Canavan disease(CD) is a rare autosomal recessive leukodystrophy caused by the deficiency of aspartoacylase and the accumulation in brain of N-acetylaspartate(NAA). CD has been reported mainly Ashkenazi Jews but also occurs in other ethnic groups. Usually it presents as early as the third month of life with megalencephaly, hypotonia later progressing to hypertonia, psychomotor and mental retardation, blindness, occasionally deafness and seizure. Diagnosis is based on the clinical feature, N-acetylaspartic aciduria, radiologic and pathologic findings. Histologically, the affected white matter shows extensive vacuolation and demyelination. There is no treatment for CD and the only prevention is through genetic counselling and prenatal diagnosis. We experienced a case of Canavan disease that was presented with hypotonia and developmental delay. Diagnosis was confirmed histologically. Radiologic findings are extensive high signal throughout the white matter on T2-weighted MRI and increased NAA peak and decreased choline peak of the white matter on MR spectroscopy.
Blindness ; Brain ; Canavan Disease* ; Choline ; Deafness ; Demyelinating Diseases ; Diagnosis ; Ethnic Groups ; Humans ; Intellectual Disability ; Jews ; Magnetic Resonance Imaging ; Magnetic Resonance Spectroscopy ; Muscle Hypotonia ; Prenatal Diagnosis ; Seizures

A rostro-caudal topography of the oculomotor nerve fascicles is relatively well known in animal but not in human. We experienced two cases of midbrain infarctions presenting with isolated oculomotor nerve palsies. The MRI of the patient who had monocular elevation palsy with ptosis revealed a lesion in the middle to lower midbrain, whereas that of the other who had monocular depression and adduction palsy with pupillary mydriasis showed a discrete lesion in the upper to middle midbrain. We present the rostro-caudal arrangement of the oculomotor fascicles within midbrain based on MRI findings.
Animals ; Depression ; Humans ; Infarction* ; Magnetic Resonance Imaging ; Mesencephalon* ; Mydriasis ; Oculomotor Nerve ; Oculomotor Nerve Diseases ; Paralysis

Cancer of the gastric stump, first described by Balfour in 1922, is defined as the cancer detected more than 5 years after surgery for a benign disease. We experienced a case of cancer found at the gastric stump after gastrojejunostomy in a 53 years old male patients, proven pathologically as a early cancer. He visited to our hospital with the chief complaint of epigastric pain and indigestion for 1 Months. On past history, he has been received gastrojejunostomy due to duodenal ulcer obstruction, 23 years ago, Gastrofiberscopy was done, and we could find the early gastric cancer lesions at the anterior wall of gastric angle as type Ilc+III and antrum as type IIa. The microscopic finding of the multiple endoscopic biopsies at the gastic angle and antrum revealed the adenocarcinoma of signet ring cell type infiltrated to the level of submucosa. And so, we could diagnose these lesions as a early gastric cancer in the gastric stump after gastrojejunostomy. He was treated with subtotal gastrectomy and discharged with cured condition. Therefore, we report this case with a literature review.
Adenocarcinoma ; Biopsy ; Duodenal Ulcer* ; Dyspepsia ; Gastrectomy ; Gastric Bypass* ; Gastric Stump* ; Humans ; Male ; Middle Aged ; Stomach Neoplasms

No abstract available.
Blood Banks*

BACKGROUND AND PURPOSE: The oculomotor nerve fascicles arise along its entire length and sweep ventrally to exit the midbrain at the medial edge of the crus cerebri. A rostro-caudal topography among the fascicular fibers is relatively well established. There are, however, some controversies whether medio-lateral topography also exists. METHODS: We retrospectively reviewed the clinical records and MRI of the 8 patients showing isolated oculomotor nerve palsy due to midbrain infarction. Brain MRI was performed using a 1.5-T magnet with 2mm thickness and 0.1 mm slice interval. The anterior-posterior axis(X) was defined as the midline crossing the center of the cerebral aqueduct and the medio-lateral axis(Y) as the line crossing the same point. For rostro-caudal measurement, the intercommissural line was used as base line of the Z axis. The location of the lesions was defined by measuring actual distance of the margins of the lesions in millimeter from each axis; anterior, right, and caudal direction was defined as positive values in X, Y and Z coordinates, respectively. RESULTS: The mean values and range of the X, Y and Z are as follows: X=7.56+/-4.34, 1< or =X< or =15; Y=3.43+/-1.37, 0< or =Y< or =6; Z=6.51+/-3.91, 0< or =Z< or =12.5. CONCLUSIONS: The distribution of all the MRI lesions was 0< or =|Y|< or =6 (mm), 0< or =|Z|< or =12.5 (mm) in mediolateral and rostrocaudal direction respectively, which is almost the same as the previously reported divergent range of the oculomotor fascicles in midbrain tegmentum. We suggest that our method of three dimensional measurements of the MRI lesion in midbrain tegmentum could be a useful tool for the study of oculomotor fascicular arrangement.
Axis, Cervical Vertebra ; Brain ; Cerebral Aqueduct ; Humans ; Infarction ; Magnets ; Mesencephalon ; Oculomotor Nerve ; Oculomotor Nerve Diseases ; Retrospective Studies

