Superficial siderosis Kumar, N; Cohen-Gadol, A A; Wright, R A ...
Neurology,
04/2006, Letnik:
66, Številka:
8
Journal Article
Recenzirano
Superficial siderosis (SS) of the CNS is caused by repeated slow hemorrhage into the subarachnoid space with resultant hemosiderin deposition in the subpial layers of the brain and spinal cord. ...Despite extensive investigations, the cause of bleeding is frequently undetermined.
To review the clinical and imaging features of 30 consecutive patients with SS and provide insights into the underlying causes of subarachnoid bleeding in this disabling disorder.
The authors reviewed the medical records of 30 consecutive patients with clinical and MRI evidence of SS.
The commonest neurologic manifestations included gait ataxia and hearing impairment. A clinical history of subarachnoid hemorrhage was relatively rare. Possible predisposing conditions were identified on history in 22 patients, the commonest being a prior trauma (15 patients). In addition to the characteristic MRI findings of SS, 18 patients had abnormalities on MRI possibly related to chronic bleeding. The most common of these was the presence of a fluid-filled collection in the spinal canal seen in 14 patients.
A history of prior subarachnoid hemorrhage is often absent in patients with superficial siderosis (SS). A past history of trauma is common. Prior intradural surgery may be an additional risk factor. Xanthochromia or the presence of red blood cells in the CSF is a common finding. Only rarely does angiography demonstrate the bleeding source. The presence of a fluid-filled collection in the spinal canal is a common finding on MRI and is likely related to the SS. With longitudinally extensive cavities, a dynamic CT myelogram may help localize the defect and direct the site of laminectomy. Surgical repair of a dural defect, if present, should be considered. Surgical correction of bleeding should be documented by CSF examination months after surgery. Friable vessels in the dural defect are a possible source of the chronic bleeding.
Recent studies suggest that factors other than the degree of carotid stenosis are involved in ischemic stroke pathogenesis, especially modifications of plaque composition and related complications.
...To examine the role of carotid plaque rupture and thrombosis in ischemic stroke pathogenesis in patients undergoing carotid endarterectomy, excluding those with possible cardiac embolization or with severe stenosis of the circle of Willis.
A total of 269 carotid plaques selected from an Interinstitutional Carotid Tissue Bank were studied by histology after surgical endarterectomy between January 1995 and December 2002. A total of 96 plaques were from patients with ipsilateral major stroke, 91 plaques from patients with transient ischemic attack (TIA), and 82 plaques from patients without symptoms.
Differences in the frequency of thrombosis, cap rupture, cap erosion, inflammatory infiltrate, and major cardiovascular risk factors between study groups.
A thrombotically active carotid plaque associated with high inflammatory infiltrate was observed in 71 (74.0%) of 96 patients with ipsilateral major stroke (and in all 32 plaques from patients operated within 2 months of symptom onset) compared with 32 (35.2%) of 91 patients with TIA (P < .001) or 12 (14.6%) of 82 patients who were without symptoms (P < .001). In addition, a fresh thrombus was observed in 53.8% of patients with stroke operated 13 to 24 months after the cerebrovascular event. An acute thrombus was associated with cap rupture in 64 (90.1%) of 71 thrombosed plaques from patients with stroke and with cap erosion in the remaining 7 cases (9.9%). Ruptured plaques of patients affected by stroke were characterized by the presence of a more severe inflammatory infiltrate, constituted by monocytes, macrophages, and T lymphocyte cells compared with that observed in the TIA and asymptomatic groups (P = .001). There was no significant difference between groups in major cardiovascular risk factors.
These results demonstrate a major role of carotid thrombosis and inflammation in ischemic stroke in patients affected by carotid atherosclerotic disease.
Abstract
BACKGROUND:
Embolization of spinal dural arteriovenous fistulae (SDVAFs) has emerged as an alternative to surgery. However, surgical disconnection is a simple and effective procedure.
...OBJECTIVE:
To review results and complications of surgical treatment of 154 consecutive SDAVFs.
METHODS:
The records of 154 consecutive patients with SDAVFs were retrospectively reviewed.
