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Cerebral venous thrombosis in nephrotic syndrome.

This report describes cerebral venous sinus thrombosis, a rare and perhaps under-diagnosed complication of nephrotic syndrome. We review the pathophysiology of the coagulopathy associated with nephrotic syndrome including abrupt renal loss of antithrombin III. We propose a rationale approach to treating this condition with low-molecular-weight heparin and antithrombin III replacement.

Aged↗

Utility of contrast-enhanced 3D turbo-flash MR angiography in evaluating the intracranial venous system.

The objective of this study was to compare the effectiveness of contrast-enhanced 3D turbo-flash and 2D time-of-flight (TOF) magnetic resonance angiography (MRA) sequences in the visualization and evaluation of the intracranial venous system. A prospective study was carried out on 41 patients referred to our Magnetic Resonance Imaging (MRI) unit with clinical findings suggestive of dural sinus thrombosis. Contrast-enhanced 3D turbo-flash and 2D TOF MRA sequences were performed, and the dural sinuses and cerebral veins were classified into five grades according to the quality of visualization and presence of thrombosis. We found the dural sinuses and cerebral veins to be normal in all sequences in 31 patients. Thrombosis of dural sinuses was detected in ten patients, with four of these ten cases found only in the contrast-enhanced 3D turbo-flash sequence. In general, complete visualization of cerebral veins and dural sinuses was significantly better accomplished with contrast-enhanced 3D turbo-flash MRA than with 2D TOF in either coronal or sagittal/oblique planes. Although 2D TOF MRA may be superior in detecting chronic dural sinus thrombosis, contrast-enhanced 3D turbo-flash MRA sequences may offer advantages for the early diagnosis and management of acute and subacute dural sinus thrombosis.

Adolescent↗

Cranial nerve syndrome in thrombosis of the transverse/sigmoid sinuses.

Cerebral venous thrombosis (CVT) is a cerebrovascular disease associated with a wide variety of clinical signs and symptoms, which may often delay appropriate diagnosis. Single or multiple cranial nerve palsies (III-VIII) without evidence of other signs and symptoms have not, so far, been considered a relevant syndrome of CVT. This event turned out to be a characteristic finding in five patients with thrombosis of the ipsilateral transverse/sigmoid sinus, who were recruited prospectively over a 14-month period. The diagnosis was supported by non-invasive MRI with the application of a newly developed subtraction technique. In view of the considerable mimics of the syndrome, and the long-standing need for conventional angiography to confirm the disease, it is likely to have been underestimated in the past; since appropriate treatment seems possible the diagnosis and utility of early MR venography should be considered in patients with single or multiple cranial nerve lesions of uncertain aetiology.

Adult↗

Neuropsychological manifestations in a case of bilateral thalamic infarction.

Neuropsychological manifestation has been reported with lesions of the anterior and non-specific thalamic nuclei and mammilothalamic tract (MMT). These have been reported in the setting of arterial infarction and/or haemorrhage. Cerebral venous sinus thrombosis (CVT) is a rare cause of brain infarction. It occurs in the setting of oral contraceptive administration or pregnancy. Inherited thrombophilias are documented risk factors. The most frequent being heterozygous factor V Leiden mutation. We report a single case of bilateral thalamic infarction due to cerebral vein and sinus thrombosis. Clinically the case manifested with memory impairment and dysexecutive symptoms. Predisposing factor for venous thrombosis was a homozygous factor V Leiden mutation. The patient was treated with anticoagulation and made a good recovery.

Adult↗

[Intra-extracranial meningioma in the cranial vault].

