Abstract: OBJECTIVE: The aim of the present study was to examine the relationship between magnetic resonance imaging (MRI) findings and cognitive functioning in a group of patients with primary Sjögren Syndrome (SS). METHODS: Fifteen subjects with primary SS and fifteen control subjects diagnosed with migraine were assessed. All subjects received a detailed neuropsychological assessment especially sensitive to fronto-subcortical disorders and a MRI study. Volumetric measures of the ventricular system and intracranial volume and measures of the severity and distribution of signal hyperintensities were obtained. RESULTS: Patients with SS showed larger ventricular volume than control subjects with migraine. The severity of MRI signal hyperintensities and ventricular volume were related to several cognitive and psychiatric variables. CONCLUSION: Patients with primary SS have morphological abnormalities that are related to neuropsychological and psychiatric disturbances. These findings provide some support for the organic etiology of cognitive and psychiatric dysfunction and thus for central nervous system involvement in SS.
Abstract: BACKGROUND AND PURPOSE: Abnormalities of dermal connective tissue have been detected in patients with spontaneous cervical artery dissections (sCAD), suggesting an underlying structural defect of the arterial wall. Alpha1-antitrypsin (A1-AT) is a circulating serine proteinase inhibitor of proteolytic enzymes that helps to maintain the integrity of elastic and collagen fibers. METHODS: To test the hypothesis that moderate deficiency of A1-AT may be a risk factor for sCAD, 22 cases with sCAD and 113 controls were included in the study. RESULTS: Patients with sCAD had significantly mean lower levels of A1-AT compared with controls (116.0+/-24.9 versus 141.1+/-31.7 mg/dL; P<0.01). Low levels of A1-AT (<90 mg/dL) were more frequently observed in patients with sCAD compared with controls (27.3% versus 2.7%; P<0.001). A positive correlation between age and plasma levels of A1-AT was found (r=0.22; P<0.01). A1-AT levels were not affected by sex or vascular risk factors, including smoking habit. On multivariate analysis, A1-AT <90 mg/dL was associated with sCAD independently of age, sex, or vascular risk factors (odds ratio, 17.7; 95% confidence interval, 2.9 to 105.6). CONCLUSIONS: Low plasma levels of A1-AT may be a risk factor for sCAD.
Abstract: An important feature of central nervous system (CNS) immune privilege is that antigens expressed in CNS are sequestered and not available for central tolerance induction. Tissue distribution and, more specifically, thymic expression of many of the CNS putative autoantigens have not yet been clearly established in humans. We have addressed this question for the putative multiple sclerosis(MS) autoantigens alphaB-crystallin, S100beta, proteolipid protein (PLP) and myelin oligodendrocyte glycoprotein (MOG)-alpha and MOG-beta isoforms, using quantitative RT-PCR on human thymus (total, cell fractions and microdissected specimen) and on a panel of peripheral tissues. alphaB-crystallin, S100beta and the DM20 isoform of PLP were clearly expressed in the thymus and also in selected peripheral tissues. In contrast, the expression of MOG out of the CNS was not observed. Within the human thymus, the level of CNS antigen expression was found higher in the stromal epithelial enriched cell fraction, and in microdissected samples of the medullary compartment. These results indicate that most of the antigens involved in MS are expressed in the thymus, suggesting a possible role in central tolerance. However, MOG and, to a lesser extent PLP, conform the classical concept of sequestered antigens, thus supporting the involvement of MOG in autoimmune demyelinating diseases.
Abstract: Changes in the apparent diffusion coefficient (ADC) are well established in acute ischemic stroke of arterial origin. However, ADC behaviour and its prognostic significance in cerebral venous thrombosis (CVT) are not fully understood. Diffusion-weighted imaging (DWI) findings in a 34-year old woman with deep cerebral venous thrombosis are described. Recent literature concerning DWI and cerebral venous thrombosis is also reviewed. A MRI performed within 7 hours from onset revealed hyperintensities in deep grey matter bilaterally (FLAIR/T2), without changes in ADC maps, suggesting vasogenic edema. After anticoagulation a new MRA disclosed complete recanalization of venous thrombosis. Despite her good clinical outcome the MRI showed hemorrhagic lesions suggesting venous infarct. Lesions detected in acute CVT with DWI may have normal ADC values. There is no good correlation between the acute ADC values and clinical and radiological evolution. The prognostic value of ADC in the acute phase of CVT remains unsettled.
