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Protein S Tokushima: an abnormal protein S found in a Japanese family with thrombosis.

An abnormal protein S (PS) was found in a Japanese family with a high incidence of thrombosis. The proband is a woman who was born in Tokushima Prefecture. She had superior sagittal sinus thrombosis, thrombophlebitis of the left leg, and thrombosis of the placenta. She had a normal plasma level of free PS antigen but decreased PS activity. Her mother and aunt also had thrombophlebitis of the leg, and together with four other family members also showed a normal level but decreased activity of PS. This suggests that hereditary dysfunction of PS is inherited in this family as an autosomal dominant trait. The proband's PS appears to have a slightly higher molecular weight than normal PS both in the intact and modified form, suggesting that it has a molecular defect on the carboxyl-terminal side of the thrombin-sensitive site. This abnormal PS with apparently unique characteristics was named PS Tokushima.

Adult↗

Primary presentation of spontaneous jugular vein thrombosis to the otolaryngologist--in three different pathologies.

An otolaryngologist is conversant with the potential of middle-ear disease leading to lateral sinus thrombosis and subsequent jugular vein thrombosis, although this is becoming an increasingly rare event. Spontaneous jugular vein thrombosis from non-otological causes, however, is not well described in the otolaryngology literature. We present three cases of jugular vein thrombosis who presented primarily to the Otolaryngology department each attributable to a different pathology including discoid lupus erythematosus, antiphospholipid syndrome and metastatic malignancy. Awareness needs to be increased of this condition in the differential diagnosis of acute neck swelling and neck pain.

Adult↗

Intracerebral thrombosis. Case report and brief clinical review.

A 29-yr-old writer presented with seizures and left hemiparesis 8 days post-partum. Studies revealed right parietal hemorrhagic infarction secondary to superior sagittal sinus thrombosis. An anticoagulant was given for clot extension associated with increasing cerebral edema and coma. Inpatient rehabilitation was undertaken for residual left hemiparesis, most severe in the leg. Left arm strength rapidly returned to normal. Significant improvement in left leg strength occurred but was delayed for many months. Intracerebral thrombosis is an uncommon but significant cause of stroke in young adults. It frequently occurs in the puerperium and may be associated with unilateral or bilateral neurologic deficits. Treatment with anticoagulants is controversial because of the risk of hemorrhagic cerebral infarction, but may be beneficial in some cases. Recovery may be delayed for several months pending recanalization of the sinus or the development of collateral circulation. The overall prognosis for neurologic and functional recovery in survivors of intracerebral thrombosis is good.

Adult↗

Pulsatile tinnitus--a review of 84 patients.

Pulsatile tinnitus can be annoying for a patient and can also be the only clue to a potentially devastating and life-threatening disease. In order to understand its clinical spectrum and management better we analysed the files of 84 patients seen at our institution over a 10-year period. Noninvasive techniques (ultrasound, computed tomography, magnetic resonance imaging) and angiography were employed as investigations tailored to the individual patient. A vascular disorder [i.e. arteriovenous fistula, dissection of the internal carotid artery (ICA), fibromuscular dysplasia, aneurysm of the ICA and sinus thrombosis] was found in 36 patients (42%), most commonly a dural arteriovenous fistula or a carotid-cavernous sinus fistula. In 26 patients with a vascular abnormality, pulsatile tinnitus was the presenting symptom. In 12 patients (14%), nonvascular disorders such as glomus tumour or intracranial hypertension with a variety of causes explained the tinnitus. We conclude that patients with pulsatile tinnitus should be investigated with noninvasive techniques. If these are negative or to clarify abnormal findings of noninvasive techniques selective angiography is needed for diagnosis and to guide treatment.

Adolescent↗

Intermittent coronary sinus occlusion in dogs: reduction of infarct size 10 days after reperfusion.

