Cri-du-chat syndrome is a rare genetic disorder in which the patient presents with a characteristic high-pitched monotonous cry and recurrent aspiration pneumonia, attributed to abnormalities in the larynx, epiglottis, and nervous system. The most prominent brain MRI findings are the presence of pontine and cerebellar hypoplasia, which primarily involve posterior cranial fossa structures. Although atrophy of supratentorial structures were also a common radiological finding, it was considered to be a secondary change due to pontine hypoplasia. Here, we present the case of a three-month-old patient presenting with cri-du-chat at our institution. The patient also showed the presence of prominent pontine hypoplasia similar to previously reported cases; however, contrary to other cases, there was a general delayed myelination of brain instead of decreased myelination of anterior limb of internal capsule. Since the larynx, pons, and cerebellum all originated from similar notochord level, which suggests anomaly in early stage of development, laryngeal, and brain anomaly characteristically observed in the cridu-chat syndrome.
Park, Kee Hyung;Kim, Sung-Wan;Shin, Dong-Jin;Park, Hyun-Mi;Lee, Yeong-Bae;Seung, Young-Hee
Korean Journal of Biological Psychiatry
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v.15
no.1
/
pp.46-53
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2008
Posterior cortical atrophy(PCA) is a presenile dementia that presents primarily with signs and symptoms of cortical visual dysfunction, while memory is relatively preserved until the late stage of the disease. We report a patient with PCA, confirmed by brain magnetic resonance imaging (MRI) and $F^{18}$-fluorodeoxyglucose positron emission tomography(FDG PET). A 58-year-old right-handed woman presented initially with visual dimness and difficulty finding things around her. She had partial Balint's syndrome, partial Gerstmann syndrome, and idiomotor apraxia. She also had a mild memory disturbance, but preserved insight of her disease. Neuropsychological evaluation showed decreased parietal and left temporal functions bilaterally. Brain MRI and $F^{18}$-FDG PET revealed typical bilateral occipitoparietal atrophy and hypometabolism, which were slightly worse on the right side. Cholinesterase inhibitor administration for 6 months improved the memory impairment slightly, but not the cortical visual dysfunction. This is a typical case of PCA, confirmed by neurologic signs and imaging findings.
Anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis is a common autoimmune encephalitis that is noted to be a severe but treatable disease entity. Patients with anti-NMDAR encephalitis often develop psychotic symptoms, including delusions, hallucinations, and paranoia, as well as memory impairment and persistent loss of attention. However, MRI findings in such patients show no abnormalities in most cases. Although typical brain abnormality features, known as T2 hyperintensities, involve the brain parenchyma and contrast enhancement at the cerebral cortex or overlying meninges, isolated leptomeningeal enhancement has been rarely reported in anti-NMDAR encephalitis. Herein, we report a patient with anti-NMDAR encephalitis who presented with isolated leptomeningeal enhancement, additionally showing the diagnostic value of contrast-enhanced fluid-attenuated inversion recovery imaging.
Liu Haiying;Shin Tae-Beom;Youn Seong-Kuk;Oh Jong-Yong;Lee Young-Il;Choi Sun-Seob
Investigative Magnetic Resonance Imaging
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v.8
no.1
/
pp.17-23
/
2004
Purpose : To evaluate changes in total cerebral blood flow (tCBF) with aging, parenchymal volume changes and vascular abnormalities, using 2 dimensional (D) phase-contrast magnetic resonance imaging (PC MRI). Materials and Methods : Routine brain MRI including T2 weighted image, time-of-flight (TOF) MR Angiography (MRA) and 2D PC MRI were performed in 73 individuals, including 12 volunteers. Normal subjects (12 volunteers, and 21 individuals with normal MRI and normal MRA) were classified into groups according to age (18-29, 30-49 and 50-66 years). For the group with abnormalities in brain MRIs, cerebral parenchymal volume changes were scored according to the T2 weighted images, and atherosclerotic changes were scored according to the MRA findings. Abnormal groups were classified into 4 groups: (i) mild reduction in volume, (ii) marked reduction in volume by parenchymal volume and atherosclerotic changes, and (iii) increased volume and (iv) Moya-moya disease. Volumetric flow was measured at the internal carotid artery (ICA) and vertebral artery bilaterally using the velocity-flow diagrams from PC MRI, and combined 4 vessel flows and tCBF were compared among all the groups. Results : The age-specific distribution of tCBFs in normal subjects were as follows: $12.0{\pm}2.1ml/sec$ in 18-29 years group, $11.8{\pm}1.9ml/sec$ in 30-49 years group, $10.9{\pm}2.2ml/sec$ in 50-66 years group. The distribution of tCBFs in the different subsets of the abnormal population were as follows: $9.5{\pm}2.5ml/sec$ in the group with mild reduction in volume, $7.6{\pm}2.0ml/sec$ in the group with marked reduction in volume, and $7.3{\pm}1.2ml/sec$ and $7.0{\pm}1.1ml/sec$ in the increased parenchymal volume and Moya-moya disease groups respectively. Conclusion : Total cerebral blood flow decreases with increasing age with a concomitant reduction in parenchymal volumes and increasing atherosclerotic changes. It is also reduced in the presence of increased parenchymal volume and Moya-moya disease.2D PC MRI can be used as a tool to evaluate tCBF with aging and in the presence of various conditions that can affect parenchymal volume and cerebral vasculature.
