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

Antiphospholipid syndrome is characterized with arterial and venous thrombosis. In this article, a 38-year-old man presented with headache and swelling of his left limb, which had lasted for 2 months. Duplex ultrasonography showed thrombosis of the left femoral vein. Cerebral magnetic resonance imaging also showed cerebral sinus thrombosis. Serological examination showed that antiphospholipid syndrome was the underlying disease condition in this patient. Despite adequate anticoagulant therapy, deep vein thrombosis and cerebral sinus thrombosis exacerbated, resulting in inferior vena cava occlusion, papilloedema, and abducent nerve paralysis. Optic canal decompression and cistern-peritoneal shunt operation were performed, following which his neurological symptoms were relieved. The occurrence of cerebral sinus thrombosis as initial presentation of antiphospholipid syndrome is extremely rare and remains a diagnostic challenge. Although the clinical presentation is highly variable, the diagnosis should be considered in patients with antiphospholipid syndrome presenting with recent unusual headaches. Improved diagnosis and treatment strategy may ultimately improve the clinical outcome of these patients.
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PMID:Simultaneous presentations of deep vein thrombosis and cerebral sinus thrombosis in a case of primary antiphospholipid syndrome. 1838 66

Migraine headache (with and without aura) is common in the general population and is known to be influenced by genetic factors with heritability estimates between 34-57%. Antiphospholipid syndrome (APS) is a hypercoagulable state characterized by clinical features including venous and arterial thromboses, pregnancy loss and migraine, and by association with antiphospholipid antibodies (aPL). Numerous small studies have investigated whether aPL are associated with migraine in the general population--with contradictory results. In this study, the question was addressed by studying the prevalence of aPL in members of monozygotic (MZ) twin pairs differing in their migraine status. Such twins provide a unique natural experiment, matched as they are for age, sex and genetic factors, and allow the role of environmental factors, such as aPL, to be determined. Despite 95% power to detect a difference of 0.59 IgG units per litre in anticardiolipin antibody IgG titres, no difference in prevalence of aPL could be detected in migraine-discordant MZ twins.
Cephalalgia 2008 Oct
PMID:Migraine and antiphospholipid antibodies: no association found in migraine-discordant monozygotic twins. 1862

The antiphospholipid syndrome (APS) is defined by the presence of antiphospholipid antibodies (aPL), associated with thrombosis or recurrent spontaneous abortions. APS can occur alone or secondary to other conditions, especially associated to inflammatory systemic autoimmune diseases. Among the neurological manifestations associated with aPL, only ischemic stroke is recognized by the actual classification criteria for APS. Other neurological manifestations have been, however, repeatedly reported in case studies of APS patients. Headache, and especially migraine, was commonly reported in APS patients and is one of the classical features described by Hughes as related to aPL, but studies failed to confirm this association. We studied retrospectively the association between headache syndromes and aPL in 428 patients with inflammatory connective tissue diseases admitted in the Neurology and Internal Medicine Departments of Colentina Hospital-Bucharest. We found that migraine alone, not headache of all types, is significantly associated with aPL in patients with systemic immune disease. We studied the presence of cerebral ischemia in patients with headache and aPL. In SLE patients, headache (all types) is significantly associated with positive titers of aPL, and cerebral ischemic lesions are significantly encountered. Even if both migraine and aPL are conditions with high frequency in patients with immune systemic disease and their association may be coincidental, the presence of ischemic lesions in patients showing this association suggests the need to define a sub-group at risk, for whom headache can be a marker and anticoagulants can be discussed.
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PMID:Antiphospholipid antibodies and migraine: a retrospective study of 428 patients with inflammatory connective tissue diseases. 1876 11

Antiphospholipid syndrome (APS) is one of the most common reasons of thromboembolic complications in the course of connective tissue diseases. There is a strong relationship between presence of antiphospholipid antibodies and the risk of thrombosis. APS related thromboembolic complications range from superficial vein thrombosis up to rapidly developing, life threatening, multi-organ embolism. We present a case of a 21-year old female who presented with myocardial infarction as the first symptom of systemic lupus erythematosus and APS. Afterwards she was consequently treated with antithrombotic and anti-aggregation agents but it did not prevent her from reoccurrence of middle retinal vein. During the 10-year follow up she presented with reoccurring neurological symptoms i.e.: headache, memory deficits, concentration loss and impairment of the cognitive functions. She was found many times to be positive for IgG and IgM anticardiolipin antibodies, anti-beta2glicoprotein-I antibodies and lupus anticoagulant.
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PMID:[Therapy resistance antiphosholipid syndrome in the course of systemic lupus erythematosus: case report]. 1877 25

Postpartum cerebral vein thrombosis is a very rare entity (3-4 cases per million). Clinical manifestations vary, though persistent headache is almost always reported, meaning that differential diagnosis should be performed to rule out other causes of postpartum headache. Recognized risk factors for this disease are the prothrombotic state of pregnancy (third trimester), excess weight, and thrombophilia Accidental dural puncture, protein C and S deficiencies, factor V Leiden mutation, antiphospholipid syndrome, and the use of oral contraceptives have also been implicated. The diagnostic test of choice is magnetic resonance imaging, as it is convenient and harmless, though transcranial Doppler ultrasound can also be used. Pulmonary angiography is the gold-standard test. The treatment of choice is anticoagulant therapy with heparin (a treatment that is controversial, however, due to the high risk of rebleeding), followed by long-term treatment with antivitamin K drugs. We report a case of thrombosis of the upper longitudinal sinus associated with persistent postpartum headache and a history of a failed attempt at epidural puncture for analgesia during labor. The case posed interesting diagnostic questions.
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PMID:[Sagittal sinus thrombosis as a rare cause of postpartum headache: a case report]. 1885 84