The study was the clinical analysis of 509 patients with pure ocular injuries who visited to Chonnam University Hospital Emergency center from July 1, 1996 to June 30, 1997 retrospectively. Clinical data survey including sex and age distribution, causes of trauma, injury site, ocular disease, and surgical intervention was done. Most of ocular injury patients were male. 3rd and 4th decade who were socially active were nearly 50%. Direct and indirect injury from foreign body was the most common cause of ocular injuries followed by fist blow, traffic accident, falling down, and sport injury. Cornea was the most common ocular injury site (209 patients, 41.1%). The incidence of the traumatic ocular disease showed corneal erosion (117 patients, 22.9%) and then eyeball perforation, traumatic hyphema etc. by frequency of order. Eyeball perforation was the most common ocular injury which needed a emergency surgical intervention. As a results, emergency physicians have to pay attention to the cornea in case of any type of ocular injuries and eyeball perforation which caused by direct and indirect injury from foreign body.
Accidents, Traffic ; Age Distribution ; Cornea ; Emergencies ; Foreign Bodies ; Humans ; Hyphema ; Incidence ; Jeollanam-do ; Male ; Retrospective Studies ; Sports

Our purpose was to study the correlation between elevated maternal serum alpha-fetoprotein(MSAFP) or human chorionic gonadotropin(HCG) levels and pregnancy-induced hypertension (PIH), preeclampsia, preterm delivery. MSAFP and HCG levels were measured in stored second-trimester(14~22 weeks) serum obtained from 510 women. The criteria for patients with unexplained MSAFP elevations were a MSAFP level 2.5 or greater multiples of the median(MoM) and HCG elevations were a HCG level 2.0 or greater multiples of the median(MoM) excluding multiple pregnancy, fetal malformation or death on ultra-sonography and molar pregnancy. In contrast, patients with MSAFP levels 0.5 to < 2.5 MoM, HCG levels < 2.0 MoM were served as controls. Women with elevated HCG levels had more significant association with PIH than control group(22.2 % versus 3.4 % ; p < .005). Elevated MSAFP was significantly associated with preeclampsia(7.7 % versus 1.1 % ; p < .05). But no significant differences were observed in the incidence of preterm delivery. We suggested elevated second-trimester MSAFP or HCG levels appear to be correlated with high risk for PIH and preeclampsia. So, These patients require careful monitoring with adequate obstetric managements.
alpha-Fetoproteins* ; Chorion ; Chorionic Gonadotropin* ; Female ; Humans* ; Hydatidiform Mole ; Hypertension, Pregnancy-Induced ; Incidence ; Pre-Eclampsia ; Pregnancy ; Pregnancy, Multiple

OBJECTIVE: Sensory symptoms are common in patients with Parkinson disease (PD), although its exact prevalence and causes are not well known. We aimed to investigate whether peripheral pathology contribute to chronic sensory symptoms in patients with PD by nerve conduction test. METHODS: We recruited consecutively 34 patients with clinically probable PD (Hoehn-Yahr stage, 1–3) who developed persistent sensory symptoms in their lower extremities during optimal dopamine replacement therapy. Twenty-five patients who are above age of 70 or have other possible causes of neuropathy as determined by laboratory testing or medical record were excluded. Age- and gender-matched PD patients with same disease severity who have never developed sensory symptom during the same period were allocated to paired controls. Total 18 subjects participated in this study. Nerve conduction tests on peroneal motor nerve and sural sensory nerve were performed in all subjects. Amplitude and conduction velocity were compared between the paired subjects using Wilcoxon signed rank test. RESULTS: Subjective sensory symptoms of the 9 patients were variable: pain (4/9), chilling (3/9), burning (1/9), and tingling sense (1/9). There were no significant difference of mean values of compound muscle action potential/sensory nerve action potential amplitude and conduction velocity between patients and their paired controls. CONCLUSION: This study supports the hypothesis that the origin of chronic sensory symptoms in PD patients is more central than peripheral.
Action Potentials ; Burns ; Dopamine ; Humans ; Levodopa* ; Lower Extremity ; Medical Records ; Neural Conduction* ; Parkinson Disease* ; Pathology ; Prevalence

