• Korean Journal of Psychosomatic Medicine
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• v.26 no.2
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• pp.119-126
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• 2018

Objectives : The aim of this study is to investigate correlation between degree of white matter hyperintensities (WMH) and neurocognitive function along with behavioral and psychological symptoms of dementia (BPSD) in Korean patients with Alzheimer's disease (AD) and mild cognitive impairment (MCI). Methods : Participants were 115 elderly subjects diagnosed with Alzheimer's disease or mild cognitive impairment in this retrospective study. WMH in brain MRI were rated with standardized visual rating scales (Fazekas scales) and the subjects were divided into two groups according to Fazekas scale. Cognitive function was evaluated with Korean version of the consortium to establish a registry for Alzheimer's Disease (CERAD-K), and BPSD was evaluated with Korean neuropsychiatric inventory (K-NPI). Independent t-test was performed to analyze the relationship between the degree of WMH and neurocognitive functions & BPSD. Results : Especially, the group with high severity of WMH showed significantly lower language fluency (p<0.05). In addition, the group with high severity of WMH showed significantly higher score in K-NPI. Conclusions : There was a significant association between WMH and neurocognitive test related with executive function. Moreover, WMH seems to affect BPSD severity. Evaluation of WMH would provide useful information in clinical settings.

• Dementia and Neurocognitive Disorders
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• v.22 no.1
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• pp.28-42
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• 2023

Background and Purpose: We investigated the correlation between the deep distribution of white matter hyperintensity (WMH) (dWMH: WMH in deep and corticomedullary areas, with minimal periventricular WMH) and a positive agitated saline contrast echocardiography result. Methods: We retrospectively recruited participants with comprehensive dementia evaluations, an agitated saline study, and brain imaging. The participants were classified into two groups according to WMH-distributions: dWMH and dpWMH (mainly periventricular WMH with or without deep WMH.) We hypothesized that dWMH is more likely associated with embolism, whereas dpWMH is associated with small-vessel diseases. We compared the clinical characteristics, WMH-distributions, and positive rate of agitated saline studies between the two groups. Results: Among 90 participants, 27 and 12 met the dWMH and dpWMH criteria, respectively. The dWMH-group was younger (62.2±7.5 vs. 78.9±7.3, p<0.001) and had a lower prevalence of hypertension (29.6% vs. 75%, p=0.008), diabetes mellitus (3.7% vs. 25%, p=0.043), and hyperlipidemia (33.3% vs. 83.3%, p=0.043) than the dpWMH-group. Regarding deep white matter lesions, the number of small lesions (<3 mm) was higher in the dWMH-group(10.9±9.7) than in the dpWMH-group (3.1±6.4) (p=0.008), and WMH was predominantly distributed in the border-zones and corticomedullary areas. Most importantly, the positive agitated saline study rate was higher in the dWMH-group than in the dpWMH-group (81.5% vs. 33.3%, p=0.003). Conclusions: The dWMH-group with younger participants had fewer cardiovascular risk factors, showed more border-zone-distributions, and had a higher agitated saline test positivity rate than the dpWMH-group, indicating that corticomedullary or deep WMH-distribution with minimal periventricular WMH suggests embolic etiologies.

• Clinical and Experimental Pediatrics
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• v.53 no.1
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• pp.106-110
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• 2010

Hippocampal sclerosis (HS) is one of the most common features of intractable temporal lobe epilepsy. Generally it can be identified through brain magnetic resonance imaging (MRI) with high degree of sensitivity and specificity. Typical brain MRI findings of HS are hippocampal atrophy with hyperintense signal confined to the lesion. On the other hand cortical dysplasia exhibits blurring of the gray-white matter junction and abnormal white matter signal intensity. We present a case where preoperative brain MRI strongly suggested the presence of diffuse cortical dysplasia in the left temporal lobe but postoperative pathology revealed the temporal lesion to be unremarkable except for hippocampal sclerosis.

