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1.
Neurological complications are common in sickle cell disease (SCD). However, it is often difficult to relate the clinical presentation to conventional neuroimaging, because subclinical infarction is common and stroke has been described in the absence of large-vessel disease. We studied 48 patients with SCD aged 4-34 (median 13) years with T2-weighted, diffusion and perfusion magnetic resonance imaging (MRI) and with MR angiography. Forty-four underwent transcranial Doppler (TCD). Abnormalities on perfusion imaging were seen in 25 cases, 24 of whom had been symptomatic. The remaining patient had evidence of executive dysfunction and reduced perfusion in the frontal lobes. The perfusion abnormality was larger than the area of infarction in 9 patients and was seen in an arterial distribution with no infarction in a further 9. In 3 patients with transient ischemic attacks, perfusion abnormalities were demonstrated in the absence of any other neuroimaging abnormalities, and perfusion changes were seen in 3 others despite normal MR angiography and TCD. Perfusion abnormalities are associated with neurological symptoms in patients with SCD, whether or not MRI, MR angiography, and TCD are abnormal. It is likely that this technique will guide management in individual patients.  相似文献   

2.
We compared magnetic resonance imaging (MRI), magnetic resonance angiography, and transcranial Doppler ultrasonography as predictors of specific neurocognitive functions in children with sickle cell disease. Participants were 27 children with sickle cell anemia (hemoglobin SS) who were participants in the Stroke Prevention Trial in Sickle Cell Anemia (STOP) and had no documented history of stroke. Children's MRIs were classified as normal or silent infarct, and their magnetic resonance angiograms were classified as normal or abnormal. The highest time-averaged mean flow velocity on transcranial Doppler ultrasonographic examination of the major cerebral arteries was analyzed. Age and hematocrit also were analyzed as predictor variables. The battery of neurocognitive tests included measures of intellectual functioning, academic achievement, attention, memory, visual-motor integration, and executive functions. MRI, magnetic resonance angiography, transcranial Doppler ultrasonography, age, and hematocrit were analyzed as predictors of participants' performance on the various measures of neurocognitive functioning. Age and hematocrit were robust predictors of a number of global and specific neurocognitive functions. When age and hematocrit were controlled, transcranial Doppler ultrasonography was a significantly unique predictor of verbal memory. We found an association between low hemoglobin and neurocognitive impairment. We also found that abnormalities on transcranial Doppler ultrasonography can herald subtle neurocognitive deficits. (J Child Neurol 2006;21:37-44).  相似文献   

3.
Twenty-one children and young adults with sickle/beta-thalassemia without overt stroke were examined with magnetic resonance imaging and angiography (MRA), transcranial Doppler (TCD), visual (VEP) and median nerve somatosensory (SEP)-evoked potential recordings, and neuropsychological testing (Wechsler Intelligence Scale [WISC-III]). Eight (38%) had silent infarction in the parietooccipital cortex, deep white matter, or basal ganglia, including two of three with previous seizures. Of 17 undergoing TCD, none had maximum middle cerebral artery (MCA) velocities greater than 126cm/sec, but 9 were abnormal, with low velocities and difficulty in tracking the MCA and/or asymmetry. Three patients had abnormal MRA, one of whom also had silent infarction. One patient had pathological VEP recordings, whereas all SEP recordings were normal. WISC-III was performed in all 11 children, 4 with silent infarction: all but 1 had IQ scores greater than 85 (mean, 97.7; standard deviation, 14.2). We conclude that Greek children and young adults with Sbeta-thalassemia and no history of clinical stroke have TCD abnormalities and silent infarction similar to those reported in children and adolescents with sickle cell anemia, but cognitive function is not necessarily compromised. International collaboration is needed to establish the risk factors for central nervous system sequelae in patients with sickle cell disease, including Sbeta-thalassemia, leading to evidence-based prevention.  相似文献   

