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Query: UMLS:C0018681 (headache)
 56,091 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

As many as 30 patients suffering from systemic lupus erythematosus (SLE) with the clinical signs of central nervous system derangement were examined. The mean age of the patients was 31.1 years. Using EIA, antibodies against cardiolipin (a-CL) were detected in 21 patients (70%). A-CL were revealed in all the patients with cerebral circulation impairment (CCI), choreic hyperkinesis, and convulsive syndrome. A-CL were discovered in 12 out of 18 SLE patients suffering from migraine-like headaches and in 4 out of 5 patients with mental disorders. Antibodies reacting with cardiolipin were mostly represented by the IgM isotype (80%) whereas a-CL-IgG were only identified in 13% of the patients, being associated in all the cases with a-CL-IgM. The high level of a-CL-IgG in blood serum was recorded in patients with the gravest patterns of nervous system derangement: CCI, occlusion of the retinal artery, psycho-organic and convulsive syndromes. All these patients demonstrated generalized reticular livedo. The high levels of a-CL-IgM were observed in SLE patients with choreic hyperkinesis and migraine-like headaches. Thus, the studies made it possible to trace the relationship between the development of certain neurological disorders (CCI, chorea, convulsive syndrome) in SLE patients and a-CL.
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PMID:[The clinico-immunological characteristics of central nervous system involvement in systemic lupus erythematosus: the relationship with antibodies to cardiolipin]. 145 60

An examination has been carried out of 46 patients (33 females, 13 males, a mean age 40) with Sneddon's syndrome characterized by cerebrovascular disturbances and marked livedo. A clinical spectrum of the syndrome included miscarriage and intrauterine death of the fetus (20 cases), peripheral vein thromboses (12 cases), coronary heart disease (18 cases), thrombocytopenia (8 cases), arterial hypertension (27 cases), headache (39 cases), epileptic seizures (5 cases). Similar manifestations are usually seen in antiphospholipid syndrome (AFLS). Antibodies to phospholipids, those to cardiolipin, lupus anticoagulant were detectable in 78, 50 and 61% of the cases, respectively. Clinical and immunological signs of AFLS in the absence of SLE-typical symptoms provided grounds for considering them primary AFLS. Similar clinical patterns in 36 patients with cardiolipin antibodies and/or lupus anticoagulant and 10 patients without the antibodies and anticoagulant suggest these cases to be AFLS too.
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PMID:[Sneddon's syndrome and the primary antiphospholipid syndrome]. 805 89

Three children with pronounced livedo reticularis present since birth (cutis marmorata-telangiectasia congenita) have been followed to the ages of eight, 17 and 21 years. During childhood they developed frequent recurrent transient stroke-like hemipareses, affecting either side of the body, associated with ipsilateral pain, headache, visual symptoms, dysphasia, fits and confusion. Intellectual failure and, in one, progressive spasticity have followed. Attacks were more frequent in winter. Other problems have included abnormal peripheral vascular responses to temperature change, gastro-intestinal bleeding, glaucoma, local tissue hypertrophy and, in the two older patients, renal involvement with hypertension. Their condition represents a form of congenital vasculopathy. Anticonvulsants, anti-migraine agents, anti-platelet drugs and flunarizine have been ineffective. Nifedipine prevented further attacks in one patient and reduced attacks in another, but has not helped the third child. Adequate clothing and warmth may also be important.
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PMID:Congenital livedo reticularis and recurrent stroke-like episodes. 840 21

Sneddon syndrome (SS) is increasingly recognised as a cause of ischaemic stroke in young adults. As the natural course of SS is not well defined, the authors performed a prospective six year clinical and neuroradiological follow up study. Thirteen patients with definite diagnosis of SS (livedo racemosa, characteristic skin biopsy, and history of stroke) entered a follow up programme that consisted of clinical examinations, two magnetic resonance imaging (MRI) investigations, and a comprehensive laboratory follow up protocol. The most frequent clinical findings during follow up had been headache (62%) and vertigo (54%). Seven patients (54%) suffered from transient ischaemic attacks, however, completed stroke has not been obtained during follow up. Progression of white matter lesions detected in MRI were present in 10 of 13 patients. Laboratory follow up protocol revealed transient antiphospholipid antibodies in two subjects. This prospective six year follow up study suggests a low incidence of territorial stroke but outlines progressive leucencephalopathy in patients with SS.
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PMID:The natural course of Sneddon syndrome: clinical and magnetic resonance imaging findings in a prospective six year observation study. 1264 88