• Clinical and Experimental Pediatrics
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• v.46 no.9
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• pp.934-938
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• 2003

Canavan disease, also known as van Bogaert-Bertrand disease, is a rare autosomal recessive disorder characterized by early an onset and a progressive spongyform degeneration of the brain, associated with an edema of the central nerve system, intramyelinic swelling and neurologic symptoms. This disorder is most prevalent in people of Ashkenazi Jewish descent but has been observed in other ethnic groups. Patients have severe mental retardation, poor head control, macrocephaly and seizures. Canavan disease is caused by the accumulation of N-acetylaspartic acid(NAA) in the brain as the result of a deficiency of aspartoacylase(ASPA) activity. Most children are reported to have the infantile form, becoming symptomatic between three and six month of age, after unremarkable prenatal and perinatal course. We experienced a case of Canavan disease in a six day old female newborn baby, associated with seizure, degeneration of brain white matter and markedly elevated urine N-acetylaspartic acid(NAA) level. So, we report the case with a brief review of the related literature.

• Journal of Korean Neurosurgical Society
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• v.52 no.1
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• pp.55-57
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• 2012

Alveolar soft part sarcoma (ASPS) with skull and brain metastases is extremely rare. A 53-year-old patient diagnosed as skull metastasis of ASPS visited our clinic complaining of an outgrowing scalp mass in spite of radiation therapy. Past medical history revealed that the patient had been diagnosed and treated for ASPS of the thigh 4 years ago. Magnetic resonance imaging revealed a hyperintense ovoid mass on the T2-weighted image, an isointense on the T1-weighted image, and a homogeneous enhanced mass with gadolinium. Another small-sized enhanced mass with mild peritumoral swelling was found at the deep white matter of the left frontal lobe. A gross total resection of the skull lesion with cranioplasty was performed for the surgical defect. A histologic examination of the specimens revealed metastatic ASPS involving the skull. Surgery with a total removal of the lesions may be effective for improving a patient's symptoms especially from neurological dysfunction.

• Journal of Genetic Medicine
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• v.14 no.2
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• pp.67-70
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• 2017

Down syndrome (DS, trisomy 21) is associated with neuroanatomical abnormalities, including choroid plexus cysts and various types of brain tumors. Trisomy 21 is associated with oncogenic factor, especially in brain tumor. The brain of DS patients had a smaller volume of gray and white matter and an unbalanced cerebellum volume, indicating a smaller volume overall than normal. We report a case of a DS male patient who had an incidentally discovered neuroglial cyst in left cerebellar vermis. He visited our hospital with gait disturbance and fatigue. But, the neurologic exam was normal. To the best of our knowledge, this is the first reported case of a neuroglial cyst in a trisomy 21 patient. As the developmental mechanisms of a cyst and the choroid plexus are related, more research is needed.

• The Journal of Korean Physical Therapy
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• v.15 no.4
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• pp.199-207
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• 2003

This review describes the neurophathological mechanisms that are implicated in perinatal brain injury. Perinatal brain injury is the most important cause of morbidity and mortality to infants, often leading to spastic motor deficits, mental retardation, seizures, and learning impairments. The immature brain injury is usually caused by cerebral hypoxia-ischemia, hemorrhage, or infection. The important form of perinatal brain injury is the hypoxic-ischemic injury and the cerebral hemorrhage. The pathology of hypoxic-ischemic injury include delayed energy failure by mitochondrial dysfunction, neuronal excitotoxicity and vulnerability of white matter in developing brain. The immature brain has the fragile vascular bed of germinal matrix and can not effectively centralize their circulation. Therefore, the cerebral hemorrhage process is considered to be involved in the periventricular leukomalacia.