4.
A systematic review was performed to assess whether cerebral blood flow with different imaging modalities could identify brain abnormalities in children with sickle cell disease where structural magnetic resonance imaging and transcranial Doppler velocity appeared normal. A total of 11 studies were identified which reported cerebral blood flow abnormalities alongside structural magnetic resonance imaging or transcranial Doppler velocity abnormalities in patients with sickle cell disease. Potential for bias was assessed with the quality assessment of diagnostic accuracy studies scale in addition to treatment bias. Subjects of each study were categorized into patients with and without stroke. The prevalence of abnormalities for each modality was then separately calculated in each group. The included studies had mostly moderate degrees of bias. The prevalence of blood flow abnormalities compared with structural magnetic resonance imaging abnormalities was equal to or lower in patients with stroke and equal to or greater in patients without stroke. Blood flow abnormalities were more prevalent than transcranial Doppler abnormalities in four studies of patients without stroke and in one study of patients with stroke. The studies suggest that the assessment of cerebral blood flow in sickle cell disease can be of potential value in addressing brain abnormalities at the tissue level; however, further studies are warranted.  相似文献   

5.
BACKGROUND AND PURPOSE: Cerebral infarction in sickle cell disease is associated with arterial narrowing or occlusions of intracranial arteries. Primary stroke prevention would be feasible if a noninvasive screening test could be developed to detect intracranial disease in patients before symptoms develop. METHODS: To determine the sensitivity and specificity of transcranial Doppler in detecting significant (greater than or equal to 50% lumen diameter reduction) intracranial arterial lesions, we compared transcranial Doppler and cerebral angiography in a primarily young, symptomatic group of 33 patients (18 males and 15 females) with sickle cell disease. RESULTS: From a total of 34 examinations, transcranial Doppler detected significant abnormalities in 26 of 29 (sensitivity 90%, specificity 100%). Five were normal by both techniques. The transorbital examination detected abnormalities in two patients whose studies were otherwise unremarkable. CONCLUSIONS: Transcranial Doppler is sensitive and specific for the detection of arterial vasculopathy of sickle cell disease. Screening should include a transorbital examination of the distal internal carotid artery as well as examination using the transtemporal approach.  相似文献   

6.
After obtaining familial informed consent, between January 1996 and July 1997, 173 children (5 to 15 years old) with sickle cell disease were enrolled in a prospective multicenter study using blood screening, transcranial Doppler ultrasonography (n = 143), cerebral magnetic resonance imaging (n = 144), and neuropsychologic performance evaluation (n = 156) (Wechsler Intelligence tests WISC-III, WIPPSI-R), which were also performed in 76 sibling controls (5 to 15 years old). Among the 173 patients with sickle cell disease (155 homozygous for hemoglobin SS, 8 sickle cell beta0 thalassemia, 3 sickle cell beta+ thalassemia, 7 sickle cell hemoglobin C disease SC), 12 (6.9%) had a history of overt stroke, and the incidence of abnormal transcranial Doppler ultrasonography (defined as mean middle cerebral artery velocity > 200 cm/sec or absent) was 8.4% in the overall study population and 9.6% in patients with homozygous sickle cell anemia The silent stroke rate was 15%. Significantly impaired cognitive functioning was observed in sickle cell disease patients with a history of stroke (Performance IQ and Full Scale IQ), but also in patients with silent strokes (Similarities, Vocabulary, and Verbal Comprehension). However, infarcts on magnetic resonance imaging were not the only factors of cognitive deficit: Verbal IQ, Performance IQ, and Full Scale IQ were strongly impaired in patients with severe chronic anemia (hematocrit < or = 20%) and in those with thrombocytosis (platelets > 500 x 10(9)/L). Multivariate logistic regression analysis showed that abnormal magnetic resonance imaging (odds ratio [OR] = 2.76) (P = .047), hematocrit < or =20% (OR = 5.85) (P = .005), and platelets > 500 x 10(9)/L (OR = 3.99) (P = .004) were independent factors of cognitive deficiency (Full Scale IQ < 75) in sickle cell disease patients. The unfavorable effect of low hematocrit has already been suggested, but this is the first report concerning an effect of thrombocytosis and showing that silent stroke alone is not a factor of cognitive deficit when not associated with low hematocrit or thrombocytosis. The effect of hydroxyurea, which is known to increase hematocrit and decrease platelet count, on cognitive functioning of sickle cell patients should be evaluated prospectively.  相似文献   

