Leucine-rich repeat-containing G-protein coupled receptor 5 (LGR5) has been reported to play critical roles in the proliferation of various cancer cells. However, the roles of LGR5 in brain tumors and the specific intracellular signaling proteins directly associated with it remain unknown. Expression of LGR5 was first measured in normal brain tissue, meningioma, and pituitary adenoma of humans. To identify the downstream signaling pathways of LGR5, siRNA-mediated knockdown of LGR5 was performed in SH-SY5Y neuroblastoma cells followed by proteomics analysis with 2-dimensional polyacrylamide gel electrophoresis (2D-PAGE). In addition, the expression of LGR5-associated proteins was evaluated in LGR5-inhibited neuroblastoma cells and in human normal brain, meningioma, and pituitary adenoma tissue. Proteomics analysis showed 12 protein spots were significantly different in expression level (more than two-fold change) and subsequently identified by peptide mass fingerprinting. A protein association network was constructed from the 12 identified proteins altered by LGR5 knockdown. Direct and indirect interactions were identified among the 12 proteins. HSP 90-beta was one of the proteins whose expression was altered by LGR5 knockdown. Likewise, we observed decreased expression of proteins in the hnRNP subfamily following LGR5 knockdown. In addition, we have for the first time identified significantly higher hnRNP family expression in meningioma and pituitary adenoma compared to normal brain tissue. Taken together, LGR5 and its downstream signaling play critical roles in neuroblastoma and brain tumors such as meningioma and pituitary adenoma.
Brain ; Brain Neoplasms ; Cell Proliferation ; Dermatoglyphics ; Electrophoresis, Polyacrylamide Gel ; GTP-Binding Proteins ; Heterogeneous-Nuclear Ribonucleoproteins ; Humans ; Intracellular Signaling Peptides and Proteins ; Meningioma ; Neuroblastoma ; Pituitary Neoplasms ; Proteomics

BACKGROUND: Pediatric epilepsy can result in a heavy burden of illness for the family and the role of family caregivers is becoming more important for the management of the disease. However, there are only few studies about care-giver burden of pediatric epilepsy. This study examined the factors which may influence care-giver burden of pediatric epilepsy patients METHODS: We surveyed 87 care-givers of pediatric epilepsy patients. The demographic and social data of the care-givers, along with the social and clinical data of the patients were obtained. The Korean version of the Burden Interview (KBI) and the Korean version of the Beck Depression Inventory (KBDI) were used. The relationship between the demographic and social data of care-givers, the clinical factors of the patients and KBI score were evaluated. RESULTS: Of the 87 participants, sixty-nine (79.3%) were the mothers. The mean age of caregivers was 41.3 years and the mean score on the KBI was 20.2 (+/-16.8) points. The mean age of the patients was 11.1 years and 48 patients (55.8%) were male. The number of antiepileptic drugs prescribed, severity of the disease and school record had a significant correlation with KBI in a multivariate linear regression analysis. CONCLUSIONS: We suggest that in addition to the strict control of the seizure, that the consideration for the academic functions of pediatric patients is also important for reducing care-giver burden in the treatment of pediatric epilepsy.
Anticonvulsants ; Caregivers ; Cost of Illness ; Depression ; Epilepsy* ; Humans ; Linear Models ; Male ; Mothers ; Seizures

A hemodialysis arteriovenous fistula may cause high output cardiac state by volume overload and decreased systemic vascular resistance. Arteriovenous fistulas for hemodialysis consist usually of either a radiocephalic fistula at the wrist or a more proximal brachiocephalic fistula at the antecubital fossa. In this study the contribution of arteriovenous fistula to cardiac function has been evaluated by comparing cardiac performance before and after the point occlusion of the anastomotic site. The results suggest that the occlusion of arteriovenous fistula may decrease end-diastolic left ventricular volume (VOLd), increase end-systolic left ventricular volume (VOLs), decrease stroke volume (SV), decrease ejection fraction (EF) and decrease cardiac output (CO). This study shows that arteriovenous fistula influences directly to cardiac performance, so careful determination of dialysis option is required for the end stage renal failure patients with marginal heart function.
Arteriovenous Fistula ; Cardiac Output ; Dialysis ; Fistula ; Heart ; Humans ; Renal Dialysis ; Renal Insufficiency ; Stroke Volume ; Vascular Resistance ; Wrist