Intermittent balloon occlusion of the coronary sinus was applied to 11 open chest dogs subjected to 3 hours of ligation of the left anterior descending coronary artery followed by 8 to 12 days of reperfusion. Anticoagulants were not given during the reperfusion period. Risk region was assessed by planimetry of autoradiographs made from ventricular slices. Infarct size was equivalent when assessed by planimetry of ventricular slices before and after staining with triphenyltetrazolium chloride. In the seven survivors, 30 +/- 8% of the risk region was infarcted. Seven of 11 control dogs survived (p = NS); 75 +/- 4% of the risk region was infarcted in the control animals (p less than 0.01 versus treated survivors). Light microscopic inspection of specimens stained with hematoxylin-eosin confirmed the border between necrotic and preserved myocardium. Thrombus was observed in the coronary sinus in all survivors in the treatment group. These findings confirm earlier short-term studies that demonstrated a potent anti-ischemic effect of intermittent coronary sinus occlusion. At the same time, coronary sinus thrombosis warrants caution in the application of this technique to myocardial ischemia in humans.

Animals↗

Intracranial dural arteriovenous fistulas: analysis of 60 patients.

OBJECTIVE: To analyze and update the clinical symptomatology, CT and MRI findings, angiographic features, and therapeutic outcomes of patients with dural arteriovenous fistulas (DAVFs). BACKGROUND: Studies of DAVFs in a large number of patients have seldom appeared in neurology literature. METHODS: The authors investigated 60 consecutive patients with DAVFs who were admitted between January 1991 and January 2001. The DAVFs were graded into five types according to the classification of Cognard et al. [Radiology 1995;194:671-680]. Presumable etiologies, clinical features, imaging findings and therapeutic outcomes were evaluated on the basis of the location and type of DAVFs. RESULTS: Sinus thrombosis, previous trauma, craniotomy, cerebral infarction and acupuncture were detected as possible etiologic factors of DAVFs. The cavernous sinus (57%) was the most common location of DAVFs. Although the neurological symptoms were closely related to the location of the DAVFs, in some patients, there were also symptoms that did not reflect the location. Although the women outnumbered the men, the men presented with aggressive neurological manifestations more often (p < 0.05). Ten out of 12 patients (83%) with DAVFs involving locations other than the large sinuses presented with aggressive neurological manifestations. 70% of brain CTs and 81% of brain MRIs showed abnormal findings suggestive of DAVFs. Of 33 patients who underwent only endovascular embolization, 29 patients (88%) were cured or improved. Radiosurgery and surgical excision done in some patients provided fair results. Patients with DAVFs involving large sinuses showed a better therapeutic outcome than those in whom locations other than the large sinuses were involved, while patients without venous ectasia had more a favorable outcome than those with it (each, p < 0.01). CONCLUSIONS: DAVFs result from various etiologic factors, show diverse manifestations usually reflecting the location and can be treated successfully in most patients. Factors related to poor clinical outcome include male sex, the presence of venous ectasia and involved locations other than the large sinuses.

Adult↗

Cerebral venous thrombosis in Behçet's disease: clinical study and long-term follow-up of 25 cases.

Among 250 patients with Behcet's disease, we describe 25 cases of angiographically proven cerebral venous thrombosis. Intracranial hypertension was the most frequent manifestation. Two initially untreated patients relapsed. Treatment of cerebral venous thrombosis consisted of combined heparin and steroids in 19 patients, steroids alone in three, and heparin alone in three others. Neurologic symptoms improved rapidly in all. Nineteen patients received long-term anticoagulation, and two received aspirin. Relapse of cerebral venous thrombosis or development of optic atrophy did not occur in treated patients. Partial or total recanalization of the occluded sinus was frequent. After more than 3 years of follow-up, the prognosis of dural sinus thrombosis is satisfactory.

Adolescent↗

Cerebral venous thrombosis during diabetic ketoacidosis.

Neurological deterioration during an episode of diabetic ketoacidosis is usually assumed to be caused by cerebral oedema. We present a case of cerebral venous sinus thrombosis presenting in a similar manner, also associated with severe iron deficiency anaemia. Computed tomography scanning provided the correct diagnosis and allowed institution of anticoagulation with improvement in neurological outcome. Neuroimaging should always be performed in suspected cerebral oedema associated with diabetic ketoacidosis in order to exclude other pathologies.

Anticoagulants↗

Management of catheter-related injuries to the coronary sinus.

