• Clinical and Experimental Pediatrics
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• v.53 no.12
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• pp.994-999
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• 2010

Purpose: Mitochondrial dysfunction can present with various symptoms depending on the organ it has affected. This research tried to analyze the ophthalmologic symptoms and ophthalmologic examination (OE) results in patients with mitochondrial disease (MD). Methods: Seventy-four patients diagnosed with mitochondrial respiratory chain complex defect with biochemical enzyme assay were included in the study. They were divided into 2 groups based on the OE results by funduscopy and were analyzed on the basis of their clinical features, biochemical test results, morphological analysis, and neuroimaging findings. Results: Thirty-seven (50%) of the 74 MD patients developed ophthalmologic symptoms. Abnormal findings were observed in 36 (48.6%) patients during an OE, and 16 (21.6%) of them had no ocular symptoms. Significantly higher rates of prematurity, clinical history of epilepsy or frequent apnea events, abnormal light microscopic findings in muscle pathology, diffuse cerebral atrophy in magnetic resonance imaging, and brainstem hyperintensity and lactate peaks in magnetic resonance spectroscopy were noted in the group with abnormal OE results. Conclusion: Although the ophthalmologic symptoms are not very remarkable in MD patients, an OE is required. When the risk factors mentioned above are observed, a more active approach should be taken in the OE because a higher frequency of ocular involvement can be expected.

• Journal of Korean Neurosurgical Society
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• v.29 no.12
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• pp.1563-1569
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• 2000

Objective : Delayed ischemic neurologic deficit(DIND) is one of the major complications following aneurysmal subarachnoid hemorrhage(SAH). However, the correlation between angiographic vasospasm(AV) and DIND after SAH is not precisely known. The authors investigated the timing, incidence, characteristics of DIND, and analyzed correlation between AV and DIND. Patients and Methods : A series of 126 patients with SAH and performed cerebral angiography which, confirmed anterior circulation aneurysm, admitted to between January 1996 to December 1998, were studied retrospectively. A comparative analysis between group 1(G1) in which AV patients presented with DIND, and group 2(G2) patients did not DIND, were done. AV was graded according to location, distribution and degree. Location of vasospasm was classified as basal type(BT), distal type(DT). BT was involved horizontally and include the bilateral carotid systems, proximal middle cerebral artery(MCA) and proximal anterior cerebral artery(ACA). DT was involved vertically and include the MCA branches as they become vertically or posteriorly oriented and the ACA distal to the anterior communicating artery. BT and DT all defined ether as localized type(LT) or combined type(CT). Distribution of vasospasm was classified as type I, type II and type III. Type I represents the involvement of bilateral carotid systems and bilateral anterior cerebral artery, type II was designed as one carotid system without involving anterior cerebral artery, and type III when only some portions of the anterior cerebral artery were involved, bilaterally. Degree of vasospasm was classified as mild(less than 25%), moderate(between 25-50%), severe(greater than 50%), and those were determined by comparing the caliber of the artery in vasospasm to that of the nearest area of apparently normal vessel. Results : The incidence of AV & DIND was 57/126(45.2%), 29/126(23.0%), and timing of DIND was 9 days(${\pm}4.1$) after initial hemorrhage. As for the location, BT was seen in 12 cases(40.0%), DT 11 cases(36.7%) and CT 7 cases (23.3%), respectively. Where as G1, BT was seen 5 cases(18.5%), DT 5 cases(18.5%) and CT 17 cases(63.0%), respectively in G2. CT AV was more correlated with DIND than LT AV(p<0.05). For distribution, type I was seen in 16 cases(59.2%), type II 4 cases(14.8%), type III 7 cases(25.9%) in G1 where as type I was seen in 7 cases(23.3%), type II 10 cases(33.3%), type III 13(43.3%) in G2. Type I AV was well correlated with DIND unlike to type II or type III(p<0.05). As for the degree, mild was seen in 4 cases(14.8%), moderate 14 cases(51.9%), severe 9 cases (33.3%) in G1, and mild 16 cases(18.5%), moderate 11 cases(36.7%) and severe 3 cases(10.0%) in G2. Moderate to severe type AV was well correlated with DIND(p<0.05). Conclusion : These results indicate that it may be possible to predict DIND according to careful analysis of location, distribution, degree of AV in patients with aneurysmal SAH.