RESULTS:
There were 120 males and 34 females (male/female ratio 3.5:1, mean age 63.6 years). The SDAVFs were located at the thoracic level in 92 patients and at the lumbar and sacral spine levels in 45 and 15 patients, respectively. The most common presenting symptoms were motor dysfunction (65 patients), sensory loss (31 patients), and paresthesias without sensory loss (13 patients). The mean interval from symptom onset to definitive diagnosis was 24.7 months (median 12 months). Surgery resulted in complete exclusion of the fistula at first attempt in 146 patients (95%). There were no deaths or major neurological complications related to the surgery. Six percent of patients experienced subjective or objective worsening of preoperative symptoms and signs by the time of discharge that persisted at follow-up. Other surgical complications consisted of wound infection in 2 patients and deep venous thrombosis in 3. Eight patients were lost to follow-up; 141 patients (96.6%) experienced improvement (120 patients, 82.2%) or stability (21 patients, 14.4%) of motor function at last follow-up compared with their preoperative status. Other symptoms such as numbness, sphincter dysfunction, and dysesthesias/neuropathic pain improved in 51.5%, 45%, and 32.6%, respectively.
CONCLUSION:
Surgical obliteration of SDAVFs is safe and very effective. Prognosis of motor function is favorable after surgical treatment.
The aim of this study was to determine age-related differences in short-term (1-year) outcomes in patients with unruptured intracranial aneurysms (UIAs).
Four thousand fifty-nine patients ...prospectively enrolled in the International Study of Unruptured Intracranial Aneurysms were categorized into 3 groups by age at enrollment: < 50, 50-65, and > 65 years old. Outcomes assessed at 1 year included aneurysm rupture rates, combined morbidity and mortality from aneurysm procedure or hemorrhage, and all-cause mortality. Periprocedural morbidity, in-hospital morbidity, and poor neurological outcome on discharge (Rankin scale score of 3 or greater) were assessed in surgically and endovascularly treated groups. Univariate and multivariate associations of each outcome with age were tested.
The risk of aneurysmal hemorrhage did not increase significantly with age. Procedural and in-hospital morbidity and mortality increased with age in patients treated with surgery, but remained relatively constant with increasing age with endovascular treatment. Poor neurological outcome from aneurysm- or procedure-related morbidity and mortality did not differ between management groups for patients 65 years old and younger, but was significantly higher in the surgical group for patients older than 65 years: 19.0% (95% confidence interval CI 13.9%-24.4%), compared with 8.0% (95% CI 2.3%-13.6%) in the endovascular group and 4.2% (95% CI 2.3%-6.2%) in the observation group. All-cause mortality increased steadily with increasing age, but differed between treatment groups only in patients < 50 years of age, with the surgical group showing a survival advantage at 1 year.
Surgical treatment of UIAs appears to be safe, prevents 1-year hemorrhage, and may confer a survival benefit in patients < 50 years of age. However, surgery poses a significant risk of morbidity and death in patients > 65 years of age. Risk of endovascular treatment does not appear to increase with age. Risks and benefits of treatment in older patients should be carefully considered, and if treatment is deemed necessary for patients older than 65 years, endovascular treatment may be the best option.
To characterize clinical and imaging features, biopsy findings, etiologic factors, and outcome in the syndrome of intracranial hypotension, headaches, and diffuse pachymeningeal gadolinium ...enhancement on magnetic resonance imaging (MRI).
We describe our experience with 26 consecutive patients with orthostatic headaches and diffuse pachymeningeal gadolinium enhancement, for all of whom clinical, imaging, and follow-up data were available. For 10 patients who had undergone meningeal biopsy, slide material was also reviewed.
The 15 men and 11 women ranged from 24 to 76 years of age. All 26 patients had postural headaches; in 22 patients, the headaches were completely alleviated by recumbency. Nausea or emesis, neck pain, horizontal diplopia, changes in hearing, photophobia, upper limb pains or paresthesias, visual blurring, or dysgeusia was noted in some of the patients. Cardinal MRI features were diffuse pachymeningeal gadolinium enhancement (100%), subdural collections of fluid (69%), and evidence of descent of the brain (62%) that sometimes resembled type I Chiari malformation. Cerebrospinal fluid (CSF) opening pressures were 40 mm or less in only 46%. In three patients, CSF pressures were consistently no less than 90 and as high as 130 mm of water. A variable pleocytosis of 5 or more cells/mm3 was noted in 15 patients (more than 40 cells/mm3 in 4 patients). A variable increase in CSF protein was noted in at least one spinal tap in 23 patients. Six patients had overdraining CSF shunts; CSF leak was documented in another 11 patients. Shunt revision or ligation and surgical correction of the leak led to a resolution of the clinical and MRI abnormalities in all cases thus treated. Improvement occurred with epidural blood patch in four patients. Three of the 12 patients treated supportively have remained symptomatic. Histologically, a thin subdural zone of fibroblasts and thin-walled vessels was noted in an amorphous matrix. Two patients with prolonged symptoms had a more pronounced proliferative reaction.
