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1.
Functional reorganization after focal brain injury can lead to altered cerebral metabolism of glucose. Sturge-Weber syndrome (SWS) with unilateral involvement is a clinical model for evaluating the effects of early focal brain injury on brain metabolism and function. In this study, 2-deoxy-2[(18)F]fluoro-D-glucose (FDG) positron emission tomography (PET) was used to measure glucose metabolism in cortex and basal ganglia, both ipsilateral and contralateral to the angioma, in 17 children (eight males, nine females; age range 1y 8mo-10y 4mo; mean 5y 7mo [SD 2y 11mo]) with unilateral SWS and epilepsy. The PET findings were compared with those of a control group of 11 age-matched children (four males, seven females; age range 3y-10y 8mo; mean 6y [SD 2y 10mo]) with partial epilepsy but normal magnetic resonance imaging and PET scans. In the SWS group, visual and parietal cortex showed decreased glucose metabolism on the side of the angioma (p=0.001) but increased metabolism on the contralateral side (p=0.002). In particular, glucose metabolism was very high in contralateral visual cortex of childrenwith SWS, showing severe occipital hypometabolism on the side of the angioma. Eight children with visual field defect showed increased metabolism in the contralateral visual cortex (p=0.012). These findings indicate that early, severe unilateral cortical damage in SWS may induce increased glucose metabolism in the contralateral visual cortex, probably reflecting reorganization.  相似文献   

2.
目的分析并比较因脑血管畸形导致难治性癫痫从而接受癫痫外科手术的15例患者的临床及病理学特点。方法回顾性分析接受脑病灶及致痫灶切除手术的15例脑血管畸形患者的临床资料及病理资料。结果 15例患者均表现为难治性癫痫,其中包括8例海绵状血管瘤,7例Sturge-Weber综合征(SWS)。男性8例,女性7例,发病年龄1月龄~29岁,病程2~24年。手术年龄2~31岁。2例行单脑叶切除,12例行多脑叶切除,1例行功能性大脑半球切除。脑标本镜下可见海绵状血管瘤并局灶性皮质发育不良(FCD)Ⅲc型;软脑膜血管瘤病、灰质中弥漫钙化,脑实质内小血管壁钙化,并伴发局灶性皮质发育不良(FCD)Ⅲc型。术后随访0.5~8年,EngelⅠ级11例,EngelⅡ级2例,EngelⅢ级1例,EngelⅣ级1例。结论脑海绵状血管瘤患者接受癫痫外科手术后随访效果良好;SWS患者颅内病变程度可能与遗传学相关,SWSⅠ型患儿应尽早手术,术后效果良好;SWSⅢ型患者接受血管瘤病病灶及致痫灶切除术后,随访效果良好。  相似文献   

3.
Sturge-Weber syndrome: indications and results of surgery in 20 patients   总被引:6,自引:0,他引:6  
OBJECTIVE: To discuss the indications and timing for resective surgery in patients with Sturge-Weber syndrome (SWS) and medication-resistant epilepsy. BACKGROUND: SWS that causes epilepsy severe enough to merit surgery is rare. Because of the variable natural history of the disease, it is difficult to establish clear-cut indications for surgery and prospective studies are not feasible. Attitudes of clinicians and surgeons remain variable. METHODS: The authors assessed the presurgical epilepsy profile, criteria for surgery, monitoring techniques, and the postoperative outcome of epilepsy in all patients with SWS consecutively admitted between 1972 and 1990 to two referral centers (Paris and Montreal) and underwent surgery for intractable seizures. RESULTS: All 20 patients had a minimal postoperative follow-up of 4 years and all but one are still followed by one of the authors. One patient had a callosotomy, five underwent hemispherectomy, and 14 had cortical resection. Despite variability in the age at onset of seizures (range: 2 months to 12 years), age at operation (range: 8 months to 34 years) and surgical methods, almost all patients benefited from surgery. Visually guided complete resection of the pial angioma and underlying cortex, whenever possible, seemed sufficient; results were no better with intraoperative corticography. In children with previous hemiparesis, hemispherectomy proved particularly effective: all five became seizure free. None of the patients showed any aggravation of cognitive impairment following surgery; none of those who were operated on early presented with severe mental retardation, and 13 of 20 became seizure free. CONCLUSION: Although the natural history of SWS is imperfectly known, increasing duration of seizures and of postictal deficits, increase in atrophy or of calcified lesions or both, are indicative of its progressive nature. Despite the expected heterogeneity that renders formal comparison of the various approaches difficult, the current study provides new evidence to support early surgery in patients with SWS and drug-resistant epilepsy. The authors' results suggest that lesionectomy is a good approach, provided that the pial angioma is unilateral and the resection can be complete.  相似文献   