• Tuberculosis and Respiratory Diseases
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• v.59 no.1
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• pp.97-103
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• 2005

Hypereosinophilic syndrome (HES) is characterized by a sustained eosinophilia of $1,500/mm^3$ or more in the absence of any known causes or the signs and symptoms of organ involvement. We report a 64-year-old man with HES initially presenting with involvement of the liver and bone marrow. Despite controlling the eosinophilia by corticosteroid, he developed a cerebral infarction and later progressive interstitial pneumonia. Brain angiography revealed a severe stenosis of the proximal right internal carotid artery (ICA) and a complete obstruction of the intracranial ICA. An open lung biopsy revealed fibrosis and lymphoplasma cell infiltration without eosinophils, which were consistent with nonspecific interstitial pneumonia.

• Journal of Korean Neurosurgical Society
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• v.61 no.1
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• pp.120-126
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• 2018

Intraparenchymal meningiomas without dural attachment are extremely rare, especially when they occur in basal ganglia region in child. An 8-year-old boy was admitted at our hospital, complaining of recurrent headache and vomiting for 3 months. Neurological examination showed impaired vision and mild paresis of the left extremities. Magnetic resonance imaging demonstrated a lesion located in the right basal ganglia region extending to superasellar cistern with solid, multiple cystic and necrotic components. Computed tomography revealed calcification within the mass. Due to the anterior cerebral artery involvement, a subtotal resection was achieved and postoperative radiotherapy was recommended. Histopathological examination indicated that the lesion was an atypical meningioma. The postoperative rehabilitation was uneventful. Mildly impaired vision and motor weakness of left extremities improved significantly and the patient returned to normal life after surgery. To our knowledge, intraparenchymal atypical meningioma in basal ganglia extending to superasellar cistern was never reported. The significance in differential diagnosis of lesions in basal ganglia should be emphasized.

• Journal of Chest Surgery
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• v.13 no.2
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• pp.149-153
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• 1980

Von Recklinghausen's disease is a systemic hereditary disorder with varied manifestations in bone, soft tissue, nervous system, and skin, the most common of which is the developement of multiple, small, cutaneous tumors with a characteristic histologic picture. Tumors develop after birth and before puberty in most cases, and they increase in number until old age. Malignant neoplasms that complicate multiple neurofibromatosis include gliomas of the optic nerve, astrocytomaas of the cerebral and cerebellar hemispheres, and sarcomas of peripheral nerves (femoral, tibial and intercostal nerves) and somatic soft tissues. Little attention has been paid to the presence of cystic lung disease in association with neurofibromatosis. Currently, most think of thoracic involvement in neurofibromatosis in terms of posterior mediastinal neuroma, pheochrocytoma, meningocele or, less commonly parenchymal pulmonary neurofibromas. Author have experienced 2 cases of Von Recklinghausen's disease. One case developed a hyge malignant Schwannoma in the parietal pleura of left 4th intercostal space and multiple benign neurofibromas (two in intercostal spacees and one in the neck) , and the other has several episodes of pneumothorax resulting from diffuse cystic lung disease which required closed thoracotomy drainage.

• Journal of Korean Neurosurgical Society
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• v.50 no.1
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• pp.68-71
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• 2011

We present a case of Neuro-Behçet's disease with an unpredictable clinical course. A 47-year-old man was admitted to the neurosurgery department of our hospital with a mild headache. Three days after admission, his consciousness suddenly decreased and respiratory distress progressed rapidly. A brain MRI revealed that the previously observed abnormal signal had extended markedly to both the thalamic areas and the entire brain stem, and the surrounding brain parenchyma were compressed by cerebral edema. Based on the patient's symptoms of recurrent oral and genital ulcers, skin lesions, and uveitis, a rheumatologist made a diagnosis of Behçet's disease with CNS involvement. The patient was treated with high-dose methylprednisolone with respiratory assistance in the intensive care unit for 9 days and his neurologic symptoms improved remarkably. Neuro-Behçet's disease must be considered in the differential diagnosis in rapidly deteriorated young neurological patients along with a stroke, low-grade glioma, multiple sclerosis, and occlusive venous disease.

