Wolff-Parkinson-White Syndrome in a Patient with Mitochondrial Encephalopathy, Lactic Acidosis, and Stroke-like Episodes Syndrome Mimicking Juvenile Myoclonic Epilepsy

No abstract available.

Screening for Normal Cognition, Mild Cognitive Impairment, and Dementia with the Korean Dementia Screening Questionnaire

OBJECTIVE: The Korean Dementia Screening Questionnaire (KDSQ) is an informant-based instrument used to screen for cognitive dysfunction. However, its ability to only dichotomously discriminate between dementia and normal cognition has been previously investigated. This study investigated the ability of the KDSQ to classify not only dichotomous but also multiple stages of cognitive dysfunction. METHODS: We examined 582 participants. Receiver operating characteristic (ROC) curves were used to determine dichotomous classification parameters. Multi-category ROC surfaces were evaluated to classify the three stages of cognitive dysfunction. RESULTS: Dichotomous classification using the ROC curve analyses showed that the area under the curve was 0.92 for dementia for subjects without dementia and 0.96 for dementia in controls. Simultaneous multi-category classification analyses showed that the volume under the ROC surface (VUS) was 0.57 and that the derived optimal cut-off points were 2 and 8 for controls, MCI, and dementia. The estimated Youden index for the KDSQ was 0.48, and the derived optimal cut-off points were 5 and 10. The overall classification accuracy of the VUS and Youden index was 61.2% and 58.6%, respectively. CONCLUSION: The KDSQ is useful for classifying dichotomous and multi-category stages of cognitive dysfunction.

The Inhibitory Mechanism of Gentamicin on Electrical Field Stimulation Response in Rat Bladder Smooth Muscle

To see the inhibitory mechanism of gentamicin in response to electrical field stimulation (EFS) using the rat bladder smooth muscle, atropine or guanethidine was treated but had no effect. Methylsergide, a non-selective 5-HT1, 5-HT2 receptor antagonist was also treated but had on effect. Kinase inhibitors, such as chelerythrine (PKC inhibitor), ML-9 (MLCK inhibitor), or Y27632 (rho kinase inhibitor) were pretreated before gentamicin treatment, but did not have effect. For U73122, a phospholipase C (PLC) inhibitor however, the inhibitory effect to gentamicin was significantly attenuated in all frequencies given by the EFS. Therefore gentamicin induced inhibitory effect on EFS response in rat bladder smooth muscle was not mediated by the activation of adrenergic, cholinergic, or serotonergic receptor. The inhibition of gentamicin might be mediated through the PLC dependent pathway, but not through the PKC, MLCK or rho kinase dependent pathway.

Factors Correlated with the Reducibility of Varus Deformity in Knee Osteoarthritis: An Analysis Using Navigation Guided TKA

BACKGROUND: We hypothesized that a number of clinical and radiologic parameters could influence the reducibility of varus deformity in total knee arthroplasty. The aim of this study was to identify the factors correlated with reducibility of varus deformity and predict more accurately the amount of medial soft tissue release required in varus deformity total knee arthroplasty. METHODS: One hundred forty-three knees with preoperative varus alignment and medial osteoarthritis were included in this retrospective study. The total knee arthroplasties were performed using a navigation system (OrthoPilot) by single surgeon. To assess varus deformity, the authors measured preoperative mechanical axis angles and valgus stress angles. Mechanical tibial angles, mechanical femoral angles, femoral osteophyte sizes, and tibial osteophyte sizes were measured. The Ahlback grading scale was applied for radiologic parameters, and clinical parameters (age, body mass index, sex, duration of pain, and preoperative range of motion) were documented. Correlations between these factors and preoperative valgus stress angle were analyzed. RESULTS: A negative correlation was found between preoperative mechanical axis angle and preoperative valgus stress angle (p < 0.01, r = -0.38), and a positive correlation was found between the preoperative mechanical tibial angle and preoperative valgus stress angle (p = 0.01, r = 0.19). CONCLUSIONS: The present study shows that preoperative varus deformity and proximal tibial vara (measured by preoperative mechanical axis angle and mechanical tibial angle, respectively) are correlated with reducibility of varus deformity (measured by preoperative valgus stress angle), and clinical parameters (age, range of motion, duration of pain and body mass index) and other radiologic parameters (osteophyte size, severity of osteoarthritis and angulation of distal femoral joint surface) were not significantly correlated with reducibility of varus deformity.

