We report 2 cases of pediatric DNTs which presented with intractable seizures and no other associated neurologic abnormalities. They showed typical appearance of DNTs on neuroimaging and histopathology. Most patients with DNT can be cured by surgical treatment with exellent outcome and do not need ratio- or chemotheraphy. This study indicates that it is quite important to consider DNTs as one of differential diagnoses in patients with intractable seizures especially when they present only with seizures without other neurologic symptoms.
Child* ; Diagnosis, Differential ; Humans ; Neoplasms, Neuroepithelial* ; Neuroimaging ; Neurologic Manifestations ; Seizures*

Menkes disease is an X-linked recessively inherited neurodegenerative disorder of copper metabolism leading to death in early childhood. This disease is characterized by low serum, liver and brain Cu levels, whereas the Cu content in nearly all other organs is increased. The main clinical features are irregular kinky hair, characteristic face, progresssive degeneration of the central nervous system, bone changes, arterial rupture and thrombosis, and instability of temperature. We have experienced a case of Menkes disease in a eight months old male patient, who manifested by seizure, developmental delay, hypotonia, characteristic hair and face along with low levels of serum copper and ceruloplasmin. We report the characteristic findings of cranial MRI and MR angiography in this Menke's patient. In cranial MRI, he showed progressive brain atrophy with subdural hemorrhage and effusion. MR angiography revealed tortuosity of vessels clearly obviating necessity of more invasive conventional arteriography in diagnosis of this disease. This study suggested that cranial MRI and especially MR angiography might be helpful in making early diagnosis of this disease.
Angiography ; Atrophy ; Brain ; Central Nervous System ; Ceruloplasmin ; Copper ; Diagnosis ; Early Diagnosis ; Hair ; Hematoma, Subdural ; Humans ; Liver ; Magnetic Resonance Imaging ; Male ; Menkes Kinky Hair Syndrome ; Metabolism ; Muscle Hypotonia ; Neurodegenerative Diseases ; Rupture ; Seizures ; Thrombosis

Heat shock protein of 72 kDa (HSP70) has a role in the functional modulation of sex steroid hormone receptors and in p53-associated oncogenesis and inhibits apoptosis associated with bcl-2. However, the exact role of HSP70 in the development of endometrial adenocarcinoma has not been well established. The aim of this study is to evaluate the role of HSP70 in relation with ER, PR, p53 and bcl-2 expressions and apoptosis in benign and malignant endometrial lesions. Immunohistochemical studies for HSP70, ER, PR, p53, bcl-2 and TUNEL method for apoptosis were performed in 30 cases of adenocarcinoma and 30 cases of benign endometrial lesions consisted of each 10 cases of disordered proliferative endometrium (DP), simple or complex hyperplasia (HP), and atypical hyperplasia (AH). There were no significant differences of HSP70 and bcl-2 expression rates and apoptotic index (AI) between DP, HP, AH, and adenocarcinoma. p53 expression rate in adenocarcinoma was 36.7%, but no p53 expression was identified in DP, HP and AH (p<0.05). In adenocarcinoma, HSP70 expression rate was higher in ER and PR negative adenocarcinoma (p<0.05), and p53 expression rate was higher in nonendometrioid type and FIGO grade II and III (p<0.05), but no significant difference of bcl-2 expression rate according to the histological type and FIGO grade. AI was higher in nonendometrioid type (p<0.05). There was no correlation between HSP70, p53 and bcl-2 expressions, and no significant difference of AI according to HSP70, ER, PR, p53, and bcl-2 expressions. In conclusion, higher HSP70 expression rate in poorly differentiated and ER and PR negative adenocarcinoma suggests that HSP70 inhibits ER and PR expression and may be involved in the development of poorly differentiated endometrial adenocarcinoma.
Adenocarcinoma* ; Apoptosis* ; Carcinogenesis ; Endometrium ; Female ; Heat-Shock Proteins ; Hyperplasia ; In Situ Nick-End Labeling

No abstract available.
Female ; Fetomaternal Transfusion* ; Pregnancy

Cushing’s syndrome is an endocrinologic disorder that occurs due to an excessive secretion of adrenal cortex-stimulating hormones or an overproduction of cortisol in the adrenal glands. Patients present with a variety of clinical symptoms such as obesity, hypertension, impaired glucose tolerance, and cardiovascular complications have been known for major contributors to the mortality. The inferior petrosal sinus sampling (IPSS) test is a useful diagnostic tool for identifying the source of Cushing’s disease. This procedure involves process of collecting samples through venous access and is generally considered safe. Here, we present 44-year-old patient diagnosed with Cushing’s disease presenting pulmonary thromboembolism and multiple embolic infarctions following IPSS.

OBJECTIVE: To identify factors affecting test results of the quiet standing balance evaluation conducted by posturography and to investigate the standardized method by comparing results according to feet width. METHOD: The study cohort consisted of 100 healthy individuals. We assessed the quiet standing balance of subjects by using 3 different methods: standing on a force plate with feet width the same as shoulder width (test 1); with feet width the same as half the shoulder width (test 2); with feet width determined by the subject's comfort (test 3). Subjects underwent each test with their eyes open and closed for 30 seconds each time. Parameters for measuring standing balance included the mean mediolateral and anteroposterior extent, speed, and the velocity moment of center of pressure (COP) movement. RESULTS: All parameters showed better results when the subject's eyes were open rather than closed, and the mean AP extent and speed increased as the age of the subjects increased (p<0.01). However, there was no significant correlation between height and the study parameters, and no differences between men and women. Mean mediolateral extent and speed were significantly longer and faster in test 1 compared with tests 2 and 3 (p<0.01). The results of test 2 were better than the results of test 3, but the difference was not statistically significant. CONCLUSION: COP movements increased with age and when subjects closed their eyes in an evaluation of quiet standing balance conducted by posturography. Gender and height did not affect results of the test. We suggest that an appropriate method for conducting posturography is to have the subject stand on a force plate with their feet width the same as half the shoulder width, because this posture provided relatively accurate balance capacity.
Cohort Studies ; Eye ; Female ; Foot ; Humans ; Male ; Posture ; Shoulder