The syndrome of low-pressure headaches and pacbymeningeal gadolinium enhancement is being recognized with increasing frequency. The source of the CSF leak can be demonstrated in many patients. Meningeal abnormalities are likely attributable to decreased CSF volume and hydrostatic CSF pressure changes. The prognosis is typically good.
Patients with hereditary hemorrhagic telangiectasia (HHT) are at risk for developing cerebral vascular malformations and pulmonary arteriovenous fistulae. We assessed the risk of neurological ...dysfunction from these malformations and fistulae.
Three hundred twenty-one consecutive patients with HHT seen at a single institution over a 20-year period were studied. Any evidence of prior neurological symptoms or presence of an intracranial vascular malformation was recorded. All cases of possible cerebral arteriovenous malformation were confirmed by conventional arteriography.
Twelve patients (3.7%) had a history of cerebral vascular malformations. Ten patients had arteriovenous malformations, 1 had a dural arteriovenous fistula, and 1 had a cavernous malformation. Seven patients (2.1%) presented with intracranial hemorrhage, 2 presented with seizures alone, and 3 were discovered incidentally. The average age at the time of symptomatic intracranial hemorrhage was 25.4 years. All patients with a history of intracranial hemorrhage were classified as Rankin grade I or II at a mean follow-up interval of 6.0 years. A history of cerebral infarction or transient ischemic attack was found in 29.6% of patients with HHT and a pulmonary arteriovenous fistula.
The risk of intracranial hemorrhage is low among people with HHT. Furthermore, a majority of these patients have a good functional outcome after hemorrhage. The data do not suggest a compelling indication for routine screening of patients with HHT for asymptomatic cerebral vascular malformations. By comparison, pulmonary arteriovenous fistulae are a much more frequent cause of neurological symptoms in this population.
In primary murine hippocampal neurons we investigated the regulation of EAAT3-mediated glutamate transport by the
Clostridium botulinum
C3 transferase C3bot and a 26mer peptide derived from full ...length protein. Incubation with either enzyme-competent C3bot or enzyme-deficient C3bot
156–181
peptide resulted in the upregulation of glutamate uptake by up to 22% compared to untreated cells. A similar enhancement of glutamate transport was also achieved by the classical phorbol-ester-mediated activation of protein kinase C subtypes. Yet comparable, effects elicited by C3 preparations seemed not to rely on PKCα, γ, ε, or ζ activation. Blocking of tyrosine phosphorylation by tyrosine kinase inhibitors prevented the observed effect mediated by C3bot and C3bot 26mer. By using biochemical and molecular biological assays we could rule out that the observed C3bot and C3bot 26mer-mediated effects solely resulted from enhanced transporter expression or translocation to the neuronal surface but was rather mediated by transporter phosphorylation at tyrosine residues that was found to be significantly enhanced following incubation with either full length protein or the 26mer C3 peptide.
Size of an unruptured intracranial aneurysm (UIA) may be an important risk factor for rupture. Accordingly, serial noninvasive imaging is commonly used to assess untreated UIA for enlargement. Few ...data exist regarding the frequency and predictors of enlargement. We obtained this information from a group of patients followed with serial MR angiography (MRA).
We retrospectively identified 165 patients with 191 UIA followed with serial MRA. Fusiform aneurysms, UIA <2 mm, and UIA that were surgically or endovascularly treated before the first MRA were excluded. MRA was performed using 1.5-T and 3-T MRI. Maximal diameter was determined on MRA source images. Multivariate regression analysis was used to determine independent risk factors for growth.
Twenty aneurysms (10%) grew over a median follow-up period of 47 months. Frequency of enlargement was 6.9%, 25%, and 83% for aneurysms <8 mm, 8 to 12 mm, and >or=13 mm, respectively (P<0.001 for trend). Of the variables we evaluated, original aneurysm diameter (OR, 1.28 per mm; 95% CI, 1.07 to 1.58) was the only independent predictor of enlargement. Aneurysms >or=8 mm in diameter were at highest risk for enlargement (OR, 7.25; 95% CI, 1.96 to 27.1). There was a trend toward increased risk of enlargement in patients with multiple aneurysms (OR, 2.50; 95% CI, 0.86 to 7.53).
Over a median follow-up period of 47 months, 10% of UIA enlarged. Larger aneurysms had a significantly increased risk of enlargement. The likelihood of enlargement was highest in aneurysms with diameters >or=8 mm. However, a clinically significant proportion of small aneurysms grow, and this growth can be detected by serial MRA.
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