Between 1982 and 1993, 11 cases of intra-extracranial vault meningiomas were treated in Créteil, by the same multidisciplinary team. The average age was 53.5 years and the Karnofsky rating was high (> 80 in 7 cases). Ten of the 11 cases had medial tumours with venous sinus thrombosis occurring in 9 of these. The location of the tumour was frontal in 6 and parietal in 4 patients. Of the medial tumours, 5 (half) were bilateral intracranial lesions. The average duration of symptoms was more than 10 years in four cases, between 1 and 3 years in five, and the diagnosis was made immediately in two patients. MRI coronal sections, after venous contrast injection, allowed diagnosis of the lesion and visualisation of the sinus thrombosis. Embolisation was performed pre-operatively in all cases scheduled to have surgery, resulting in safe excision of the tumour. Surgery was performed in 9 patients with complete tumour removal achieved in 8. Of those who did not undergo surgery, one received radiotherapy and the other died before treatment. The bony defect was covered with cadaveric bone, autogenous bone, coral and methyl-methacrylate in one, two, two and two patients respectively. Complications occurred in 2 cases: extradural secondary infection and CSF rhinorrhea treated with a lumbo-peritoneal shunt. The post-operative Karnofsky rating was greater than 90 in seven cases, 70 in one, and 50 in two cases. After an average follow-up of 4 years, 3 patients had tumor recurrence at 4, 7, and 8 years; the first having a locally malignant character, the second showing radiological recurrence in a case of subtotal removal, and the third a marginal recurrence. Two of the 3 patients underwent further surgery. The malignant case, received radiotherapy but unfortunately a new recurrence occurred 14 months later. As radiotherapy was used in only 3 cases, statistically significant conclusions concerning efficacy cannot be drawn.

Adult↗

Neuro-Behçet disease. A review.

BACKGROUND: Behçet disease is a vasculitis with mucocutaneous, ocular, arthritic, vascular, and other manifestations. Its neurologic manifestations (neuro-Behçet disease) are relatively rare, but they must be thoroughly investigated due to their grave prognosis. REVIEW SUMMARY: The frequency of neurologic manifestations, more common in male Behçet patients, is between 5% and 30%. Both the central and peripheral nervous systems can be involved. Central nervous system manifestations can be divided into 2 main groups: (1) parenchymal involvement, which includes brainstem involvement, hemispheric manifestations, spinal cord lesions, and meningoencephalitic presentations; (2) nonparenchymal involvement, including dural sinus thrombosis, arterial occlusion, and/or aneurysms. Peripheral neuropathy and myopathy are relatively rare. Cerebrospinal fluid analysis reveals pleocytosis and elevated protein levels. Magnetic resonance imaging is the investigation of choice which often reveals iso-/hypointense lesions in T1-weighted images and hyperintense lesions in T2-weighted images, mostly in the mesodiencephalic junction, cerebellar peduncles, and other parts of the brainstem. Corticosteroids and adjuvant immunosuppressive therapy are used for parenchymal manifestations, and corticosteroids and anticoagulants are used for treatment of dural sinus thrombosis. CONCLUSION: Neuro-Behçet disease must be considered in the differential diagnosis of stroke in young adults, multiple sclerosis, movement disorders, intracranial hypertension, intracranial sinovenous occlusive diseases, and other neurologic syndromes.

Behcet Syndrome↗

Orbital abscess: management and outcome.

PURPOSE: To discuss the diagnosis, management and outcome of various types of orbital abscess. METHODS: The medical records of 13 patients diagnosed and treated for orbital abscess were reviewed. The sources of infection included: paranasal sinusitis (n = 5), odontogenic origin of infection (n = 4), one each, temporal fossa abscess, palatal abscess, furuncle on the nose, and secondary to retrobulbar injection of steroid. Computed tomographic scans revealed the presence of an abscess in all 13 cases. Associated findings on CT scan included: sinus disease (n = 8), cavernous sinus thrombosis (n = 2) and subdural empyema (n = 2). All patients were treated with intensive, multiple, intravenous antibiotics and early surgical drainage. RESULTS: Purulent material collected surgically from the orbit cultured Staphylococcus aureus (n = 3), two each Pseudomonas aeruginosa, Proteus mirabilis, Acinetobacter species and one each beta-haemolytic Streptococci, Citrobacter frundi and Enterobacter. Final visual acuity was good in 6 patients (6/12-6/6) and no light perception in 6 others. Visual acuity could not be recorded in the infant. The other complications were intracranial abscess (n = 4), cavernous sinus thrombosis (n = 2) and restricted ocular motility (n = 1). CONCLUSIONS: A high index of suspicion is necessary, along with early institution of appropriate diagnostic imaging, and aggressive medical and surgical treatment for a favourable outcome in cases of orbital abscess.

