Ganglioneruroblastoma and neuroblastoma are among commonest types of childhood malignancy and a number of unique paraneoplastic syndromes have associated with both localized and disseminated neuroblastoma. The coincidence of neuroblastoma and myoclonic encephalopathy or other paraneoplastic syndromes occurs relatively rare, and therefore, failure to recognize this association could result in delays in both diagnosis and treatment, and the result could prove to be unfortunately fatal. The mechanism which underlies the remote damaging effect of neural crest tumor, especially neuroblastoma, on the nervous system resulting in myoclonic encephalopathy is by no means clear. In addition the nature and the extent of the pathologic lesion are inconsistent. We experienced a case of myoclonic encephalopathy associated with an occult mediastinal ganglioneuroblastoma in a 22-month-old girl who was hospitalized for inability to walk without support and tilting of the head to the left side. She became increasingly ataxic, and during the hospitalization myoclonic jerks of upper extremities and head along with chaotic, rapidly flickering, multidirectional spontaneous eye movements, were noted. Laboratory data included normal complete blood count, urinalysis, BUN and creatinine, electrolytes and bone marrow. Chest X-ray and chest CT revealed a relatively well marginated right posterior mediastinal mass. In a 24 hours urine excretion test, VMA and catecholamines were increased. Over the next 2 weeks, a surgical exploration revealed a right posterior mediastinal mass. Microscopically the mass proved to be a ganglioneuroblastoma, extending to right innominate artery and right axillary lymph nodes. Within 2 weeks after the surgery, radiotherapy (2,400 rads) and chemotherapy (CTX, DTIC, VCR) were started, but corticosteroid was not used. She has been free of tumor and abnormal neurological systemic symptoms and signs for 1 1/2 year since the completion of chemotherapy. In the 3 1/2 years follow-up period, her neurologic symptoms has completely resolved by the completion of 2 years chemotherapy. We report a case of mycoclonic encephalopathy associated with hidden ganglioneuroblastoma in 22-month-old girl.
Blood Cell Count ; Bone Marrow ; Brachiocephalic Trunk ; Catecholamines ; Creatinine ; Dacarbazine ; Diagnosis ; Drug Therapy ; Electrolytes ; Epilepsies, Myoclonic* ; Eye Movements ; Female ; Follow-Up Studies ; Ganglioneuroblastoma* ; Head ; Hospitalization ; Humans ; Infant ; Lymph Nodes ; Myoclonus ; Nervous System ; Neural Crest ; Neuroblastoma ; Neurologic Manifestations ; Paraneoplastic Syndromes* ; Radiotherapy ; Thorax ; Tomography, X-Ray Computed ; Upper Extremity ; Urinalysis

PURPOSE: This is a retrospective study to analyze the functional results of closed reduction and external fixation of unstable fractures of the proximal humerus. MATERIALS AND METHODS: Ten unstable proximal humerus fractures were managed with closed reduction and external fixation in which other operative methods are not proper due to comminution, osteoporosis or poor general condition of patients. 4 cases of 2-part and 6 cases of 3-part fracture were included. Radiologically union of fracture, malunion and the evdence of avascular necrosis of humeral head were assessed and the functional results were analyzed with Neer scoring system. RESULTS: Radiologically all fractures were healed but in 2 cases malunion was resulted because of reduction loss in proximal fragment. Pin site infection was developed in 7 cases and oral antibiotics were needed. The functional results were excellent in 4, satisfactory in 3 and unsatisfactory in 3 cases. 2 cases with malunion and one case with lack of postoperative cooperation resulted in functionally unsatisfactory. CONCLUSION: External fixation is an alternative method in the treatment of unstable proximal humerus fractures in which open reduction or percutaneous pinning are not proper due to comminution, osteoporosis or poor general condition of patient.
Anti-Bacterial Agents ; Humans ; Humeral Head ; Humerus* ; Necrosis ; Osteoporosis ; Retrospective Studies

No abstract available.
Lasers, Dye* ; Melanosis*

Hemichorea is usually caused by lesions in the contralateral subthalamus and basal ganglia. Ipsilateral lesions have rarely been reported to be responsible for the abnormal movement. A 27 year-old woman with well-controlled hyper-thyroidism presented with sudden involuntary movements in the right limbs and a mild headache. The movements were random, irregular, repetitive, and most prominent in the right hand and forearm, but also found in the right leg and face. She experienced no weaknesses in the contralateral limbs. A brain magnetic resonance imaging(MRI) taken after 7 days showed early subacute hematoma in the right basal ganglia. There were no lesions in the left hemisphere. In a cerebral angiography, the bilateral major cerebral vessels were narrowed around the circle of Willis. We critically review previous reports of and explanations for the development of ipsilateral hemichorea.
Adult ; Basal Ganglia Hemorrhage* ; Basal Ganglia* ; Brain ; Cerebral Angiography ; Cerebral Hemorrhage ; Circle of Willis ; Dyskinesias ; Extremities ; Female ; Forearm ; Hand ; Headache ; Hematoma ; Humans ; Leg ; Subthalamus