The meninges composed of the dura, the arachnoid and the pia are significant sites of blood-brain barrier. Physical disruption of the integrity of the meninges from avariety of causes including surgery results in various patterns of meningeal enhancement on contrast enhanced MR images. It is important to distinguish normal reactive or benign postoperative enhancement from more serious leptomeningeal metastasis or infection, particularly in children with intracranial neoplasms. We present various pat-terns of meningeal enhancement on MRI in children following surgery for brain neop lasms.
Arachnoid ; Blood-Brain Barrier ; Brain Neoplasms* ; Brain* ; Child* ; Humans ; Magnetic Resonance Imaging* ; Meninges ; Neoplasm Metastasis

PURPOSE: To evaluate the CT findings in patients with Langerhans cell histiocytosis (LCH) involving the orbit. MATERIALS AND METHODS: Orbital CT scans of six children with pathologically proven LCH were retrospectively analyzed. Follow-up CT (n = 5) and MR (n = 1) imaging findings were also revi ewed. RESULTS: Initial CT scans revealed varying degree of bone destruction with soft-tissue masses, and on nonenhanced images the mean attenuation value was 44 Hounsfield units(HU). All masses showed mild to moderate enhancement with a mean attenuation value of 74 HU. The bony margins abutting onto soft tissue masses were irregular but clearly demarcated. No evidence of calcification or periosteal reaction was noted. Suprasellar mass and rib involvement was noted in one patient and hepatos-plenomegaly in two. Follow-up CT and MR images showed that the soft tissue masses were almost completely resolve d, with bone remodeling and reossification. CONCLUSION: A soft tissue mass with irregular but clearly demarcated bone destruction is thought to be a characteristic finding of LCH involving the orbit. Follow-up images after treatment showed bone remodeling and reossification.
Bone Remodeling ; Child ; Follow-Up Studies ; Histiocytosis ; Histiocytosis, Langerhans-Cell* ; Humans ; Orbit* ; Retrospective Studies ; Ribs ; Tomography, X-Ray Computed

PURPOSE: The purpose of this study is to describe the rib changes seen in patients with bronchopulmonary dysplasia (BPD). MATERIALS AND METHODS: Serial chest radiographs of nine premature infants with BPD who showed diffuse rib enlargement were reviewed for hyperinflation, which was compared with the observed degree of rib enlargement. Vibrator chest physiotherapy was performed in all cases, and five infants underwent conventional ventilation plus high frequency oscillatory ventilation therapy. Their calcium level was normal where-as alkaline phosphatase and phosphate levels were high. In all infants except one, liver enzyme levels were normal. For the treatment of patent ductus arteriosus, infection, and BPD, medications including indomethacin, antibiotics, and examethasone were administered. Vitamin D was given to all patients with total parenteral nutrition. RESULTS: Rib enlargement was found to be severe (n=4), moderate (n=3), or mild (n=2) with undulating margins or posterior tapering (n=2). Hyperinflation was noted in eight patients, in seven of whom it was moderate to seve r e. Among these seven, rib enlargement was severe (n=2), moderate (n=3), or mild (n=2). In one infant with mild hyperinflation, rib enlargement was seve r e. Bilateral irregular infiltrates and at-electases were noted in all patients. CONCLUSION: In BPD patients, rib enlargement may be seen. In order to differentiate this process from systemic bone disease or bony dysplasia, an awareness of the rib changes occurring in patients with BPD may be important.
Alkaline Phosphatase ; Anti-Bacterial Agents ; Bone Diseases ; Bronchopulmonary Dysplasia ; Calcium ; Ductus Arteriosus, Patent ; Humans ; Indomethacin ; Infant ; Infant, Newborn ; Infant, Premature* ; Liver ; Parenteral Nutrition, Total ; Radiography, Thoracic ; Ribs* ; Thorax ; Ventilation ; Vitamin D

PURPOSE: To investigate the degree of reunion in rabbit eyes of the superior oblique tendon after several surgical weakening procedures. METHODS: A total of 32 rabbits (64 eyes) were used in this study. The rabbits were randomly assigned to four groups, eight rabbits (16 eyes) in the tenotomy group, eight rabbits (16 eyes) in the tenectomy group, eight rabbits (16 eyes) in the disinsertion group and eight rabbits (16 eyes) in the recession group. The degree of reunion or reattachment of the superior oblique tendon on the globe were examined on four eyes in each group at postoperative weeks two, four, six and eight. RESULTS: At eight weeks, the newly created insertion site remained at the same site in all eyes in the recession group, and the distal end of the superior oblique tendon was reattached at the medial border of the superior rectus muscle in all four eyes in the tenotomy and disinsertion groups, and in three of four eyes in the tenectomy group. CONCLUSIONS: From this experimental study, it was speculated that superior oblique recession is more effective than other superior oblique weakening procedures. This result could be helpful in the prediction of time of recurrence for superior oblique overaction after superior oblique weakening procedures.
Animals ; Oculomotor Muscles/*physiopathology/*surgery ; *Ophthalmologic Surgical Procedures ; Prostheses and Implants ; Rabbits ; Silicones ; Tendons/*physiopathology/*surgery ; Wound Healing