4.
Sturge-Weber syndrome (SWS) involves vascular malformations of the skin (facial port-wine stain), eye (glaucoma) and the brain (leptomeningeal angioma). Children born with a port-wine stain on the upper part of the face are also at risk for brain involvement. These infants and young children often develop seizures and other neurologic impairments. Progression in neurologic deficits does occur in some patients, but this is quite variable. A diagnosis of brain involvement is made with head computed tomography and contrast-enhanced MRI, but the sensitivity of standard imaging in young asymptomatic infants is low. Seizures occur in more than 75% of affected individuals. Clinical course and functional imaging suggest a role for both cerebral perfusion impairments and seizures in the development of neurologic deficits. Several controversies exist in the management of seizures and other neurologic impairments in SWS. Continued efforts are needed to develop a multicentered network for SWS clinical trials. Future research should be focused on this goal and on studies to improve our understanding of the cause(s) and molecular neuropathology of SWS.  相似文献   

5.
We investigated seizure, intelligence quotient (IQ), and neurological outcomes including the process of motor function recovery after functional right hemispherectomy in 3 children with Rasmussen's encephalitis (RE). Before the procedure, they were unable to walk, nor sit without support due to progressive worsening of left hemiplegia and relentless epilepsia partialis continua (EPC) of the left extremities, which were refractory to antiepileptic drug and immunological treatment. After functional right hemispherectomy, EPC completely disappeared, although complete left hemiplegia was sustained. However, they recovered up to being able to walk independently with assistance devices, and to have an ordinary life with family support within 1.5 to 5 months through rehabilitation. At the same time, the interictal EEG improved on the unaffected side of hemisphere, exhibiting a posterior alpha rhythm. Their IQ also improved, and they were able to attend school. Early functional hemispherectomy should be considered before patients with RE are left in a serious condition due to progressive worsening of hemiplegia and seizures refractory to the available treatment.  相似文献   

6.
OBJECTIVE: To utilize diffusion tensor tractography and evaluate the integrity of the corticospinal tract in children with unilateral Sturge-Weber syndrome (SWS). METHODS: Sixteen children (age: 1.5-12.3 years) with SWS involving one hemisphere and varying degrees of motor deficit, underwent magnetic resonance imaging (MRI) as part of a prospective clinical research study. Diffusion tensor imaging (DTI) was obtained and fiber tracking of the corticospinal tract was performed yielding average FA and ADC values along the pathway. These values were compared between the two hemispheres (affected vs. unaffected) and also correlated with the degree of motor deficits, after correction for age. RESULTS: Corticospinal tract FA values on the side of the affected hemisphere were lower (p=0.008) and ADC values were higher (p=0.011) compared to the normal side. Furthermore, FA and ADC values on the side of the angioma did not show the normal age-related variations, which the contralateral corticospinal pathway values did demonstrate. Although none of the patients had severe hemiparesis, those with moderate motor deficit had increased ADC values, as compared to those with mild (p=0.009) or no motor deficit (p=0.045). CONCLUSION: MRI with DTI shows abnormalities of the corticospinal tract in children with SWS even before severe motor impairment develops. Thus, DTI can be a clinically useful method to evaluate the integrity of the corticospinal tract in young children who are at risk for progressive motor dysfunction.  相似文献   

7.
A case of left facial port-wine stain with right hemiparesis is reported. Radiologically, two arteriovenous malformations (AVM) and diffuse venous abnormalities were observed in the left hemisphere. AVM were seen in the basal ganglia. One was fed by a perforating artery from the MCA and drained into the superior petrosal sinus, and the other was fed by a perforating artery from the basilar artery and drained through the vein of Rosenthal into an extremely dilated vein of Galen. Venous abnormalities were obstruction and a decrease in the superficial cortical veins, as well as the formation of collateral veins in both the cortex and white matter. This case resembles Sturge-Weber syndrome (SWS), but there were no signs of leptomeningeal angiomatosis or venous angioma. We have been unable to find any case reports on SWS with AVM or AVM with diffuse venous abnormalities. We discuss the differences between our case and SWS.  相似文献   