PURPOSE: Although breast milk is considered the best source of nutrition for infants because of its superiority over powdered milk, there are circumstances when breast milk is unavailable. Committee on Nutrition of the Korean Pediatric Society conducted an investigation into the current status of formula feeding in Korea. METHODS: This investigation was conducted using questionnaires produced by our committee and distributed to mothers of children under the age of two who were using formula feeding at 10 university hospitals and 22 public health centers nationwide between March 1999 to September 1999. A comparative analysis according to location, birth order, mother's occupation, education, housing tenure and normal birth status was performed on all 2696 questionnaires using the chi-square test. RESULTS: Formula feeding was found in 49.4% and formula feeding combined with breast milk were 21.5% of the respondents. The percentage of formula feeding was higher in working mothers who underwent a Cesarean section in metropolitan areas. The inadequacy of breast milk was the most common reason for feeding powdered rnilk followed by the unhealthy condition of the mother and mother's return to work. Of those investigated, 94.9% said that they changed products at each stage as recommended by the manufacturer. Promotions for special formulas were more active in metropolitan areas than other areas, and 57.7M said that they used special formulas based on recornmendations by the doctor and acquaintances(15.5%) or after seeing an advertisement(14.0%). CONCLUSION: This investigation revealed that many rnothers were misinformed concerning formula feeding and pediatricians should provide mothers with education, shedding light on proper feeding methods.
Birth Order ; Cesarean Section ; Child ; Surveys and Questionnaires ; Education ; Feeding Methods ; Female ; Hospitals, University ; Housing ; Humans ; Infant* ; Korea ; Milk ; Milk, Human ; Mothers ; Occupations ; Parturition ; Pregnancy ; Public Health ; Return to Work

BACKGROUND: Cardiovascular autonomic dysfunction is one of the most frequent non-motor symptoms in idiopathic Parkinson disease (IPD). Several cardiovascular autonomic indexes (CAIs) have been reported to represent the degree of autonomic dysfunction in various neurodegenerative diseases. However, quantitative assessment by autonomic function tests in IPD has not been fully evaluated yet. The aim of this study is to investigate the usefulness of the quantitative autonomic test for detecting subclinical cardiovascular autonomic dysfunction and their correlation to the clinical severity of motor symptoms in IPD. METHODS: Four parasympathetic and sympathetic indexes during cardiovascular autonomic tests were compared between patients with IPD (n=31, age=65.8+/-9.14, Hoen&Yahr (H&Y) stage=2.1+/-0.68) and age matched healthy controls (n=30, age=63.2+/-7.56). Parasympathetic indexes include expiration:inspiration ratio (E:I ratio), valsalva ratio, 30:15 ratio, and vagal barosensitivity. Sympathetic indexes are pressure recovery time, sympathetic index 1, sympathetic index 3 and adrenergic baroseneitivity. To demonstrate the correlation between severity of clinical motor symptoms and the autonomic abnormality, we also compared the H&Y stage and the abnormalities of those CAIs. RESULTS: E:I ratio (p=0.009) and Valsalva ratio (VR) (p<0.001) were significantly different between IPD and control groups. Among the parameters, E:I ratio (r=-0.466, p=0.005) showed significant negative correlation with severity of clinical motor symptoms in IPD (H&Y< or =3). CONCLUSIONS: Among the CAIs, E:I ratio, VR are useful in detecting subclinical autonomic cardiovascular dysfunction in IPD. E:I ratio may be the possible evaluation method revealing the severity of clinical motor symptoms in IPD.
Humans ; Neurodegenerative Diseases ; Parkinson Disease*