and kidney; 2) of the species studied, the rat contains the largest activities of AmP; and 3) AmP appears to be located on the luminal surface of the rat pulmonary endothelium.
Journal of Pharmacology and Experimental Therapeutics 01/1992; 259(3):1301-7. · 3.83 Impact Factor
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Seminars in Neurology 01/1992; 11(4):314-28. · 1.64 Impact Factor
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ABSTRACT: We undertook a prospective study of consecutive patients to determine the frequency of elevated IgG and IgM anticardiolipin antibodies in transient ischemic attack and ischemic stroke and found elevated IgG antibodies in 8.2% (9 of 110) and IgM in 9.1% (10 of 110), only the former being significantly greater than in a healthy control population. We suggest that anticardiolipin screening be concentrated on the young.
Neurology 05/1991; 41(4):525-8. · 8.31 Impact Factor
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ABSTRACT: We have reported the case of a young woman with prekallikrein deficiency who had a cerebral infarction despite a prolonged APTT. She had bleeding complications with heparin administration. From the limited information in the literature, anticoagulation may be hazardous in these patients, and it should be undertaken with caution.
Southern Medical Journal 05/1991; 84(4):507-8. · 0.83 Impact Factor
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ABSTRACT: We describe a 62-year-old patient who presented with ataxia and dystonia with mild dementia and was shown at autopsy to have Creutzfeldt-Jakob's disease. Movement disorders may be the presenting feature of Creutzfeldt-Jakob's disease, and this entity should be considered in the differential diagnosis of an otherwise obscure movement disorder even when the typical electroencephalographic pattern is absent.
Movement Disorders 02/1991; 6(2):157-62. · 4.51 Impact Factor
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ABSTRACT: We describe the first case of Miller Fisher syndrome, a Guillain Barré variant, complicating systemic lupus erythematosus. The symptoms and signs mimicked a brainstem syndrome. Despite treatment with high dose gamma globulin, our patient worsened and required mechanical ventilation. After plasma exchange, the patient improved.
The Journal of Rheumatology 12/1990; 17(11):1520-2. · 3.69 Impact Factor
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Journal of Neurology Neurosurgery & Psychiatry 10/1990; 53(9):804-5. · 4.76 Impact Factor
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ABSTRACT: We describe a case of pathologically confirmed Alexander's disease in which serial cranial ultrasound studies demonstrated unique findings of enlarging subependymal cysts with evolving periventricular hyperechogenicity. Computed tomographic scan of the head showed low attenuation of the periventricular white matter and centrum semiovale. Magnetic resonance imaging (MRI) demonstrated diffuse, confluent high signal predominantly in frontal white matter. These imaging modalities, particularly cranial ultrasound and MRI, may be useful tools in the diagnostic evaluation of children with degenerative neurologic disease, megalencephaly, and suspected Alexander's disease.
Journal of Child Neurology 08/1990; 5(3):248-52. · 1.75 Impact Factor
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ABSTRACT: Voluntary gaze may evoke a number of neurological phenomena such as vertigo, tinnitus, blepharoclonus, eyelid nystagmus, "facial nystagmus," involuntary laughter, and seizures. We report two patients in whom eccentric gaze evoked facial twitching and arm movement. Electroencephalograms remained unchanged during these movements. The pathogenesis of these movements is unclear but may involve ephaptic transmission.
Movement Disorders 02/1990; 5(2):139-42. · 4.51 Impact Factor
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ABSTRACT: The prevalence of dystonia was studied in 125 veterans on chronic antipsychotic therapy using a detailed and systematic examination. Twenty-seven out of 125 had dystonic manifestations. The most common areas involved were hands and jaw. There was no relation between the presence or absence of dystonia, and duration of neuroleptic therapy. There was a tendency for tardive akathisia to occur more frequently in patients with dystonia than in those without it (Fisher's exact probability test, p = 0.0656). Tardive dystonia in its milder forms may be more common than currently believed.
Movement Disorders 02/1990; 5(4):319-21. · 4.51 Impact Factor
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ABSTRACT: Epilepsia partialis continua (EPC) in multiple sclerosis is extremely rare. We report a case of a patient with clinically definite multiple sclerosis who developed EPC. Magnetic resonance imaging (MRI) depicted high signal in subcortical white matter and basal ganglia. A combination of lesions may be important in the generation of EPC.
International Journal of Neuroscience 02/1990; 50(1-2):109-11. · 0.97 Impact Factor
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Journal of the Medical Association of Georgia 01/1990; 78(12):815-20.
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ABSTRACT: Hemiballism and hemichorea are recognized manifestations of cerebral infarction. Paroxysmal dyskinesias are less well recognized symptoms of transient cerebral ischemia. We describe a 41-year-old man with distal internal carotid artery occlusion and poor intracranial collaterization who developed repetitive stereotyped involuntary arm movements and subsequent cerebral infarction. This peculiar manifestation of transient cerebral ischemia may indicate poor intracranial collateralization with reliance upon leptomeningeal collaterals. Earlier recognition may help avert cerebral infarction.
Cerebrovascular Diseases. 08/1970; 1(1):54-57.