The Association of Gait Disturbance and Cognitive Impairment

BACKGROUND: The incidence and prevalence of gait disturbance increases as age increases. The importance of cognitive aspects of gait disturbances has been studied in various types of dementia and MCI. This study was performed to identify relationship between cognitive impairment and change in gait, and investigate specific domains of cognitive function that may have affects in gait disturbance. METHODS: Three hundred eighty three participants over 60 years old volunteered for the study. Three stages evaluation were performed, dementia screening and timed up and go test (TUG), work up for cognitive impairment and dementia, and classifying cognitive impairment and dementia into subcategories by evaluating cause of the disease. To evaluate cognitive impairment, MMSE-KC (MMSE in the Korean version of the CERAD assessment packet) and Korean version of Consortium to Establish a Registry of Alzheimer's Disease (CERAD-K) assessment were used. One hundred forty one people were excluded from study and 242 people were analyzed. Timed Up and Go test (TUG) was performed for evaluation of gait disturbance. Relationship in every subcategory of CERAD-K and TUG was studied by correlation and multiple logistic analysis. RESULTS: Of the 242 participants, 122 were without cognitive impairment, 51 had mild cognitive impairment, 56 had Alzheimer's disease and 13 had vascular dementia. These four groups showed different results in TUG. Normal group had the lowest values in TUG compare to other groups significantly. MMSE score also correlated with the value of TUG (r=-0.528, p<0.001). Every subcategory of CERAD-K score had significant correlation with the value of TUG. But there were no specific subcategory that had predominant effect in TUG. Only age was an independently significant factor influencing TUG (p<0.05). CONCLUSIONS: This study shows that increase in age and impairment in cognitive function has an association with gait disturbance. Therefore clinician should be concerned about cognitive factors for evaluation of a patient who has gait disturbance.

Ultrastructure of Rapidly Proliferating Preretinal Membrane of Very Extensive Ischemic Diabetic Retinopathy

PURPOSE: Using transmission electron microscopy (TEM), we studied the ultrastructures of rapidly proliferating preretinal membranes of young patients with very extensive ischemic proliferative diabetic retinopathy and diabetes with uncontrollable blood sugar level. METHODS: Nine cases of preretinal membranes were obtained from six eyes of five patients with rapidly progressed proliferative diabetic retinopathy (mean age, 35 years) during vitrectomy. We obtained each preretinal membrane bimanually as one single sheet membrane using intraocular scissors and forceps. Each tissue was fixed in 3% glutaraldehyde in the operating room. All specimens were prepared and studied using TEM. RESULTS: The preretinal membranes were composed of blood vessels and some interstitial cells. The blood vessels within the preretinal membranes varied in developmental stages, from the immature stage to the mature stage. The blood vessels were highly active, in that primitive cells showed a large nucleus and prominent chromatin clumping with abundant cytoplasm. Highly active fibroblast-like cells were also noted. CONCLUSIONS: We observed highly active angiogenesis in preretinal membranes, which rapidly proliferated in cases of severe retinal ischemia in young diabetes patients. This is the first report of such a finding, which may help to explain the poor prognosis of this disease modality.

Usefulness of Frontal Sinus Reconstruction Using Polymethyl-methacrylate

OBJECTIVE: Many methods for preventing central nervous system infection and cerebrospinal fluid leakage through opened frontal sinus have been reported. Nevertherless, the most effective procedure was not confirmed yet. The aim of this study is to test the efficacy of a simple technique of frontal sinus obliteration using polymethyl-methacrylate(PMMA) cement instead of more traditional methods such as pericranial flap, free muscle or fat graft and lumbar drainage. METHODS: Twenty three patients whose frontal sinus were opened during frontal craniotomy or due to trauma underwent frontal sinus obliteration procedure using PMMA bone cement. No other adjuncts for prevention of cerebrospinal fluid leakage through the sinus were used. Demographics, indications for frontal sinus obliteration, immediate and late complications, and long term outcomes were investigated. These results were compared with those in the current literatures. RESULTS: During follow-up of 18 months, there were no cerebrospinal fluid leaks, infection, resorption, cosmetic deformity, and morbidity of donor site CONCLUSION: Polymethyl-methacrylate bone cement seems be a simple and effective method for frontal sinus obliteration and prevention of cerebrospinal fluid leakage.

Ultrastructure of Corneal Epithelial Cells Foll owing Inoculation of Herpes Simplex Virus in Rabbit

This study was performed to investigate the ultrastructural changes of the infected corneal epithelial cell by herpes simplex virus in rabbit eyes. Herpes simplex virus(type 1, Kos strain)was inoculated on the injured cornea in 5 rabbit. The eyeballs, which was enucleated on days 3, 5, 7, 10 and 14, respectively following the inoculation of herpes simplex virus was examined by the transmission electron microscopy. The intercellular space of the infected corneal epithelial cells were widened with the loss of the desmosomal attachment, followed by the detachment of the adjacent epithelial cell. The nuclear change was characterized by the chromatin clumping, intranuclear inclusion body and virus particles within the nucleus. The basal epithelial cells were detached from the corneal stroma with the relatively well preserved desmosomal attachment between the adjacent epithelial cell. These findings suggest that the intercellular space of the infected corneal epithelial cells was widened, followed by the loss of the epithelial cells. Around 2 weeks following the inoculation of herpes simplex virus, the remodelling of both the corneal epithelial cell-cell and epithelial cell-stromal interface was nearly completed.