8.
OBJECTIVE: To develop hypotheses regarding the relationship between Sturge-Weber syndrome (SWS) and other abnormalities in a subset of patients. DESIGN: We retrospectively reviewed medical records in a group of 28 patients with SWS, noting the main features of SWS and accompanying unexpected abnormalities. We also conducted a literature review of abnormalities associated with SWS. RESULTS: Twenty-eight medical records of patients with SWS were reviewed. Of this number, we found 8 (29%, 2 female) patients who manifested other abnormalities. Our review of the literature uncovered 15 additional cases with associated abnormalities. CONCLUSIONS: We hypothesize that the abnormalities associated with SWS suggest testable insights regarding pathogenesis and that chromosome 17p1-p13 may be a candidate region for genes involved with SWS. We also propose that some patients with SWS may have disorders of cholesterol biosynthesis or carbohydrate glycosylation.  相似文献   

9.
The author discusses the problem of surgical treatment of intracranial angiomas situated in basal ganglia and diencephalon. Vascular malformations in this area were found in 7.5% of cases in a group of 80 patients with intracranial angiomas. In all 6 cases the onset of the disease was sudden with meningocerebral haemorrhage, prolonged coma and hemiplegia. The malformations had usually the features of arteriovenous angioma. The afferent vessels come usually from the medial short and long vessels branching off from the anterior and middle cerebral arteries, the choroid arteries and the posterior communicating artery. The author isolated two types of malformations differing in their situation, shape and range of vascularization. In the "subventricular" type situated within the nucleus caudatus and lenticularis, internal capsule and thalamus the malformation can be exposed well from the approach through the lateral ventricle. The angiomas situated nearer to the base of hemisphere (the parabasal type), in the diencephalon, in the area of the olfactory triangle, substantia perforata anterior and even crus cerebri can be exposed best using the subfrontal approach. The author believes that in many cases of these malformations regarded usually as inoperable, radical operation can be done with selective removal of angioma by means of microsurgery. This procedure was applied in 2 cases (Fig. 2 and 3).  相似文献   

10.
Chronic benzodiazepine usage and withdrawal in insomnia patients   总被引:5,自引:0,他引:5  
We studied the sleep of patients with insomnia during continuous and very long-term use of benzodiazepines (BZDs), and after withdrawal. A group of 25 patients (mean age 44.3+/-11.8 years) with persistent insomnia, who had been taking BZDs nightly for 6.8+/-5.4 years was selected. The control group was comprised of 18 age-matched healthy individuals. Sleep stage parameters were analyzed during Night 1 (while taking BZDs), Night 2 (first night after completing BZD withdrawal), and Night 3 (15 days after gradual BZD withdrawal). Sleep data for control subjects was monitored in parallel. Sleep EEGs of the patients were analyzed using Period Amplitude Analysis (PAA), during Nights 1 and 3 only. During BZD use, a significant reduction of Total Sleep Time (TST) and increased sleep latency were found in the insomniac group when compared to controls. We found an increase in stage 2 non-REM (NREM) sleep, and a reduction in Slow Wave Sleep (SWS) when comparing to night 3 (after withdrawal). Sleep EEGs analysis showed an increase in sigma band and decrease in delta count in stages 2, 3, 4 NREM and REM sleep in the BZD group when comparing to night 3 (after withdrawal). During the BZD withdrawal period, six out of nine subjects taking lorazepam failed withdrawal. In the remaining 19 subjects, gradual withdrawal of BZDs was associated with immediate worsening of nocturnal sleep, as indicated by sleep parameters. However, 15 days after withdrawal (Night 3), some of the sleep structure parameters of patients were not significantly different from baseline (while taking BZDs), except for a significant increase in SWS and in delta count throughout most sleep stages, and a decrease in stage 2 NREM sleep. These values were not different from those shown by control subjects. REM sleep parameters showed no significant variation across the experimental conditions. Subjective sleep quality was significantly improved on Night 3 compared with Night 1. Conclusions: Chronic intake of BZDs may be associated with poor sleep in this population. A progressive 15-day withdrawal did not avoid an immediate worsening of sleep parameters. But at the end of the protocol, SWS, delta count, and sleep quality were improved compared to those recorded during the chronic BZD intake, despite the lack of change in sleep efficiency.  相似文献   