Output of antiphospholipid antibodies (aPL) coupled with arterial and/or venous thromboses, miscarriage and some other clinical manifestations is denoted as antiphospholipid syndrome (APS). The syndrome is primary (PAPS) in the absence of other autoimmune diseases. Arterial thromboses occur most frequently in the cerebral arteries, leading to ischemic disorders of cerebral circulation (DCC). The present work summarises the results of our own studies and of the reported data on DCC in PAPS. Their characteristic features Include the relationship with occlusion of the intracerebral or intracranial rather than of the great vessels of the head, liability for recurrences in the absence of secondary prophylaxis, frequently occurring association with primary disorder of cerebral circulation (PDCC), good restoration of the focal neurologic deficit after the first stroke, more frequent development in women. The clinical recognition of DCC which stem from aPL output is favoured by the presence in the patients of the basic non-cerebral signs of PAPS (miscarriage peripheral venous thrombosis, myocardial infarction) as well as by the presence of the additional evidence of PAPS (livedo, induration of heart valves according to the EchoCG data, epileptic syndrome, migraine-like headaches, chorea in the anamnesis, and so forth). In most cases, they precede the first DCC by several years or months. The secondary prophylaxis of DCC in PAPS includes the intake of indirect anticoagulants and. small doses of aspirin.
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PMID:[Ischemic stroke at the young age: the role of antiphospholipid antibodies]. 1562 29

Anti-phosphatidylethanolamine antibodies (aPE) belong to the group of anti-phospholipid antibodies (aPL) and are directed against neutral phospholipid, connected with co-factor protein, while cardiolipin antibodies (aKL) are directed against negative phospholipid. The paper presents a study of prevalence and clinical significance of IgG aPE in 28 patients (22 women and 6 men, mean age 47.6 +/- 11.6 years) with Sneddon's syndrome (SS), which consists in cerebrovascular disturbances and extensive livedo reticularis. IgG aPE were detected by immune-enzyme assay. The upper normal limit, calculated as mean + 3SD after studying 19 healthy donors, was 0.303 optic density units. aPE were found in 15 (54%), aKL and/or lupus anticoagulant (LA)--in 6 (21%) patients with SS. aPE were found in 10 (46%) out of 22 aKL- and LA-negative patients. Among the aPE-positive patients there was a higher incidence of cortic dementia (53% vs. 8%, p = 0.02), the widening of cortical sulci, detected by means of computed tomography and magnetic resonance imaging (73% vs. 31%, p = 0.05), and mild renal syndrome (73% vs. 16%, p = 0.03). Besides, they displayed a higher rate of headaches (87% vs. 62%), chorea (33% vs. 8%), epilepsy (27% vs. 8%), non-carrying of pregnancy (91% vs. 50%), peripheral venous thrombosis (27% vs. 15%), coronary heart disease (47% vs. 31%), cardiac valvular thickening, detected by means of EchoCG (93% vs. 69%), arterial hypertension (87% vs. 54%), thrombocytopenia (20% vs. 0), anemia (40% vs. 15%); however, the difference was not significant. The results show that aPE detection, performed in addition to detection of classic immunological antiphospholipid syndrome markers (aKL and LA), increases the portion of aPE-positive patients with SS by 33%. aPE are often (in 46% of cases) found in aKL- and LA-negative patients with SS. aPE is likely to be the most significant factor of thrombosis in small arteries of the brain cortex and kidneys, which could explain their association with dementia and renal syndrome.
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PMID:[Anti-phosphatidylethanolamine antibodies in patients with Sneddon's syndrome]. 1598 83