Objectives: Recently, the effect of acupuncture has been approved not only in the East but also in the West, so the interest on acupuncture was greatly improved. Especially, functional magnetic resonance imaging(fMRI) was embossed as the study tool for the mechanism of acupuncture noninvasively and many studies on the mechanism of acupuncture using fMRI were carried out. We archived the fMRI study on the brain activity induced by manual acupuncture at BL62(申脈). Methods: The study was the acupuncture at BL62(申脈) and we acquired 9 fMRI results from 6 persons$(age\;20{\sim}30,\;4\;male\;and\;2\;female)$. These studies employed The block design for mapping brain activity and acupuncture was perfomed at BL62(申脈) on the left foot. Results: The brain related motor function was cerebellum, basal ganglia and cerebral cortex and thalamus connected these elements. In the result of this study, the regions of significant activation in the cerebellum was centered on the spinocerebellum in the anterior lobe, so we presumed that this result showed the input of stimulation by the acupuncture on BL62(申脈). But basal ganglia and cerebral cortex showed the regions of significant activation in the left larger than the right and regions of the cerebral cortex was the motor and sensory cortex. Such a result explained that acupuncture at BL62(申脈) could have influence the motor function and acupuncture at left BL62(申脈) could affect the right side through the activation of the left basal ganglia and cerebral cortex. Conclusions: In the theory of crossing needling at collaterals(繆刺論), it the pathogenic factor invaded in the Yang Heel channel(陽?脈) that was one of the eight Extra meridians(奇經八脈), we recognized the disease of the collateral channel and used contralateral BL62(申脈) for treatment of the Yang Heel channel(陽?脈). Moreover the result of this study could bear the construction that acupuncture at the left BL62(申脈) treats the contralateral lesion and this construction is related to the theory of crossing needling at collaterals(繆刺論).
Lee, Hyun Sil;Ahn, Kook Jin;Choi, Hyun Seok;Jang, Jin Hee;Jung, So Lyung;Kim, Bum Soo;Yang, Dong Won
Investigative Magnetic Resonance Imaging
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v.18
no.4
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pp.332-340
/
2014
Purpose : A relative increase in deoxyhemoglobin levels in hypoperfused tissue can cause prominent hypointense signals in the draining veins (PHSV) within areas of impaired perfusion in susceptibility-weighted imaging (SWI). The purpose of this study is to evaluate the usefulness of SWI in patients with acute cerebral infarction by evaluating PHSV within areas of impaired perfusion and to investigate the usefulness of PHSV in predicting prognosis of cerebral infarction. Materials and Methods: In 18 patients with acute cerebral infarction who underwent brain MRI with diffusion-weighted imaging and SWI and follow-up brain MRI or CT, we reviewed the presence and location of the PHSV within and adjacent to areas of cerebral infarction qualitatively and measured the signal intensity difference ratio of PHSVs to contralateral normal appearing cortical veins quantitatively on SWI. The relationship between the presence of the PHSV and the change in the extent of infarction in follow-up images was analyzed. Results: Of the 18 patients, 10 patients showed progression of the infarction, and 8 patients showed little change on follow- up imaging. On SWI, of the 10 patients with progression 9 patients showed peripheral PHSV and the newly developed infarctions corresponded well to area with peripheral PHSV on initial SWI. Only one patient without peripheral PHSV showed progression of the infarct. The patients with infarction progression revealed significantly higher presence of peripheral PHSV (p=0.0001) and higher mean signal intensity difference ratio (p=0.006) comparing to the patients with little change. Conclusion: SWI can demonstrate a peripheral PHSV as a marker of penumbra and with this finding we can predict the prognosis of acute infarction. The signal intensity difference of PHSV to brain tissue on SWI can be used in predicting prognosis of acute cerebral infarction.
Purpose : In order to investigate the functional brain anatomy associated with mental calculation, functional magnetic resonance imaging was performed. Materials and Methods : In six normal right handed subjects, functional MR images were obtained using a 1.57 MR scanner and the EPI BOLD technique. The study included experiment I and experiment II. Each experiment consisted of five resting and four activation periods with each period of 30 seconds. During the activation period of both experiment I and II, calculation equations[an example: $(4+5)\times8=72$] were presented and the subjects were instructed to decide true or false of them. During the resting period of experiment I, the subjects were instructed to visually fixate on a crosshair. During the resting period of experiment II, two diagrams (an example: $(\bullet,\;\blacksquare)$)were presented and the subjects were instructed to decide they are same or not. For the post-processing of images, the SPM program was used, with the threshold of significance set at p<0.00001. The activated areas during the tasks were assessed. Results : In experiment 1, the inferior frontal gyrus, prefrontal cortex, promoter area, supplementary motor area, and intraparietal sulcus including superior parietal cortex were activated bilaterally. Although these areas were also activated in experiment II, the activated signals in the right frontal and parietal lobes were lessened. Conclusion : The left inferior frontal gyrus and prefrontal cortex and bilateral intraparietal sulci were activated during mental calculation. The right frontal and parietal lobes might be related to attention and decision making.