Amblyopia and Sensory Fusional Anomaly in Intermittent Exotropia

This study is designed to determine the frequency of amblyopia and sensory fusional anomaly in intermittent exotropia and the relationship between amblyopia and sensory fusional anomaly. Ninety eight intermittent exotropic patients were selected and using full corrected visual acuity, the selected patients were divided into amblyopia group and non-amblyopia group. Then, refractive errors, Bagolini lens test, Worth 4 dot test and stereoacuity test were measured and these values were statistically analyzed. The frequency of amblyopia among intermittent exotropia group was 14 patients[14.3%]. Anisometropia among amblyopia group was 6 patients[43.9%] and among non-amblyopia group was 6 patients [7.1%]. Anisometropia was statistically meaningful in amblyopia group compared with non-amblyopia group[p0.064]. In stereoacuity, it decreased statistically meaningful when amblyopia[p<0.003] or sensory fusional anomaly[p<0.019]were present.

Ocular Dominance Determined by Near Point of Convergence Test in Intermittent Exotropia

This study was designed to validate the usefulness of the near point of convergence(NPC)test in determination of dominant & non-domnant eyes in intermittent exotropia patients. We performed NPC test in 36 intermittent exotrpic patients, and then, determined the non-dominant eye which deviated outward beyond the NPC. The dominant eyes determined by the NPC were compared to those that were determined by amblyopia, sensory fusion and photophobia. Best corrected visual acuity was used to divide patients into two groups : 26 patients without amblyopia, and 10 with amblyopia. Among the amblyopia group, if the eyes with better visual acuity were taken as dominant eyes, then eight(80%)cases were identical with the NPC test and two(20%)cases were different. Ten patients had sensory anomaly, and among then, nine(90%)cases were identical with the NPC test and one(10%)case was different. Twenty six(64%)patients had photophobia in one eye and all(100%)cases were identical with the NPC test. In intermittent exotropia, determination of dominant eye by the NPC test showed high coincidence with other tests. Therefore, we suggest that the NPC test is an easy and accurate method in determining either the dominant or non-dominant eye for surgery.

Three Cases of Cerebellar Hemangioblastoma in a von Hippel-Lindau Family: Case Report

Hemangioblastoma is one of the benign tumors in the central nervous system. It is often associated with von Hippel-Lindau disease(VHL disease), a well known autosomal dominant hereditary tumor syndrome. We have experienced three cases of hemangioblastoma associated with von Hippel-Lindau disease in a same family, mother(case 1, 58 years old), son(case 2,19 years old, dead) and daughter(case 3, 17 years old). All of them had cerebellar hemangioblastomas. Renal cyst was associated in the case of mother, but not in the son or daughter. Hemangioblastoma associated with von Hippel-Lindau disease frequently occurres in multiple regions such as cerebellum, spinal cord, medulla oblongata. Also it is frequently presented with renal cell carcinoma, pheochromocytoma, cyst in pancreas, kidney and liver, and epididymal cystadenoma. The age of onset of von Hippel-Lindau disease in the second generation was younger than that of the first generation in this family.

Microvascular Decompression for Essential Hypertension

Idiopathic arterial hypertension, termed "essential" or "neurogenic", is a common generalized cardiovascular syndrome comprised of a sequence of pathologic changes and accommodations. Although an extensive literature exists concerning that, the primary etiology has been unclear yet. However, Jannetta and coworkers have reported a possible etiological connection between essential hypertension and intraoperatively observed neurovascular compression of the ventrolateral medulla at the level of the root entry zone of the ninth and tenth cranial nerves on the left, recently. They have also introduced one of new therapeutical concept for essential hypertension by microvascular decompression(MVD) of offending vessels. Based on Jannetta and coworker's hypothesis, the authors have made some operations for the control of hypertension. Seven essential hypertensive patients have been underwent retromastoid craniectomy and MVD in the left ventrolateral medulla between July 1992 and June 1993. Five of them showed multiple episodes of intracerebral hemorrhages, one was an intractable hypertension case and the other one had a left hemifacial spasm with essential hypertension. The most common offending vessel was the posterior inferior cerebellar artery and it had been confirmed during operation. Postoperatively, in three cases, blood pressure was lowered to normal without medications. Of remaining four cases, blood pressure was significantly improved in one and slightly improved in three. There were no major complications in patients with surgery and no poor outcomes. These results indicate that the MVD for essential hypertension is relatively safe procedure and alternative choice as one of the management of intractable essential hypertension, repeated hypertensive intracerebral hemorrhages and left cranial nerve hyperfunction disorders combined with essential hypertension.