BACKGROUND: Although coronary sinus catheter-related injuries (CSCRIs) are rare, they are potentially lethal. The purpose of this study was to evaluate such injuries, the repair methods used, and to identify related risk factors for mortality. METHODS: A retrospective review of 10,552 cardiac surgical procedures from 1995 to 2000 in which retrograde cardioplegia was used revealed 10 cases (n = 10) of CSCRIs (0.095%) at our center. These injuries occurred during coronary bypass, valve replacement, and combined procedures. Management included direct suture, vein patch, or pericardial "on-lay" patch repair. RESULTS: Two deaths occurred (20% mortality) from failure of CSCRI repair; 8 of 10 injuries (80%) were successfully repaired. One patient had delayed, localized pericardial tamponade, which resolved spontaneously. Two patients had recurrent angina that was assessed 3 and 5 years later by coronary angiography; the coronary sinus was found to be patent in both cases. The remaining 6 patients have been asymptomatic. CONCLUSIONS: Repair of CSCRIs can be challenging as it can be complicated by inadequate myocardial protection, inadvertent coronary artery injuries, and possibly, subsequent coronary sinus thrombosis. Repair of CSCRIs should be carried out on an arrested, well-protected heart providing secure hemostasis and coronary sinus patency.

Aged↗

Lupus anticoagulant, factor V Leiden, and methylenetetrahydrofolate reductase gene mutation in a lupus patient with cerebral venous thrombosis.

We describe the case of a young Lebanese woman with systemic lupus erythematosus (SLE) and a positive lupus anticoagulant (LAC) who developed right internal jugular vein and sigmoid sinus thrombosis. Coagulation studies showed that in addition to the LAC the patient was heterozygous for the factor V (FV) Leiden mutation, and C677T mutation of the methylenetetrahydrofolate reductase gene. The high prevalence of FV Leiden in the eastern Mediterranean region suggests that we should probably screen our SLE patients in this area, especially those with anticardiolipin antibodies and/or LAC who have no history of thrombosis, for this and other thrombophilia markers. The detection of such abnormalities may have major practical consequences for the long-term management of these patients to prevent further thrombotic episodes.

Adolescent↗

Paroxysmal nocturnal haemoglobinuria in pregnancy: case report.

Pregnancy in a woman with paroxysmal nocturnal haemoglobinuria was complicated by hepatic venous thrombosis, portal hypertension and haematemesis due to gross oesophageal varices. Labour was induced and a live female infant was born. Dural sinus thrombosis and intracerebral haemorrhage resulted in death on the 46th day post partum. A review of the literature shows that venous thrombosis is common in paroxysmal nocturnal haemoglobinuria.

Adult↗

Cerebral thrombosis in a newborn with a congenital deficiency of antithrombin III.

An Israeli Arab family with type I antithrombin III (AT-III) deficiency with several affected symptomatic members in three generations is reported. The propositus presented with deep vein thrombosis and pulmonary emboli associated with gestation. The propositus infant presented at the age of 2 weeks with superior sagittal and rectus sinus thrombosis. Hereditary AT-III deficiency should be considered in infants with cerebral thrombosis, especially if they have a family history of thromboembolism. The role of prophylactic therapy by AT-III concentrates in these infants should be further assessed.

Adult↗

Restricted diffusion in bilateral optic nerves and retinas as an indicator of venous ischemia caused by cavernous sinus thrombophlebitis.

A 44-year-old man developed bilateral blindness following severe periorbital cellulitis and pansinusitis. CT and MR imaging demonstrated bilateral cavernous sinus thrombosis. Diffusion-weighted imaging revealed reduced apparent diffusion coefficient in bilateral optic nerves, suggesting optic nerve ischemia caused by the cavernous sinus thrombophlebitis (CST). Following surgical debridement of pansinusitis, antimicrobial therapy, and anticoagulation, the patient recovered from the infectious episode but sustained permanent bilateral blindness. This case shows that both retinal and optic nerve ischemia can be the cause of blindness after CST. Arguments supporting an arterial-versus-venous origin for the ischemia are discussed.

Adult↗