11.
We sought to develop a risk profile that would predict worsening consciousness from edema after hemispheric infarction. Charts were reviewed correlating initial computed tomography scan, neurologic examination, demographic features, and ischemic mechanism with worsening consciousness from massive edema after hemispheric infarction. An edema risk profile composed of two of three clinical factors (gaze preference, hemiplegia, or hemineglect) and evidence of acute cortical infarction on initial computed tomography scan highly correlated with the later development of worsening consciousness from edema. The edema risk profile correlated with worsening consciousness from edema after hemispheric infarction. The profile requires prospective verification before use for family counseling, for anticipatory care, and for randomizing patients in acute stroke trials aimed at controlling the formation and sequelae of edema after ischemic stroke.  相似文献   

12.
Giant cerebral cavernoma is a rare malformation classified as a brain occult vascular lesion. We report a case of initially misdiagnosed giant cavernous angioma revealed by a spontaneous intracerebral hemorrhage. A 40-year-old woman was admitted with right hemiplegia and altered consciousness occurring 3 days prior to presentation. A non-contrast CT scan showed a left parietal gross hematoma, and she was immediately taken to the operating room for emergency surgery. However, the procedure was interrupted by massive bleeding, and therefore a vascular lesion was suspected. Magnetic resonance images suggested a giant cavernous malformation. Afterward, she underwent total removal of this lesion. The postoperative period was uneventful, and histopathological examination confirmed the diagnosis of cavernous angioma. Thus, the differential diagnosis of gross spontaneous intracerebral hematoma should include giant cavernous angioma.  相似文献   

13.
Alkonyi B  Chugani HT  Juhász C 《Epilepsia》2011,52(7):1265-1272
Purpose: To investigate clinical correlates and longitudinal course of interictal focal cortical glucose hypermetabolism in children with Sturge‐Weber syndrome (SWS). Methods: Fluorodeoxyglucose positron emission tomography (FDG‐PET) scans of 60 children (age range 3 months to 15.2 years) with Sturge‐Weber syndrome and epilepsy were assessed prospectively and serially for focal hypo‐ or hypermetabolism. Thirty‐two patients had two or more consecutive PET scans. Age, seizure variables, and the occurrence of epilepsy surgery were compared between patients with and without focal hypermetabolism. The severity of focal hypermetabolism was also assessed and correlated with seizure variables. Key Findings: Interictal cortical glucose hypermetabolism, ipsilateral to the angioma, was seen in nine patients, with the most common location in the frontal lobe. Age was lower in patients with hypermetabolism than in those without (p = 0.022). In addition, time difference between the onset of first seizure and the first PET scan was much shorter in children with increased glucose metabolism than in those without (mean: 1.0 vs. 3.6 years; p = 0.019). Increased metabolism was transient and switched to hypometabolism in all five children where follow‐up scans were available. Focal glucose hypermetabolism occurred in 28% of children younger than the age of 2 years. Children with transient hypermetabolism had a higher rate of subsequent epilepsy surgery as compared to those without hypermetabolism (p = 0.039). Significance: Interictal glucose hypermetabolism in young children with SWS is most often seen within a short time before or after the onset of first clinical seizures, that is, the presumed period of epileptogenesis. Increased glucose metabolism detected by PET predicts future demise of the affected cortex based on a progressive loss of metabolism and may be an imaging marker of the most malignant cases of intractable epilepsy requiring surgery in SWS.  相似文献   

14.
目的探讨显微手术切除岛叶病变的方法和效果。方法回顾性分析14例岛叶病变患者的临床资料。结果胶质瘤10例,海绵状血管瘤2例,上皮样囊肿1例,结核瘤1例。肿瘤全切除10例,近全切除3例,结核瘤1例予大部切除,无手术死亡。术后无失语,1例偏瘫。结论经翼点-岛叶入路应用显微外科技术是治疗岛叶病变的有效手段。  相似文献   