Purpose: The aim of this study was to investigate the effect of perinatal risk factors on brain maturation and the relationship of brain maturation and neurodevelopmental outcomes with brain maturation scoring system in brain MRI. Methods: ELBWI infants born at the Seoul National University Children's Hospital from January 2006 to December 2010 were included. A retrospective analysis was performed with their medical record and brain MR images acquired at near full term. We read brain MRI and measured maturity with total maturation score (TMS). TMS is a previously developed anatomic scoring system to assess brain maturity. The total maturation score was used to evaluate the four parameters of maturity: (1) myelination, (2) cortical infolding, (3) involution of glial cell migration bands, and (4) presence of germinal matrix tissue. Results: Images from 124 infants were evaluated. Their mean gestational age at birth was 27.1${\pm}$2.1 weeks, and mean birth weight was 781.5${\pm}$143.9 g. The mean TMS was 10.8${\pm}$2.0. TMS was significantly related to the postmenstrual age (PMA) of the infant, increasing with advancing postmenstrual age (P<0.001). TMS showed no significance with neurodevelopmental delay, and with brain injury, respectively. Conclusion: TMS was developed for evaluating brain maturation in conventional brain MRI. The results of this study suggest that TMS was not useful for predicting neurodevelopmental delay, but further studies are needed to make standard score for each PMA and to re-evaluate the relationship between brain maturation and neurodevelopmental delay.
Objective : Total resection without consecutive postoperative whole brain radiation therapy is indicated for patients with a single or two sites of brain metastasis, with close follow-up by serial magnetic resonance imaging (MRI). In this study, we explored the effectiveness, usefulness, and safety of this follow-up regimen. Methods : From January 2006 to December 2015, a total of 109 patients (76 males, 33 females) underwent tumor resection as the first treatment for brain metastases (97 patients with single metastases, 12 with two metastases). The mean age was 59.8 years (range 27-80). The location of the 121 tumors in the 109 patients was supratentorial (n=98) and in the cerebellum (n=23). The origin of the primary cancers was lung (n=45), breast (n=17), gastrointestinal tract (n=18), hepatobiliary system (n=8), kidney (n=7), others (n=11), and unknown origin (n=3). The 121 tumors were totally resected. Follow-up involved regular clinical and MRI assessments. Recurrence-free survival (RFS) and overall survival (OS) after tumor resection were analyzed by Kaplan-Meier methods based on clinical prognostic factors. Results : During the follow-up, MRI scans were done for 85 patients (78%) with 97 tumors. Fifty-six of the 97 tumors showed no recurrence without adjuvant local treatment, representing a numerical tumor recurrence-free rate of 57.7%. Mean and median RFS was 13.6 and 5.3 months, respectively. Kaplan-Meier analysis revealed the cerebellar location of the tumor as the only statistically significant prognostic factor related to RFS (p=0.020). Mean and median OS was 15.2 and 8.1 months, respectively. There were no significant prognostic factors related to OS. The survival rate at one year was 8.2% (9 of 109). Conclusion : With close and regular clinical and image follow-up, initial postoperative observation without prompt postoperative radiation therapy can be applied in patients of brain metastasi(e)s when both the tumor(s) are completely resected.
Kim, Nam Young;Kim, Ju Hui;Lim, Jun;Kang, Seong-Hyeon;Lee, Youngjin
Journal of the Korean Society of Radiology
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v.15
no.1
/
pp.9-14
/
2021
Recently, magnetic resonance imaging (MRI), which can acquire images with good contrast without exposure to radiation, has been widely used for diagnosis. However, noise that reduces the accuracy of diagnosis is essentially generated when acquiring the MR images, and by adjusting the parameters, the noise problem can be solved to obtain an image with excellent characteristics. Among the parameters, the number of excitation (NEX) can acquire images with excellent characteristics without additional degradation of image characteristics. In contrast, appropriate NEX setting is required since the scan time increases and motion artifacts may occur. Therefore, in this study, after fixing all MRI parameters through the MRiLab simulation program, we tried to evaluate the tendency of image characteristics according to changing NEX through quantitative evaluation of brain T2 weighted images acquired by adjusting only NEX. To evaluate the noise level and similarity of the acquired image, signal to noise ratio (SNR), contrast to noise ratio (CNR), root mean square error (RMSE) and peak signal to noise ratio (PSNR) were calculated. As a result, both noise level and similarity evaluation factors showed improved values as NEX increased, while the increasing width gradually decreased. In conclusion, we demonstrated that an appropriate NEX setting is important because an excessively large NEX does not affect image characteristics improvement and cause motion artifacts due to a long scan.
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