7.
Summary: Purpose and Methods: The aim of this study was to analyze clinical, radiologic, and histopathologic findings in 60 consecutive patients with medically intractable extratemporal epilepsy who were operated on between November 1987 and May 1993.
Results: Histologically, there were distinct structural abnormalities in 50 (83%) of the surgical specimens. Signal abnormalities on magnetic resonance imaging (MRI) were present in all patients with neoplastic lesions (n = 17) and in 94% of patients with nonneoplastic focal lesions (n = 32). Overall, structural abnormalities were detected by MRI in 47 (96%) of 49 patients with focal lesions. During a mean follow-up of 4 years, 30 (54%) patients remained completely seizure free, 11 (20%) had ≤2 seizures per year, seven (12%) showed a seizure reduction of ≥75%, and eight (14%) had <75% reduction in seizure frequency. The fraction of seizure-free patients was 12 (80%) of 15 in patients with neoplastic lesions, 16 (52%) of 31 in patients with nonneoplastic focal lesions, and two (20%) of 10 for those without histopathologic abnormalities. The differences in seizure outcome between patients with and without focal lesions were statistically significant (p < 0.05), if seizure-free outcome was compared with persistent seizures.
Conclusions: Focal lesions and particularly neoplasms are associated with improved postoperative seizure control compared with patients without histopathologic abnormalities. We advise caution in considering surgery to treat extratemporal epilepsy in patients who have normal MRI scans, because the outcome with the approach described in this study is poor in such cases.  相似文献   

8.
In the initial assessment of children with new-onset seizures, the suggestion that electroencephalography (EEG) should be standard and that magnetic resonance imaging (MRI) should be optional has been questioned. The purposes of this study were to (1) describe the frequency of EEG and MRI abnormalities and (2) explore relationships between MRI and EEG findings to determine their relevance in the assessment of children with new-onset seizures who are otherwise developing normally. As part of an ongoing, prospective study of children with new-onset seizures, we studied 181 children (90 girls and 91 boys). Children were entered into the study within 3 months of their first-recognized seizure. The association between EEG and MRI abnormalities was explored using a chi-square test. Abnormal MRI findings were found in 32.6% (n = 59) of the sample. The EEG and MRI results agreed with respect to classification into normal or abnormal in 37% (n = 67). Of the 50 children with a normal EEG, however, 21 (42%) were found to have an abnormal MRI. We found an unexpectedly high frequency of imaging abnormalities in our sample of otherwise normal children, although the significance of these findings is not clear. Follow-up of these patients will help us interpret the importance of the abnormalities. Despite our relatively small sample, however, our findings indicate that a normal EEG does not reliably predict a normal MRI in children with first seizures.  相似文献   

9.
Purpose: To define seizure recurrence rates in normal children who had had a single seizure and to define electroencephalography (EEG) or magnetic resonance imaging (MRI) utility in predicting seizure recurrence. Methods: We studied 150 children (6 to 14 years) with a first afebrile, unprovoked seizure. Inclusion criteria were: Normal physical and neurological examination, undergone EEG and MRI studies of the brain, and followed for at least 27 months. These children participated in an ongoing prospective study of new onset seizures in childhood. Results: The seizure recurrence rate was 66.4%. An abnormal EEG had no association with seizure recurrence at 9, 18, or 27 months (p = 0.1806, p = 0.2792, and p = 0.2379, respectively). A “significant” MRI abnormality, which occurred in 16.0% of patients, was associated with an increased seizure recurrence risk at 9 months (p = 0.0389) but not at 18 or 27 months. Discussion: EEG findings poorly predict recurrence after a single seizure. The high rate of MRI abnormalities suggests that MRI may need consideration as a routine test to evaluate epilepsy in normal children.  相似文献   