Stroke in young adults are not common and it is often hard to find their causes. We reviewed the medical records of 154 young adult patients aged 15-45 years who were admitted to the Seoul National Urliversity Hospital with a diagnosis of stroke between March 1989 and February 1991. These cases comprised 13.8% of 1115 patients of all ages admitted for stroke. The number of young adult patients with intracerebral hemorrhage was 47 (30.5%, N&154); the main causes were hypertension, arteriovenous malformation, and moyamoya disease. Subarachnoid hemorrhage was found in 25 young patients (l6.2%, N=154); the majority were due to aneurysms. The number of intraventricular hemorrhage cases was 17 (11.0%, N&154). The causes confirmed by angiography, were moyamoya disease in 7 cases and arteriovenous malformahon in 5 cases. The remaining 73 patients (47.4%, N&154) had cerebral infarction;the major causes were young-aged atherosclerosis (75.3%) and cardiogenic emboli (24.7%). Hypertension, history of transient ischemic attack, and hyperlipidemia were major risk factors of atherosclerotic cerebral infarction. Among the cerebral infarction patients whose risk factors had not been found by conventional diagnostic methods (l6 patients), there were 5 patients in whom the presence of cardiac embolic sources could be demonstrated only by transesophageal echocardiography (left atrial thrombus in two patients; patent foramen ovale in two; and atrial septal aneurvsm in one patient). The total number of all tvpes of young adult stroke patients whose causes (or risk factors) were unable to be classified in detail was 24 (15.6%, N&154). The transesophageal echocardiography and the angiography, in many occasions, were helpful in detecting the causes of young adult stroke of which risk factors had been undetermined.
Aneurysm ; Angiography ; Arteriovenous Malformations ; Atherosclerosis ; Cerebral Hemorrhage ; Cerebral Infarction ; Diagnosis ; Echocardiography, Transesophageal ; Foramen Ovale, Patent ; Hemorrhage ; Humans ; Hyperlipidemias ; Hypertension ; Ischemic Attack, Transient ; Medical Records ; Moyamoya Disease ; Risk Factors ; Seoul ; Stroke* ; Subarachnoid Hemorrhage ; Thrombosis ; Young Adult*

Forty-one patients with hemifacial spasm received botulinum A toxin in total 73 visits. In 68 of 73 visits(93%), substantial improvement with peak effect(change in severity of hemifacial spasm 22 ) was obtained, with a mean total dose of 12.5 units. Mean severity of hemifacial spasm before and after treatrnent were 3.8 and 1.1 respectively on a 0-5 scale. Mean muscle dose of orbicularis oculi was 9.0 units There was a significant association between muscle dose and post-treatment weakness of orbicularis oculi(Spearman correlation coefficient, 0.42691). There was also a striking and significant association between post-treatment weakness of orbicularis oculi and peak effect(Spearman correlation coefficient, 0.74883). Lagophthalmos occurred only in 2 of 28 visits (7%) who received lesser than 10.0 units for orbicularis oculi as compard to 22% in the higher dose group. But peak effects showed no significant difference between the two groups(p =O.I30). We suggest that the occurrence of lagophthalmos can be reduced with the dose lesser than 10.0 units for orbicularis oculi, maintaining the significant effect.
Botulinum Toxins, Type A* ; Hemifacial Spasm* ; Humans ; Strikes, Employee

To evaluate the efficacy of magnetic resonance imaging in brainstem stroke, we studied 21 cases of clinically definite brainstem ischemic stroke with brain magnetic resonance imaging (MRI) and conventional computed tomography (CT). MRI demonstrated brainstem lesions in 79% of the cases (16.5 out of 21), while CT revealed 33% (7 out of 21) when cases with suspicious lesions counted as 0.5. Although MRI was done a few days later than CT in most cases, MRI was superior to CT in detecting the number and the size of ischemic lesions, with clear delineation of anatomy and visualization of the status of the blood flow in the vertebral-basilar artery. Disappearance of the flow signal void in the basilar artery can be an important clue in diagnosing occlusion or thrombus of the basilar artery. By delineating the extent and the location of the infarction, MRI findings allowed an interpretation of whether the ischemic vessel is a small basilar branch or a large vessel vertebral or basilar artery.
Adult ; Aged ; Brain Ischemia/complications/*diagnosis/radiography ; Brain Stem/blood supply/*pathology/radiography ; Cerebral Infarction/etiology/pathology ; Female ; Humans ; Magnetic Resonance Imaging ; Male ; Middle Aged ; Tomography, X-Ray Computed

Paraplegia following intrathecal methotrexate(MTX) instillation is a rare complication and 18 cases have been reported in the literature. It has not been reported m Korea. We recenty experienced a 56 year-old female with quadriplegia following intrathecal MTX instillation. So we report a case of paraplegia following intrathecal MTX instillation with review of the literature.
Female ; Humans ; Korea ; Methotrexate* ; Middle Aged ; Paraplegia* ; Quadriplegia

Juvenile myoclonic epilepsy(JME) of Janz is a syndrome of generalized age related epilepsy with increasing incidence and probable autosomal recessive trait. We have experienced 2 female patients who had awakening myoclonus in both arms, which had started in their early 10th. The patterns of seirures and electroencephalographic findings were consistent with those of JME which had been first described as impulsive petit mal by Janz. We report these cases with brief review of literatures.
Arm ; Epilepsy ; Female ; Humans ; Incidence ; Myoclonic Epilepsy, Juvenile* ; Myoclonus

Adie's tonic pupil usually consists of mydriasis, defect in accommodation. Poor or absent light reflex, and tonic near reflex, It is usually unilateral, but rarelY observed bilaterally. The pupillary abnormalities often accompany areflexia, which is called Adie syndrome collechvely, Other autonomic dysfunction especially hypohidrosis. Are rarely reported in association with Adie syndrome. We recently experienced 2 cases of bilateral Adie's pupils in 2 women, one of whom had hypohidrosis, and the other had areflexia. So we report 2 cases with bilateral Adie's pupils with a review of literature.
Adie Syndrome ; Female ; Humans ; Hypohidrosis ; Mydriasis ; Reflex ; Tonic Pupil*