Abscess↗

Local thrombolytic therapy for cerebrovascular disease: current Oregon Health Sciences University experience (July 1991 through April 1995).

PURPOSE: This report details experience with local intraarterial or intradural sinus thrombolytic therapy for cerebrovascular thromboembolic occlusions in 40 patients. PATIENTS AND METHODS: Between July 1991 and April 1995, intracranial local thrombolytic therapy with urokinase was used to treat 40 patients with occlusive vascular disease. Twenty-six patients had acute occlusions of the central retinal artery, middle cerebral artery, basilar artery, or combined internal carotid and middle cerebral arteries. Three patients had embolic complications related to cerebral vascular embolization procedures. Five patients were undergoing intracranial angioplasty procedures for occlusive atheromatous disease. Six patients had dural sinus thrombosis. RESULTS: Local intraarterial thrombolytic therapy for acute thromboembolic arterial occlusions resulted in excellent restoration of perfusion in 18 patients, partial restoration of flow in four patients, and no effect in five patients. Fourteen of these patients had excellent clinical outcomes, seven made moderate improvements, and six died. In the two patients with central retinal arterial occlusions, no angiographic or clinical response to thrombolytic therapy could be ascertained. There was no angiographic improvement response from thrombolytic therapy in five patients with primary intracranial atheromatous stenosis, and one patient may have had an embolic complication related to this therapy. Three of six patients with dural sinus thrombosis had clearing of the thrombus and an excellent clinical result. The remaining three with extensive dural thrombosis did not have clearing of the thrombus; one patient became blind, and two patients died. Among the 40 patients treated, significant cerebral hemorrhage occurred after therapy in four. CONCLUSION: Local thrombolytic therapy for thromboembolic occlusive cerebrovascular disease is useful in restoring perfusion of acutely occluded vessels. Further experience is needed to fully identify the most appropriate patients for therapy, dose of thrombolytic agent, timing and length of therapy, and risk factors for hemorrhage.

Adolescent↗

Multiple coagulation defects and the Cohen syndrome.

A 13-year-old male presented with new onset seizures, sagittal sinus thrombosis with cerebral hemorrhage, and extensive venous thrombosis of the lower limbs. Laboratory investigation demonstrated combined deficiency of protein C, protein S, and antithrombin III. He and his 17-year-old sister had a mental retardation-multiple anomaly syndrome associated with microcephaly, unusual facies, and lax connective tissue. Their dysmorphology included elongated faces with narrow forehead, arched eyebrows, large mouth with down-turned corners, malformed teeth, and furrowed tongue. Both had Marfanoid habitus with lax joints, pectus excavatum, kyphoscoliosis, and flat narrow feet. The most likely diagnosis for these siblings is the autosomal recessive Cohen syndrome of mental retardation, congenital hypotonia with Marfanoid habitus, microcephaly, pleasant affect, micrognathia, and open mouth with prominent incisors. The sagittal sinus thrombosis, left frontal intracranial hemorrhage, carotid aneurysm, tortuous descending aorta, and deep venous thrombosis suffered by the male sibling adds the Cohen syndrome to genetic vasculopathies that may be associated with stroke.

Adolescent↗

Isolated deep venous thrombosis--case series, literature review and long term follow up.