Sneddon's syndrome refers to the enigmatic association of ischaemic stroke and livedo reticularis. We review the Sneddon's syndrome literature examining the association of this condition with headache, including migraine. Case reports and series are stratified into two groups based on headache reference. In the group without a reference to headache, there are 208 persons, with a female to male ratio of 3 : 1. In the headache reference group, there are 175 persons, with a female to male ratio of 3.5 : 1. The proportion with headache in this second group is 58% (102 individuals), with headache described as migraine in 28 (27.5%) of the headache subjects, including six with migraine with aura. The frequency of headache is not significantly higher in persons with positive anti-phospholipid antibodies compared with the negative cohort (43% vs. 32%, P = 0.07). A review of the histopathological, radiological and serological data in Sneddon's syndrome and migraine underscores the plausibility of an association. Considered in the context of increased risk of stroke with migraine, a higher frequency of livedo in migraineurs with stroke, and the association of migraine and livedo reticularis, the question of whether livedo reticularis may be a risk marker for stroke in migraineurs is an area for further study.
Cephalalgia 2006 Mar
PMID:Sneddon's syndrome: another migraine-stroke association? 1647 27

Sneddon syndrome is a rare disorder characterised by generalised livedo racemosa of the skin with extracutaneous neurological symptoms like headache, vertigo, transient ischaemic attacks (TIA), stroke, and seizures. Diagnosis of Sneddon syndrome is based on these clinical features and positive findings in skin biopsies, namely the histological proof of occlusion of arterioles by intimal proliferation. We describe three cases of young patients with clinical characteristics of Sneddon syndrome, but in only two cases could this diagnosis be confirmed by skin biopsies. These cases stress the difficulty of diagnosing Sneddon syndrome and show the additive value of skin biopsies in this process.
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PMID:Sneddon syndrome and the diagnostic value of skin biopsies - three young patients with intracerebral lesions and livedo racemosa. 1847 64

Sneddon's syndrome is a rare vascular disease affecting mainly skin and brain arterioles leading to their occlusion due to excessive endothelial proliferation. The two main features of this syndrome are livedo reticularis and lacunar subcortical infarcts. Here, we describe the case of a 64-year-old woman presenting with a 4-year history of a throbbing, bilateral, parieto-occipital headache associated with facial pain, but without any other accompanying symptom. The pain, initially misdiagnosed as atypical trigeminal neuralgia, worsened up to chronic daily and such severely disabling headache that she was constrained to bed. She presented with reduced cognitive functions, diffuse and severe livedo reticularis, severe myalgias and mild stiffness. All diagnostic test for different diseases were performed and other diseases excluded except for Sneddon's syndrome. Her symptoms were reduced firstly using acetylsalicylic acid, then ticlopidine 250 mg bid was begun and then Pentoxyphillin, resulting in a significant improvement of symptoms with the disappearance of headache. Her worsening in the first year was characterized by obsessive-compulsive behaviours, body-image misperceptions and panic attacks, improved for a period using olanzapine. Considering this case, we remark the importance of using headache classification to avoid diagnostic errors, secondly, we describe an atypical manifestation of Sneddon's syndrome and therapeutic efficacy of using ticlopidine and pentoxyphillin.
J Headache Pain 2009 Jun
PMID:Sneddon's syndrome presenting with severe disabling bilateral headache. 1928 55

A 34-year old woman with dizziness, headache and both upper and lower extremities weakness was admitted to hospital. She had a history of photosensitivity but no asthma or allergy. On physical examination, malar rash and livedo reticularis were noted. White blood cell count was 18500/microL with 7585 eosinophils (41%). She also had positive antinuclear antibody (ANA), anti-double stranded DNA antibody (anti-ds-DNA antibody) and anticardiolipin antibody (aCL antibody). Echocardiography revealed left and right ventricular obliteration with fibromatous biventricular endothelial thickening. Brain MRI showed changes in favour of white matter ischaemia and lacunar infarction. Hypereosinophilic syndrome (HES) and systemic lupus erythematosus may be considered to have occurred concurrently in this patient.
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PMID:Systemic lupus erythematosus and hyper-eosinophilic syndrome: an unusual association. 1956 3


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