• Journal of the Institute of Electronics Engineers of Korea TC
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• v.46 no.12
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• pp.102-107
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• 2009

As technology gets developed, medical equipments are also modernized and leading-edge systems, such as PACS become popular. Many scientists noticed importance of medical image processing technology. Technique of region segmentation is the first step of digital medical image processing. Segmentation technique helps doctors to find out abnormal symptoms early, such as tumors, edema, and necrotic tissue, and helps to diagnoses correctly. Segmentation of white matter, gray matter and CSF of a brain image is very crucial part. However, the segmentation is not easy due to ambiguous boundaries and inhomogeneous physical characteristics. The rate of incorrect segmentation is high because of these difficulties. Fuzzy-based segmentation algorithms are robust to even ambiguous boundaries. In this paper a modified Fuzzy-based segmentation algorithm is proposed to handle the noise of MR scanners. A proposed algorithm requires minimal computations of mean and variance of neighbor pixels to adjust a new neighbor list. With the addition of minimal compuation, the modified FCM(mFCM) lowers the rate of incorrect clustering below 30% approximately compared the traditional FCM.

• Journal of Biomedical Engineering Research
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• v.28 no.3
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• pp.317-324
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• 2007

The application of diffusion tensor imaging (DTI) and fiber tractography to Wallerian degeneration (WD) is important because this technique is a very potent tools for quantitatively evaluating fiber tracts in vivo brain. We analyzed a case and control using tracts of interest (TOI) analysis to quantify WD. We scanned a case of old infarction and an age-matched healthy volunteer. T1 magnetization prepared rapid acquisition gradient echo (MPRAGE), fluid attenuated inversion recovery (FLAIR) and 12-direction diffusion tensor imaging (DTI) were obtained and analyzed using TOI analysis. The value of mean diffusity ($D_{av}$) and fracional anisotrophy (FA) were analyzed statistically by MWU test. A p-value of less than 0.05 was considered to indicate statistical significance. A comparison of the global fiber diffusion characteristics shows WD of both the corpus callosum and the ipsilateral superior longitudinal fasciculus. The corpus callosum in particular showed trans-hemispherical degeneration. Local fiber characteristics along the geodesic paths show WD in the corpus callosum, ipsilateral superior longitudinal fasciculus, ipsilateral corticospinal tract, and ipsilateral corticothalamic tract. We have demonstrated changes in $D_{av}$ and FA values and a clear correspondence with the WD in various tracts. TOI analysis successfully revealed radial WD in white matter tracts from a region of encephalomalacia and primary gliosis, although they were only slightly affected.

• Clinical and Experimental Pediatrics
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• v.59 no.sup1
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• pp.139-144
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• 2016

Encephalocraniocutaneous lipomatosis (ECCL) is a rare neurocutaneous syndrome that affects ectomesodermal tissues (skin, eyes, adipose tissue, and brain). The neurologic manifestations associated with ECCL are various including seizures. However, ECCL patients very rarely develop brain tumors that originate from the neuroepithelium. This is the first described case of ECCL in combination with dysembryoplastic neuroepithelial tumor (DNET) that presented with intractable seizures. A 7-year-old girl was admitted to our center because of ECCL and associated uncontrolled seizures. She was born with right anophthalmia and lipomatosis in the right temporal area and endured right temporal lipoma excision at 3 years of age. Seizures began when she was 3 years old, but did not respond to multiple antiepileptic drugs. Brain magnetic resonance (MR) imaging performed at 8 and 10 years of age revealed an interval increase of multifocal hyperintense lesions in the basal ganglia, thalamus, cerebellum, periventricular white matter, and, especially, the right temporal area. A nodular mass near the right hippocampus demonstrated the absence of N-acetylaspartate decrease on brain MR spectroscopy and mildly increased methionine uptake on brain positron emission tomography, suggesting low-grade tumor. Twenty-four-hour video electroencephalographic monitoring also indicated seizures originating from the right temporal area. Right temporal lobectomy was performed without complications, and the nodular lesion was pathologically identified as DNET. The patient has been seizure-free for 14 months since surgery. Although ECCL-associated brain tumors are very rare, careful follow-up imaging and surgical resection is recommended for patients with intractable seizures.