15.
It is well known that sleep loss activates human and experimental epilepsy. Because sleep deficits are also common among epileptics, it is possible that sleep loss enhances seizures by worsening these spontaneous sleep abnormalities. We examined this hypothesis by documenting the effects of acute (72 h) sleep deprivation on seizures in fully kindled cats. We have reported elsewhere sustained decrements in both slow-wave sleep (SWS) and rapid-eye-movement (REM) sleep with the development of amygdala kindling in this species. In the present experiment, eight cats underwent an A1 B1 A2 paradigm in which sleep state physiology (12-h polygraphic recordings) and seizure thresholds were examined in both an experimental (sleep deprivation) and control (nonsleep deprived) sequence of conditions, with order of presentation counterbalanced. Sleep deprivation was produced by a modification of the “flower pot” procedure and involved placing the cat on a small platform over water so as to prevent REM sleep. In the control sequence, cats underwent the same procedure except the platform was large enough to allow normal SWS and REM sleep. A significant reduction in both REM and SWS sleep occurred exclusively during the experimental B1 condition and was accompanied by a significant reduction in seizure threshold for partial or generalized tonic-clonic convulsions. These findings indicated that this procedure, thought to influence only REM sleep, also disrupts SWS; further, this generalized disruption of sleep reduced kindled seizure thresholds, perhaps by compounding existing sleep deficits. Collectively, these observations again suggest that the integrity of both SWS and REM sleep may be an important consideration in the etiology of amygdala-kindled seizures.  相似文献   

16.
The progressive nature of Sturge-Weber syndrome is well known, but the mechanisms of focal cortical and subcortical degeneration in this disorder are poorly understood. In the present study, we assessed the structural and functional integrity of gray and white matter in unihemispheric Sturge-Weber syndrome using quantitative magnetic resonance imaging (MRI) volumetry and MRI-based partial volume correction of [18F]fluorodeoxyglucose positron emission tomographic (PET) images. Gray- and white-matter volumes and glucose metabolism were measured in three brain regions (parieto-occipital underneath the angioma, temporal, and frontal) in six children with Sturge-Weber syndrome (two infants, ages 6 and 9 months; four older children, ages 4 to 14 years), all with unilateral parieto-occipital leptomeningeal angiomatosis. The gray-matter volumes ipsilateral to the angioma were smaller in all children, with the posterior regions underneath the angioma the most affected. In the infants, the white-matter volumes were increased in the region of the angioma, whereas in the regions remote from the angioma in the infants and in all regions of the older children, there were large decreases in white-matter volume. The decreases of frontal and temporal white-matter volume were more pronounced than the corresponding gray-matter volume decreases. The PET studies showed severe hypometabolism in the parieto-occipitalregion underneath the angioma in all of the children. However, the two infants showed glucose hypermetabolism in the frontal and temporal cortical gray matter, whereas these regions had relatively preserved metabolism in the older patients. These results demonstrate differential involvement of gray and white matter in Sturge-Weber syndrome. Both structural and functional abnormalities extend well beyond the angioma, indicating widespread abnormalities of growth and development of the affected hemisphere. Furthermore, whereas increased white-matter volume underlying the angioma may be seen in infants, ipsilateral white-matter regions outside the angioma show volume loss both in infants and in older patients. Extensive gray- and white-matter volume loss and hypometabolism ipsilateral to the angioma likely contribute to the frequently observed progressive cognitive dysfunction in these patients, regardless of the extent of the angioma.  相似文献   

17.
We report a patient with Sturge–Weber Syndrome (SWS) who developed migraine-like headaches followed by cerebral infarction. SWS without facial nevus was diagnosed based on calcification detected by CT and pial angioma detected by enhanced MRI. His migraine-like headaches were preceded by left homonymous hemianopsia, which persisted for more than 60 min. Although homonymous hemianopsia disappeared with cessation of the headache until 13 years of age, from age 14 years onward, this homonymous hemianopsia persisted after the headaches ended. Moreover, reduced cerebral blood flow was seen in the right occipital area on SPECT. At first, his left homonymous hemianopsia persisted for several months after the headache disappeared, but it had recovered completely. However, the durations of episodes of left homonymous hemianopsia, which persisted after headache disappearance, gradually became longer. At last one year after his first admission, the visual defect had become permanent. SWS is well known to be associated with migraine attacks and hemianopsia. However, the course of our present patient, i.e. recurrent homonymous hemianopsia, associated with migraine-like headaches becoming permanent, is rare. The pathophysiological mechanism underlying this clinical course is uncertain. The efficacy of valproate and propranolol as preventive therapy has been inadequate, to date.  相似文献   