To examine the clinical manifestations, intensity of oral anticoagulation and outcomes in the prevention of recurrent thromboses in patients with antiphospholipid syndrome (APS) in a tertiary rheumatology centre in Singapore. Retrospective case review of consecutive patients with APS attending a rheumatology clinic from 1st January 2004 to 31st December 2005. There were 59 (44%) patients with definite APS and 75 (56%) with probable APS. Systemic lupus erythematosus (SLE) was the most common cause of secondary APS. Hypertension and hyperlipidaemia were the most common cardiovascular comorbidities. The most common manifestations were haematological (thrombocytopaenia and haemolytic anaemia), neurological (seizure, headache) and pulmonary hypertension. Among those with definite APS, there were similar proportions with arterial and venous thromboses. Recurrent thromboses occurred in 14 (23.7%) patient with definite APS receiving warfarin, comprising 14 (73.7%) episodes of arterial and 5 (26.3%) episodes of venous thromboses. Recurrent arterial thromboses occurred at international normalized ratio (INR) of <2 in 5 (35.7%), INR 2-3 in 6 (42.9%), INR > 3 in 3 (21.4%) episodes, respectively. Recurrent venous thromboses occurred at INR < 2 in 4 (80.0%) and INR > 3 in 1 (20.0%) episode, respectively. Twenty-eight episodes of bleeding occurred in 21 (35.6%) patients, the majority (78.6%) being minor bleeding. Two-thirds of all major bleeds occurred at INR >/= 3. Venous and arterial thromboses were equally common in our patients with definite APS, although recurrent thromboses were more common in the arterial circulation. Target INR > 3 was associated with lower rates of recurrent arterial thromboses but higher rates of major and recurrent bleeding. Target INR >/= 2 appeared to be sufficient to prevent recurrent venous thromboses.
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PMID:Clinical manifestations and outcomes of antithrombotic treatment of the Tan Tock Seng Hospital Singapore antiphospholipid syndrome cohort. 1950 74

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.
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PMID:Isolated elevation of IgA anti-beta2glycoprotein I antibodies with manifestations of antiphospholipid syndrome: a case series of five patients. 1976 4

Cerebral venous sinus thrombosis, an unusual presentation of antiphospholipid syndrome, is a rare condition in pregnancy, particularly in the first trimester. The authors of this case report present the case of a 20-year-old woman with sudden onset of headaches and hemiparesis in the first trimester of pregnancy. She underwent a computed tomography scan and magnetic resonance imaging. The image findings and the autoimmune serum test were consistent with a cerebral venous sinus thrombosis complicated by primary antiphospholipid syndrome. The patient's hemiparesis improved with subcutaneous low molecular weight heparin and oral aspirin. In the third trimester of pregnancy, she developed seizures that were controlled by antiepileptic drugs. She delivered a healthy baby at 37 weeks' gestation without immediate or late postpartum complications.
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PMID:Cerebral venous sinus thrombosis in early pregnancy: an unusual presentation of primary antiphospholipid syndrome. 2002 37

Classification criteria, etiology, pathogenesis, major central nervous system (CNS) manifestations of the antiphospholipid syndrome (APS), as well as diagnostic and therapeutic approach are discussed in the article, supported by several MRI findings to illustrate differential complexity of selected topics. Close interplay of inflammation, autoimmunity, coagulation cascade, vasculature bed, neuron physiology and demyelinization in APS is elaborated. Cerebrovascular disease, multiple sclerosis-like syndrome, seizures, cognitive disfunction, headache and migraine, chorea and catastrophic antiphospholipid syndrome (CAPS) are discussed as the most prominent CNS manifestations of the APS.
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PMID:Antiphospholipid syndrome and central nervous system. 2041 26

Antiphospholipid syndrome (APS) is an autoimmune disease with recurrent thromboses and pregnancy complications (90% are female patients) that can be primary and secondary (with concomitant autoimmune disease). Antiphospholipid antibodies are prothrombotic but also act directly with brain tissue. One clinical and one laboratory criterion is necessary for the diagnosis of APS. Positive serological tests have to be confirmed after at least 12 weeks. Clinical picture consists of thromboses in many organs and spontaneous miscarriages, sometimes thrombocytopaenia and haemolytic anaemia, but neurological cases are the most frequent: headaches, stroke, encephalopathy, seizures, visual disturbances, Sneddon syndrome, dementia, vertigo, chorea, balism, transitory global amnesia, psychosis, transversal myelopathy and Guillain-Barre syndrome. About 50% of strokes below 50 years of age are caused by APS. The first line of therapy in stroke is anticoagulation: intravenous heparin or low-weight heparins. In chronic treatment, oral anticoagulation and antiplatelet therapy are used, warfarin and aspirin, mostly for life. In resistant cases, corticosteroids, intravenous immunoglobulins and plasmapheresis are necessary. Prognosis is good in most patients but some are treatment-resistant with recurrent thrombotic events and eventually death.
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PMID:[Antiphospholipid syndrome in neurology]. 2118 99


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