OBJECTIVE: The objective of this study is to evaluate the effect of therapeutic cold on the recovery process after an incomplete muscle strain injury. METHOD: We made incomplete muscle strain injuries with the predefined extent. Unilateral tibialis anterior (TA) tendons were detached at their distal insertions and stretched beyond the complete rupture points. The incomplete strain injury point was identified on the deformation- tension curve. We strained each TA of 18 rabbits to 20% deformation point and held for 2 seconds. After injury, we applied ice packs for 5 minutes on unilateral TA. Histologic findings were examined at 1, 2 days, 2, 4, 5 weeks. RESULTS: Incomplete strain injuries were occurred at 19.36 4.02% deformation. In the acute phase, cold applied TA showed fewer (15.4+/-4.2) inflammatory cells infiltration than control TA (21.8+/-5.3) at LPF (x40). But, there was no significant difference between cold applied TA and control in the recovery phase. CONCLUSION: It is possible to develop certain amount of incomplete muscle strain injuries. The results of this study suggest that the cold therapy may reduce the inflammatory reaction in the acute phase of muscle strain injury. The findings provide no significant support for the positive or negative effect of cold therapy on incomplete strain injury.
Cryotherapy ; Ice ; Rabbits ; Rupture ; Tendons

BACKGROUND: During orthotopic liver transplantation, magnesium monitoring has been done frequently because of its important role in the cardiovascular system. Generally hypomagnesemia may occur frequently during an operation, but some investigators reported serum magnesium levels returned to normal after reperfusion which is different from our result. Therefore, this study was done to confirm the changes of serum magnesium in the postanhepatic stage and also to confirm the need for prophylactic magnesium administration. METHODS: Thirteen patients plasma magnesium concentrations were measured 8 times during the operation on each patient. We also checked the total transfusion amount and required CaCl2 amount in every patient. The significance of all data were evaluated with a paired t-test and correlation method. RESULTS: Serum magnesium levels were significantly decreased after the beginning of the postanhepatic stage during an operation (P < 0.05). Correlation between transfused blood amounts and CaCl2 requirements showed significant correlation (r: 0.709), but no correlation between magnesium concentrations and transfused blood amounts was found. CONCLUSIONS: We concluded that serum magnesium concentrations were significantly decreased especially in the postanhepatic stage and an appropriate amount of a magnesium supply may be needed.
Cardiovascular System ; Humans ; Liver Transplantation ; Liver* ; Magnesium* ; Plasma ; Reperfusion ; Research Personnel

Long QT syndrome is a cardiac disorder of repolarization which is characterized by elctrocardiographic abnormalities including prolonged QT interval, T-wave abnormalities and polymorphic ventricular tachycardia known as Torsades de Pointes. Its clinical manifestation are recurrent syncope, seizure, and sudden death. Recently,we experienced Torsades de Pointes(TdP) by head-up tilt test in 24 year-old female patient presenting recurrent syncope and long QT interval. Beta-blocker and left cervicothoracic sympathetic ganglionectomy were not effictive, then we tried mexiletine. After mexiletine medication, the QT interval was significantly shortened and there was no more syncope.
Death, Sudden ; Female ; Ganglionectomy ; Humans ; Long QT Syndrome* ; Mexiletine ; Seizures ; Syncope ; Tachycardia, Ventricular ; Torsades de Pointes* ; Young Adult

BACKGROUND AND OBJECTIVES: We investigated the relationship between the degree of enophthalmos and the volume of herniated orbital tissue measured from computed tomography scan in the isolated blowout fractures of orbital wall. SUBJECTS AND METHOD: In this retrospective study, 100 patients with isolated blowout fractures were evaluated. We classified them into 4 groups according to the site of fracture (medial and inferior) and the presence of symptoms like diplopia and limitation of ocular motility, which needs an operation. The volume of orbit and herniated orbital tissue has been measured by computed tomography scans using three-dimensional reconstruction technique, and the degree of enophthalmos was evaluated with Hertel's ophthalmometer. We compared the volume from which we got from the computed tomography scan, the degree of enophthalmos and the presence of symptoms to figure out the mutual relation between the groups. RESULTS: In the case of medial blowout fracture group, the volume of herniated orbital tissues increased significantly with the presence of symptoms and was in proportion to the extent of enophthalmos (p<0.05). The volume expansion of orbit associated with 2 mm of enophthalmos as calculated by the regression curve was 3.1 ml or 12.8 % in the no-symptoms groups. Finally, in the case of inferior blowout fracture group, there was no evidence of mutual relation. CONCLUSION: These results suggest that surgical intervention is required even though there isn't any symptom for medial blowout fractures, especially when the orbit volume is more than 12.8%. In cases of inferior blowout fractures, a close follow-up is needed even though the extent of fracture is small.
Diplopia ; Enophthalmos ; Follow-Up Studies ; Humans ; Orbit ; Retrospective Studies

Ehlers-Danlos syndrome (EDS) is a very rare genetic disorder characterized by defects in the production of connective tissue. Among them, vascular EDS is the subtype known to have the worst prognosis due to fragile blood vessels. Accordingly, we would like to report a case report of a patient with vascular EDS with a review of the literature on possible massive transfusion and anesthesiological problems. Patients with vascular EDS have very weak blood vessels and tissues that are easily broken. So there is a high possibility of unexpected massive bleeding during emergency surgery in these patients. Therefore, the anesthesiologist should be fully prepared for the possibility of massive blood loss, keeping in mind the possibility of damage to large blood vessels. The central vein must be secured using ultrasound, sufficient blood and fluids must be prepared, and equipment capable of rapid administration must be perfectly prepared before the start of operation.