10.
BACKGROUND: Cortical areas showing abnormal glucose metabolism and [(11)C]flumazenil (FMZ) binding are commonly seen on PET scans of patients with intractable partial epilepsy, but it is unclear whether these must be totally resected to achieve seizure control. OBJECTIVE: To analyze whether the extent of cortex showing 2-deoxy-2-[(18)F]fluoro-D-glucose (FDG) or FMZ PET abnormalities correlates with the outcome of resective epilepsy surgery. METHODS: Cortical FDG and FMZ PET abnormalities in 15 young patients (mean age, 12.2 +/- 7.0 years) with intractable partial epilepsy of neocortical origin were marked as regions with abnormal asymmetry using an objective semiautomated software package. These marked regions were then projected and measured on the brain surface reconstructed from the coregistered high-resolution MRI. Following cortical resection, the size of nonresected cortex with preoperative PET abnormalities was also measured (calculated separately for marked areas in the lobe of seizure onset as defined by long-term video EEG monitoring, and in remote cortical areas). Extent of preoperative PET abnormalities and postoperative nonresected cortex abnormalities on PET were correlated with outcome scores. RESULTS: Large preoperative FMZ PET abnormalities were associated with poor outcome (r = 0.57; p = 0.025). Larger areas of nonresected cortex with preoperative FMZ PET abnormalities in the lobe of seizure onset were also associated with worse outcome in the whole group (r = 0.66; p = 0.007) as well as in patients with extratemporal resection (r = 0.73; p = 0.007), and in those with no lesion on MRI (r = 0.60; p = 0.049). Patients with seizure-free outcome had significantly smaller nonresected cortex with preoperative FMZ PET abnormalities than those who continued to have seizures (p = 0.022). No significant correlations between nonresected FDG PET abnormalities and surgical outcome were found. CONCLUSIONS: Extensive cortical abnormalities on FMZ PET predict poor outcome in neocortical epilepsy surgery. Resection of FMZ abnormalities in the lobe of seizure onset is associated with excellent outcome even in the absence of a structural lesion. In contrast, although FDG PET abnormalities regionalized the epileptogenic area, their size was not related to the extent of epileptogenic tissue to be removed.  相似文献   

11.
PURPOSE: Previous studies using diffusion MRI in patients with temporal lobe epilepsy have shown abnormal water diffusion in the hippocampus. Because thalamus and lentiform nuclei are considered important for the regulation of cortical excitability and seizure propagation, we analyzed diffusion tensor imaging (DTI) abnormalities in these subcortical structures and in hippocampus of children with partial epilepsy with and without secondary generalization. METHODS: Fourteen children with partial epilepsy involving the temporal lobe underwent MRI including a DTI sequence. Fractional anisotropy (FA) and apparent diffusion coefficient (ADC) values were obtained in the hippocampus, thalamus, and lentiform nucleus, and compared with DTI data of 14 control children with no epilepsy, as well as glucose positron emission tomography (PET) findings. RESULTS: Decreased FA (p < 0.001) and increased ADC (p = 0.003) values were found in the hippocampi ipsilateral to the seizure focus. Significant FA decreases (p = 0.002) also were seen in the contralateral hippocampi, despite unilateral seizure onset and excellent surgical outcome in patients who underwent surgery. ADC values showed a trend for increase in the thalami ipsilateral to the epileptic focus in the seven children with secondarily generalized seizures (p = 0.09). No group differences of ADC or FA were found in the lentiform nuclei. The DTI variables did not correlate with regional glucose metabolism in any of the structures analyzed. CONCLUSIONS: Increased ADC values in hippocampus can assist in lateralizing the seizure focus, but decreased FA in the contralateral hippocampus suggests that it too may be dysfunctional despite unilateral seizure onset. Less-robust thalamic abnormalities of water diffusion in patients with secondarily generalized seizures suggest secondary involvement of the thalamus, perhaps due to recruitment of this structure into the epileptic network; however, this must be confirmed in a larger population. DTI appears to be a sensitive method for detection abnormalities in children with partial epilepsy, even in structures without apparent changes on conventional MRI.  相似文献   