• Pediatric Infection and Vaccine
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• v.13 no.2
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• pp.196-200
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• 2006

Congenital toxoplasmosis is caused by vertical transmission of Toxoplasma gondii from mother during pregnancy. The broad clinical spectrum of congenital toxoplasmosis ranges from stillbirth or death shortly after birth to survival with either cerebral damage or mild or subclinical disease, consisting usually of ocular involvement. The authors experienced a case of congenital toxoplasmosis in prematurity baby whose mother had lived recently in Africa. The diagnosis of congenital toxoplasmosis was confirmed on the basis of clinical finding of chorioretinitis and positive serologic test for toxoplasma IgM antibody.

• Archives of Pharmacal Research
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• v.23 no.4
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• pp.413-417
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• 2000

The effect of ischemia/reperfusion-induced neuronal damage on the memory impairment were investigated using active avoidance and Morris water maze tasks in Wistar rats. Focal ischemia was induced by 1 h occlusion of the right middle cerebral artery (MCA) of Wistar male rats. Reperfusion was induced by releasing the occlusion and restoring the blood circulation for 24 h. The acquisition and preservation memory tested by active avoidance showed a significant difference between the sham and ischemia/reperfusion group. The water maze acquisition performance was also significant difference between sham and ischemia/repefusion groups in both latency and moving distance. The infarction volume was increased by the ischemia/reperfusion. Furthermore, the cresyl violet staining of the ischemia/reperfusion brain showed severe neuronal damage (pyramidal cell loss) in the cortex in addition to the striatum lesion of brain. This study shows that pyramidal cell damage in the cortex lesion may be partially related to memorial disturbance in the ischemia/reperfusion brain injury.

• Journal of Korean Medicine Rehabilitation
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• v.28 no.4
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• pp.103-112
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• 2018

Pathological laughing and crying (PLC) is a condition defined by relatively uncontrollable episodes of laughter, crying or both. PLC is an uncommon symptom usually caused by cerebral lesions. Midbrain involvement causing PLC is extremely unusual and the exact mechanism by which this condition develops is poorly understood. We recently experienced a 51-year-old woman who were diagnosed as PLC after midbrain infarction. She was treated by acupuncture, pulsed electromagnetic therapy (PEMT). After 6 weeks treatment, Pathological Laughter and Crying Scale (PLACS), Korean version of the Scale for the Assessment and Rating of Ataxia (K-SARA) are decreased and Korean version of Modified Barthel Index (K-MBI) is increased. Treatment of traditional Korean Medicine could be effective for stoke rehabilitation including post-stroke PLC. And we have considered mechanism of PLC associated with midbrain lesion, dysfunction of cortex-thalamus-hypothalamus-basal ganglia-mesencephalon and faciorespiratory nuclei pathways, cerebro-ponto-cerebellar pathways and damaged serotonergic neurotransmission can cause this based on recent neurobiology of emotion. To define exact mechanism and find effective treatment, further studies are needed.

• Journal of Korean Neurosurgical Society
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• v.30 no.2
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• pp.168-172
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• 2001

Objective : The authors have studied the clinical outcome of patients with diffuse axonal injuries(DAI) to evaluate the prognostic value of gradient-echo MR imaging findings. Materials and Methods : From March 1995 to March 1998, there were nineteen patients with DAI whose initial Glasgow coma scales were eight or less. Authors divided them into two groups according to Glasgow outcome scales ; those patients with GOS 3 or less(group A ; 9) and those with 4 or more(group B ; 10). We subdivided the lesions as superficial and deep lesion, and analyzed the numbers, anatomical loci of the lesions on the gradient echo images of each group. Results : Mean numbers of the lesions were 15 per case in group A(135/9) and 10 in group B(100/10). The common loci involved in DAI were cerebral cortex, brain stem, and corpus callosum. Cortical lesions were 31.1% in group A(42/135) and 47% in group B(47/100). Brain stem lesions were 25.9%(35/135) and 15%(15/100) each. Callosal lesions were 31.1%(26/135) and 13%(13/100) each. The frequency of callosal and brain stem lesions was significantly different between two groups(p<0.05). We divided callosal lesions as genu, body, and splenium and body lesions as anterior, middle, posterior, but no significant topographical difference of lesions was observed between two groups. Deep lesions were observed more frequently in group A(58.5%, 79/135) than group B(36%, 36/100). Conclusion : The poor outcome group showed more numbers of lesion and more frequent involvement of brain stem and corpus callosum than favorable outcome group. Gradient-echo MR imaging seems to have predictive value for clinical outcome in patients with DAI.