Cerebral Venous Sinus thrombosis may rarely be isolated to a cortical vein or to the deep venous system. When the deep venous system is involved, prognosis is generally poor. In addition, long term follow up is not reported. We conducted a retrospective review of all patients admitted to a major tertiary care center, with the diagnosis of isolated deep venous thrombosis. Two patients were identified with isolated involvement of the deep venous system, they are reviewed in detail with long term follow up. Two young South Asian women in their thirties with rapid onset of neurologic signs and symptoms are reported. Even when one patient required intubation and mechanical ventilation for stupor, both had excellent neurologic recovery. Over 6 years of follow up there has been no recurrence. In spite of stupor at presentation, complete recovery is possible without long term recurrence.

Adult↗

Behçet's Disease.

Clinical and imaging data suggest that Behçet's disease (BD) can present with a variety of neurologic complications, which may be subclassified into two forms. One is attributable to small venous inflammatory disease with focal or multifocal central nervous system involvement and is seen in the majority of patients. It is designated Central Nervous System-Neuro-Behçet syndrome (CNS-NBS). The other form is due to cerebral venous sinus thrombosis and has limited symptoms and a better prognosis. It is very uncommon for these two types of involvement to occur in the same individual. It is very likely that the two major forms have different pathogeneses. Patients with small vein inflammation should be considered for aggressive treatment. A substantial number of patients in this group will have a relapsing-remitting course; for some, this will evolve into a secondary progressive course. A few patients from this group will have progressive CNS dysfunction from the onset. Acute attacks of CNS-NBS are treated with either oral prednisone (1 mg/kg/d up to 4 weeks or until improvement is observed) or high-dose intravenous methylprednisolone (IVMP), 1 g/d for 5 to 7 days. After either treatment, an oral tapering dose of glucocorticoids should be given over 2 to 3 months to avoid early relapses. Some patients may require the long-term use of low maintenance doses of glucocorticoids to prevent exacerbations. Immunosuppressive agents (eg, azathioprine, cyclosporine A, cyclophosphamide, and chlorambucil), given either alone or in different combinations (eg, azathioprine with cyclosporine) for the long-term treatment of various systemic manifestations of BD, have not been shown to prevent the development of the neurologic complications of the disease, reduce its exacerbations, or stop its progression. Immunomodulatory treatments such as interferon alfa and thalidomide have been shown to be effective in treating some of the systemic manifestations of BD, but there is no information on their effects on the development and progression of CNS-NBS. Cerebral venous sinus thrombosis in BD is treated with either intravenous unfractionated heparin or subcutaneous low-molecular-weight heparin given together with a short course of glucocorticoids. Evidence of benefit from this treatment, however, is weak.

Journal Article↗

[Septic thrombosis of the cavernous sinus. Presentation of a clinical case].

This paper describes a case of cavernous sinus septic thrombosis with cerebellar complication. The CNS was involved after a primitive piodermitis of the face. The diagnostic tools employed in the management of the case and the therapeutic procedures in the acute phase and in the convalescence are discussed. The patient completely recovered after chemotherapeutic treatment.

Adolescent↗

Influence of cerebral venous obstruction on cerebral circulation in humans.

The influence of cerebral venous obstruction on cerebral circulation was investigated in three patients with cerebral venous and sinus thrombosis in the chronic stage. The xenon Xe 133 intracarotid injection method was used to evaluate the cerebral blood flow (CBF). Values for CBF showed a slight decrease in all patients, despite intact cerebrovascular autoregulation. In two of the three patients, hypercapnia failed to increase CBF, but hyperventilation decreased CBF normally. This phenomenon can be explained on the basis of both failure of the capacitance vessels on the venous side to dilate and an increased intracranial pressure and is not attributable to decreased carbon dioxide reactivity. Long-lasting obstruction of the venous system can lead to a slight reduction in CBF and not affect cerebral autoregulation or CO2 reactivity itself in humans.

Adolescent↗