12.
OBJECTIVE: Whether magnetic resonance imaging (MRI) is informative in febrile seizures (FS) is unknown. We undertook a study to determine the frequency of MRI-detected brain abnormalities and to evaluate their association with FS type and with specific features of complex FS. METHODS: A prospective cohort study, from 1999 to 2004, included children with first FS from one Pediatric Emergency Department. MRI of the brain was performed within 1 week of the seizure. FS type was categorized by experts blind to the prior clinical history and MRI results. MRI examinations were read blind to the child's clinical history and FS type, and interviewers were blind to MRI results. RESULTS: In 159 children with a first FS, imaging abnormalities occurred in 12.6% (N = 20). Eight of the 54 with complex FS had imaging abnormalities compared to 12 of the 105 with simple FS (n.s.). Compared to children with simple FS, children with both focal and prolonged FS (N = 14) were more likely to have imaging abnormality (OR = 4.3, 95% CI = 1.2-15.0), even after adjustment for abnormal neurological examination. Imaging abnormalities included those known to be associated with seizures (e.g., focal cortical dysplasia) and those not typically associated with seizures (e.g., subcortical focal hyperintensities > or = 5 mm). DISCUSSION: Our data suggest that brain abnormalities may lower seizure threshold in febrile children, predisposing to the development of FS. Clinical management was unaffected and therefore these data do not alter the recommendation that MRI is unnecessary in children with FS, without some other neurological indication.  相似文献   

13.
目的探讨肢体抖动性短暂性脑缺血发作的临床表现及诊断,提高临床医师对这一症候的认识。方法采用动态脑电图、经颅多普勒超声、颈部血管超声、头部核磁共振、核磁共振血管成像、全脑血管造影等辅助检查,对4例肢体抖动性短暂性脑缺血发作患者进行临床表现、辅助检查及影像学资料分析。结果4例患者均表现为肢体发作性短暂不能控制的抖动,其发作均有明显诱因,其中2例曾被误诊为癫,给予抗癫治疗无效。全部患者均有抖动肢体对侧至少1条以上颅内和(或)颅外动脉严重狭窄或闭塞,头部核磁共振3例显示抖动肢体对侧分水岭脑梗死,发作期脑电图检查未见癫波释放,给予抗血小板聚集、扩容或者血管内支架治疗后,症状均消失。结论肢体抖动性短暂性脑缺血发作常表现为发作性、无意识的肢体抖动,和局灶性运动性癫发作相似,易误诊为局灶性癫,通常提示大血管严重的狭窄或闭塞,低灌注是其最可能的发病机制,通过脑电图及全脑血管造影等检查可以明确诊断,防止漏诊及误诊。  相似文献   

14.
OBJECTIVE: To determine the incidence and type of neuroimaging abnormalities in children presenting with a first seizure. METHODS: In a prospective observational study, 411 children with a first afebrile seizure were seen between 1983 and 1992. Imaging studies were performed in 218 (53%). For this analysis we examined the most sensitive neuroimaging study performed which included 159 computed tomography scans and 59 magnetic resonance imagings (MRI). RESULTS: Four children were found to have lesions requiring intervention (brain tumor in two, neurocysticercosis in two). The remaining 407 were enrolled in a follow-up study of children with a first unprovoked seizure. After a mean follow-up of >10 years, none have developed clinical evidence of a tumor. In these 411 children, 45 (21%) of 218 imaging studies were abnormal. The most common abnormalities were focal encephalomalacia (n=16) and cerebral dysgenesis (n=11). Although children with partial seizures were more likely to be imaged (64%) than children with generalized seizures (43%) (P<0.001), the fraction of abnormal imaging studies was similar in both groups. Six children with a normal neurological examination who were initially classified as cryptogenic were subsequently found to have errors of cerebral migration on MRI. The incidence of lesions requiring acute intervention in children presenting with a first seizure is low. A significant proportion will have neuroimaging abnormalities particularly on MRI. CONCLUSIONS: Neuroimaging should be considered in any child with a first seizure who does not have an idiopathic form of epilepsy.  相似文献   