Abstract: INTRODUCTION: Percutaneous transluminal angioplastia has in recent years become an alternative to surgery and increasingly used for revascularization of the extracranial arteries in patients with cerebral ischemia. However, intracranial angioplasty is a technique which is still not widely used since it is technically more difficult and until now endoprotheses (stents) have not been available specifically designed for intracranial territory. CLINICAL CASE: A 73 year old patient with extensive extracranial and intracranial atheromatous lesions, multiple vascular risk factors and cardiac ischemia which contraindicated surgical treatment which was treated consecutively by angioplasty and angioplasty with implantation of stents in both carotid bifurcations. Subsequently, he was treated by angioplasty for a stenosing lesion of 90% of the right carotid siphon with clinical and hemodynamic repercussions. Following the procedure, which was well-tolerated by the patient, there was clinical and angiographic improvement and return to normal of the hemodynamic parameters measured by transcranial Doppler. CONCLUSIONS: Angioplasty is a technique which may be used in intracranial stenosing atheromatous lesions. Even without perfect angiographic correction, adequate blood flow is established in the hemodynamically affected lesions. As far as we know this is the first case of intracranial angioplasty of a lesion of the carotid siphon reported in Spain.
Abstract: INTRODUCTION: Continuous use of oral anticontraceptive agents has been related to adverse vascular changes in the form of venous or arterial thromboses and cerebrovascular accidents (CVA). In our case, we describe a CVA due to occlusion of the common carotid artery after an acute massive overdose of estrogens due to error of dosage when using Yuzpe's system or emergency post-coital therapy. CLINICAL CASE: We describe a 26 year old woman with no previous clinical history, who smoked 20 cigarettes per day, did not take oral contraceptive pills and suffered an acute left facial-brachial-crural hemiplegia together with reduced level of consciousness. Imaging tests showed acute occlusion of the right common carotid artery. Etiological study of the patient's cerebral vascular accident was negative. The only etiopathogenic factor to be related was having taken oral contraceptive agents for three days after coitus as emergency post-coital treatment. This treatment was incorrect, since the dose of estrogens was four times that recommended. CONCLUSIONS: The continued use of estrogens, especially at doses over 30 micrograms per day, was considered responsible for approximately 10% of CVAs in young people. The risk of cerebral vascular accident is greater when there is associated migraine and/or smoking. We describe a patient who showed that the acute use of high doses of estrogens may also cause arterial occlusion, in this case in the common carotid artery.
Abstract: We present a case with a characteristic magnetic resonance image (MRI) of bilateral open-lipped schizencephaly and atypical clinical presentation. The patient is still alive and in good health in her forties, she has never presented seizures, and although the motor dysfunction is well correlated with cerebral lobe involvement, neurobehavioral dysfunction is not proportional to the MR image of the cerebral malformation.
Abstract: Our prospective study consisted of a general and neurological evaluation in 48 patients (41 females, 7 males; mean age: 58.2 years) with primary Sjögren's syndrome (PSS). We performed serologic studies and cranial magnetic resonance imaging (MRI). Main extraglandular features were arthralgias and non-erosive arthritis (37.5%). Raynaud (21%) and pulmonary fibrosis (12.5%). Antinuclear antibodies were positive in 42.5% and anti-SS-A (Ro) in 20%. Migraine (52%), neuropsychiatric disease (29%) and a past history of focal acute neurological deficits (23%), were the central nervous system (CNS) manifestations more frequently observed. Cranial MRI examination detected hyperintense small subcortical lesions in 51.3% of patients and in 36.6% of age and sex matched controls (P < 0.001). CNS disease was not serious concerning vital prognosis, but produced significant morbidity in some patients. Late onset "migraine-like" episodes with prolonged sensoromotor deficits and coexisting neuropsychiatric disease emerged as a characteristic clinical spectrum in those patients diagnosed in a neurological setting. Cranial MRI was frequently abnormal, but findings were not specific. Neurologic manifestations reminiscent of multiple sclerosis were rarely seen.
