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

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