15.
TCD, MRA and MRI in acute cerebral ischemia   总被引:13,自引:0,他引:13  
Objectives - The aim of this study was to determine accuracy of transcranial Doppler ultrasound (TCD) and compare efficacy of three non-invasive tests [TCD, magnetic resonance angiography (MRA), and magnetic resonance imaging (MRI)] in patients with acute cerebral ischemia. Material and methods - This prospective study involved 30 patients. MRI, MRA, and TCD were performed within 24 h after onset of ictus. The 2nd MRI was repeated at 48–72 h and was used as the standard for the evaluation of sensitivity and specificity of MRA, TCD, and initial MRI. Results - TCD showed a sensitivity of 96% and a specificity of 33% for recognizing abnormal cerebral blood flow velocities. MRA showed a sensitivity of 46% and a specificity of 75% for assessing intracranial vascular anatomy, while initial MRI revealed a sensitivity of 84% and a specificity of 100% for evaluation of ischemic parenchymal changes. Conclusion - Our results revealed that TCD is an accurate indicator of blood flow status and correlated well with MRI, MRA abnormalities in acute stroke.  相似文献   

16.
EEG Abnormalities in Children with a First Unprovoked Seizure   总被引:4,自引:4,他引:0  
Summary: We examined EEG findings from an ongoing study of 347 children with a first unprovoked seizure. EEGs were available in 321 (93%), and 135 (42%) had an abnormal EEG. EEG abnormalities included focal spikes (n = 77), generalized spike and wave discharges (n = 28), slowing (n = 43), and nonspecific abnormalities (n = 7). Abnormal EEGs were more common in children with remote symptomatic seizures (60%) than in those with idiopathic seizures (38%) (p < 0.003), more common in partial seizures (56%) than in generalized seizures (35%) (p < 0.001), and more common in children age >3 years (52%) than in younger children (12%) (p < 0.001). Records including both awake and sleep tracings were available in 148 (46%) cases. For 122 (38%) only awake tracings and for 51 (16%) only sleep tracings were available. Fifty-nine (40%) of the 148 patients with both an awake and asleep tracing had abnormal EEGs. Of 50 such EEGs with epileptiform abnormalities, 15 (30%) demonstrated the abnormality either only while awake (n = 8) or only while asleep (n = 7). Of 17 patients with EEG slowing, 8 showed slowing only in the awake tracing and 9 showed slowing in both the awake and asleep tracing. Children with even a single unprovoked seizure have a high incidence of EEG abnormalities. Obtaining a combined awake and sleep EEG significantly increases the yield of EEG abnormalities. In children with an idiopathic first seizure, EEG abnormalities are associated with an increased risk of seizure recurrence.  相似文献   

17.
Brain infarction is a well-known but poorly understood complication of sickle cell disease. Seventy-three sickle cell disease patients underwent neurological examinations and high-field, heavily T2-weighted axial cranial magnetic resonance image (MRI) scanning. Eighteen of the 73 had a history of stroke, defined as an acute, focal neurological sign lasting longer than 1 hour; in the event of a convulsive onset, an MRI abnormality as a correlate was necessary. Sixteen of the 18 stroke patients demonstrated focal MRI abnormalities consistent with arterial borderzone infarctions. Fifty-five of the 73 patients had no history of stroke. Six of the 55 (11%) had focal MRI abnormalities suggesting previous subclinical stroke. Five of these lesions were in borderzone regions. The distinguishing feature in 21 of the 22 patients with MRI abnormalities was the predilection for lesions in the high cortical convexity, in the general regions of arterial borderzones between the major cerebral arteries and adjacent deep white matter. The pattern of MRI lesions suggests two pathogenetic mechanisms: (1) proximal large-vessel disease with inadequate cerebral perfusion (distal field insufficiency syndrome) and (2) distal small-vessel disease (sludging syndrome).  相似文献   