18.
Aims: The purpose of the present study was to investigate sleep structure in post‐traumatic stress disorder (PTSD) patients with and without any psychiatric comorbidities. The relationship between sleep variables and measurements of clinical symptom severity were also investigated. Methods: Sleep patterns of 24 non‐medicated male PTSD patients and 16 age‐ and sex‐matched normal controls were investigated on polysomnography on two consecutive nights. Six PTSD‐only patients and 15 PTSD patients with major depressive disorder (MDD) were also compared to normal controls. Sleep variables were correlated with PTSD symptoms. Results: Compared to the normal controls, the PTSD patients with MDD had difficulty initiating sleep, poor sleep efficiency, decreased total sleep time, decreased slow wave sleep (SWS), and a reduced rapid eye movement (REM) sleep latency. The PTSD patients without any comorbid psychiatric disorders had moderately significant disturbances of sleep continuity, and decreased SWS, but no abnormalities of REM sleep. REM sleep latency was inversely proportional to the severity of startle response. SWS was found to be inversely correlated with the severity of psychogenic amnesia. Conclusions: PTSD patients have disturbance of sleep continuity, and SWS deficit, without the impact of comorbid depression on sleep. The relationship between SWS and the inability to recall an important aspect of trauma may indicate the role of sleep in the consolidation of traumatic memories. The relationship between the severity of the startle response and REM latency may suggest that REM sleep physiology shares common substrates with the symptoms of PTSD.  相似文献   

19.
Sleep is a prominent behaviour in the lives of animals, but the unresponsiveness that characterizes sleep makes it dangerous. Mammalian sleep is composed of two neurophysiological states: slow wave sleep (SWS) and rapid-eye-movement (REM) sleep. Given that the intensity of stimuli required to induce an arousal to wakefulness is highest during deep SWS or REM sleep, mammals may be most vulnerable during these states. If true, then animals should selectively reduce deep SWS and REM sleep following an increase in the risk of predation. To test this prediction, we simulated a predatory encounter with 10 wild-caught Norway rats (Rattus norvegicus), which are perhaps more likely to exhibit natural anti-predator responses than laboratory strains. Immediately following the encounter, rats spent more time awake and less time in SWS and REM sleep. The reduction of SWS was due to the shorter duration of SWS episodes, whereas the reduction of REM sleep was due to a lower number of REM sleep episodes. The onset of SWS and REM sleep was delayed post-encounter by about 20 and 100 min, respectively. The reduction of REM sleep was disproportionately large during the first quarter of the sleep phase, and slow wave activity (SWA) (0.5-4.5 Hz power density) was lower during the first 10 min of SWS post-encounter. An increase in SWA and REM sleep was observed later in the sleep phase, which may reflect sleep homeostasis. These results suggest that aspects of sleep architecture can be adjusted to the prevailing risk of predation.  相似文献   

20.
We assessed associations between polysomnographically determined sleep, especially the amount of slow-wave sleep (SWS), and body mass index (BMI) in patients with insomnia. One hundred and forty-one insomniacs and 55 healthy volunteers completed overnight polysomnographic recordings, and we measured height and body weight. No significant correlations were obtained between total sleep time and BMI among insomniacs. Compared with normal volunteers, insomnia patients exhibited longer sleep latency and shorter total sleep duration. While the two groups had no significant differences in BMI, insomniacs presented with more N1 but less time spend in SWS and rapid eye movement sleep (REMS). Based on their SWS time, we divided insomnia patients into three groups: short (26.99±13.88), intermediate (59.24±8.12), and long (102.21±26.17) SWS groups. The short-SWS group had significantly greater BMI than the long-SWS group. Further analyses with multiple linear regression showed a significant negative correlation between the amount of SWS and BMI scores in insomniacs, whereas no such correlation was found in healthy volunteers after controlling for potential confounds (e.g., age, sex and AHI). Our study suggests that low amounts of SWS may be associated with higher BMI in patients with insomnia.  相似文献   

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