Abstract: BACKGROUND: The primary Sjögren's syndrome (SS) is a systemic disease which destroys the exocrine glands by autoimmune mechanisms. The etiology of this syndrome is unknown although different virus are involved in its genesis. METHODS: The presence of serologic reactivity IgG and IgM versus the human immunodeficiency virus (HIV-1), HIV-2, HTLV-I and HTLV-II were studied in 14 patients with SS and in 15 controls. Likewise, the presence of retroviral genomic sequences was analyzed in 7 of these patients by polymerase chain reaction (PCR). RESULTS: All the patients with SS were negative by enzymoimmunoassay (EIA) versus known retrovirus. However, more than 70% presented reactivity versus different nuclear proteins, particularly versus p24 of HTLV-I in Western blot (WB). These results were negative in the control group. Genomic analysis by PCR did not confirm the presence of specific sequences in any of the known human retroviruses in the patients with SS, nonetheless, in 3 of the 7 samples analyzed by PCR, related retroviral sequences were detected. CONCLUSIONS: The presence of serologic reactivity in Western blot versus some viral proteins and the similarity of genomic sequences in some cases suggests that a retrovirus related with those which are currently known, particularly with the HTLV-I, may be involved in the genesis of Sjögren's syndrome.
Abstract: A 59-year-old woman with breast cancer and anti-Ri antibodies developed a neurologic paraneoplastic disorder characterized by nausea, vomiting, vertigo, paresis of upward gaze, and gait ataxia, without opsoclonus. The absence of opsoclonus does not rule out the possibility of an anti-Ri-associated paraneoplastic neurologic disorder.
Abstract: A primary lymphoma involving the meninges of the frontobasal region, with focal cortical infiltration and prominent cerebral edema, was treated by surgery and radiotherapy. Eight years later the patient died of a recurrence. Morphological and immunohistochemical studies comparing the initial tumor with the recurrent lesion found similar histopathological features.
Abstract: A hemifacial spasm was symptomatic of a neurinoma of the hypoglossal nerve in a 50 year-old woman. It disappeared after the surgical cure of the tumor. Hemifacial spasm is not mentioned among the 19 cases of neurinoma of the hypoglossal nerve which have been reported up to now. The spasm could have been due to the compression of the 7th nerve by the anterior inferior cerebellar artery, displaced by the tumor.
Abstract: The clinical observations in five patients, of a family of catalan origin (NE of Spain), affected with Machado-Joseph disease are reported. The pedigree showed the presence of 22 members affected (15 men, 7 women) over six generations. The symptoms and signs were variable among the patients and also variable in a same patient during the course of the disease. However, the main neurological alterations were ataxia, akinesia, distal amyotrophy, progressive external ophthalmoplegia, facial and lingual fasciculations and bulging eyes. The neuropathological examination performed in one patient disclosed degeneration of the posterior and spinocerebellar tracts in the spinal cord, marked nerve cell loss in Clarke's column and anterior horns and axonal degeneration of the peripheral nerves, in addition to nerve cell loss in the nuclei of the III, IV and VII cranial nerves and neuronal depletion in the substantia nigra. No other structures, including the striate complex and dentate nucleus, were significantly affected.
Abstract: A case of multiple sclerosis and Hashimoto's thyroiditis confirmed by cytology is reported. The association of multiple sclerosis with other autoimmune diseases is infrequent but supports the immune hypothesis of the pathogenesis of multiple sclerosis. The authors suggest the inclusion of immunological tests in the screening of all patients diagnosed as having multiple sclerosis.