18.
OBJECTIVE: To identify clinical features related to seizure frequency after epilepsy surgery in patients with recurrent seizures. BACKGROUND: No studies have examined the differences between patients who have rare seizures and patients who experience frequent seizures after epilepsy surgery. Since seizure frequency correlates with morbidity and quality of life, it is desirable to know which preoperative clinical features predict postoperative seizure frequency. METHODS: Patients with recurrent seizures were placed in two categories: rare postoperative seizures (< or =2 per year) and frequent postoperative seizures (> or =12 per year) using seizure frequency in the second postoperative year. Variables included preoperative seizure frequency, age of first risk, age at first seizure, epilepsy duration, age at surgery, history of febrile convulsions, tonic-clonic seizures, status epilepticus, or family history, IQ, magnetic resonance imaging (MRI), and positron emission tomography (PET). Variables were analyzed using non-parametric tests to assess relationship to postoperative seizure frequency. RESULTS: Of 475 patients who had epilepsy surgery, 111 had rare or frequent seizures in the second postoperative year. After anterior temporal lobectomy (ATL), age of first risk< or =5 years and presence of mesial temporal sclerosis on MRI were associated with rare seizures (66% of patients), whereas lack of these risk factors was associated with frequent seizures (75% of patients) (p<0.03). For non-ATL operations, preoperative seizure frequency of > or =20 seizures per month was associated with frequent postoperative seizures (p=0.03). No other variables influenced outcome. CONCLUSIONS: Some preoperative clinical features correlate with postoperative seizure frequency in patients with recurrent seizures after epilepsy surgery. This has implications for the surgical decision making process and early postoperative management.  相似文献   

19.
OBJECTIVE: To investigate the presence of hippocampal atrophy (HA) and other magnetic resonance imaging (MRI) signs of hippocampal sclerosis (HS) in asymptomatic relatives of patients with familial mesial temporal lobe epilepsy (FMTLE). METHODS: We invited first-degree, asymptomatic relatives of patients with FMTLE to participate in our MRI protocol. After obtaining informed consent, all participating individuals underwent an MRI examination. Hippocampal abnormality was determined by qualitative and volumetric analyses, using a standard protocol. RESULTS: We studied 52 asymptomatic individuals (27 men), with a mean age of 32 years (range, 7-71 years), from 11 families with FMTLE. Volumetric studies showed HA in 18 (34%) of 52 individuals: 11 had left HA and 7 had bilateral HA. In addition, careful visual analysis of T1- and T2-weighted images showed additional classic MRI signs of HS (such as abnormal T2 signal and/or abnormal internal structure) in 14 of these 18 individuals. There was no age difference between individuals with and without HA (t test, P =.80). CONCLUSIONS: Our findings indicate that MRI evidence of HS is not necessarily related to seizure severity and may occur in individuals who never had seizures. In addition, these observations strongly indicate that HS in FMTLE is not a consequence of recurrent seizures and is determined by a strong genetic predisposition. The determination of seizure severity in patients with FMTLE probably depends on the interaction of different factors, both genetic and environmental.  相似文献   

20.
BACKGROUND AND PURPOSE: Since the benefit of an endarterectomy in an asymptomatic carotid stenosis is not as clear as that in symptomatic stenosis, identifying patients with a high risk of stroke will allow the improved selection of surgical candidates. To evaluate the role of transcranial Doppler (TCD) in assessing an asymptomatic stenosis, this study examined the association of a TCD-detected hemodynamic change with a stenosis-related abnormality on magnetic resonance imaging (MRI). METHODS: Asymptomatic patients with a more than 60% internal carotid artery stenosis were enrolled in this study. The hemodynamic change in the TCD was analyzed in terms of the demographic characteristics, the stroke risk factors, and the stenosis-related MRI abnormalities that are a proven risk factor of a further stroke. RESULTS: Fifty-three patients were retrospectively recruited. While there were no differences in the demographic characteristics or frequency of the stroke risk factors, the proportion of patients with stenosis-related MRI abnormality was significantly higher in patients with a hemodynamic change on TCD (13/19 vs 6/34, P < .01; odds ratio = 10.11; 95% confidence interval = 2.73-37.4). CONCLUSIONS: The TCD-detected hemodynamic changes may provide useful information for assessing the frequency of silent stroke in patients with an asymptomatic carotid stenosis.  相似文献   

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