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

No abstract available.
Electroencephalography* ; Neuronal Ceroid-Lipofuscinoses ; Neurons*

Blepharoplasty is a commonly performed aesthetic surgery, but there is still no commonly used standard method for the correction of orbital fat bulging in lower blepharoplasty. We performed dual plane method by elevating skin flap and muscle flap separately, and then repositioned orbital fat with preserving orbital septum for correction of orbital fat bulging in lower blepharoplasty. Between July, 1998 and June, 2005, one hundred and fifty six patients underwent lower blepharoplasty by author's method. The results were satisfactory in all of the patients. Complications were six cases of hematoma and ten cases of visible scar in lateral canthal area. There was no skin flap necrosis, hyperpigmentation, skin irregularity or ectropion. In lower blepharoplasty for the sufficient excision of skin and operation of orbicularis oculi, we performed precise correction of skin and muscle independently by dual plane approach, by separate elevation of skin flap and muscle flap, and were able to reposition orbital fat easily and safely with preservation of orbital septum.
Blepharoplasty* ; Cicatrix ; Ectropion ; Hematoma ; Humans ; Hyperpigmentation ; Necrosis ; Orbit* ; Skin

PURPOSE: Cytokines play important roles on the expression of various neuronal inflammatory disease and insults. The purpose of this study was to evaluate the levels of interleukine (IL)-6, IL-8, IL-10 in cerebrospinal fluid (CSF) in children with aseptic meningitis and compare them with those of the patients having other acute neurological symptoms. METHODS: We retrospectively reviewed the medical records of the children who admitted in the pediatric department of Hanyang University Guri Hospital for acute neurological symptoms and had CSF examinations from September 2012 to July 2013. We classified them into six groups as acute encephalopathy, epilepsy, febrile convulsion, headache, infantile fever, and meningitis. We analyzed the clinical and laboratory data from them. RESULTS: A total of 87 CSFs of the patients were available. The levels of CSF IL-6, IL-8, and IL-10 were significantly increased in the group with aseptic meningitis group as compared to the other groups (P<0.05). CSF IL-6 (r=0.576, P=0.000), IL-8 (r=0.329, P=0.003), and IL-10 (r=0.523, P=0.000) were all significantly correlated with CSF White bood cell (WBC) count. Among the patients with aseptic meningitis, CSF enterovirus positive patients (CSF entero+) showed significantly increased IL-6, IL-8, IL-10 levels than CSF enterovirus negative patients (CSF entero-) (P<0.05). In addition, the CSF entero+ and the increase of IL-10 were significantly correlated (x2=6.827, P=0.033). CONCLUSION: In patients with aseptic meningitis, the CSF IL-6, IL-8 and IL-10 were more expressed than in other neurological disease group. Among them, the enteroviral meningitis may be more related with IL-6, IL-8 and IL-10 expression than in other causes of aseptic meningitis.
Cerebrospinal Fluid* ; Child* ; Cytokines ; Enterovirus ; Epilepsy ; Fever ; Headache ; Humans ; Interleukin-10 ; Interleukin-6 ; Interleukin-8 ; Interleukins ; Medical Records ; Meningitis ; Meningitis, Aseptic* ; Neurons ; Retrospective Studies ; Seizures, Febrile

Angioleiomyoma is an infrequent benign smooth muscle tumor that arises from smooth muscle cells of arterial or venous walls in the tunica media layer. It would be found in the dermis, the subcutaneous tissue, or the superficial fascia of the anywhere in the body and is most often seen in the lower extremities. The typical lesion is a small, slowly growing, round, but firm and mobile nodule. We report a case of angioleiomyoma located on the anterior aspect of the elbow, which was mistaken for extradigital glomus tumor after history taking, physical examination. With point tenderness and worsening sharp pain in cold exposure for several years, the patient was referred for a further evaluation, and the lesion was 5 mm sized well-circumscribed mass in the anterior elbow with vascular signals on color and power Doppler by ultrasonography and finally diagnosed as angioleiomyoma following complete excision and histological evaluation.

Angioleiomyoma is an infrequent benign smooth muscle tumor that arises from smooth muscle cells of arterial or venous walls in the tunica media layer. It would be found in the dermis, the subcutaneous tissue, or the superficial fascia of the anywhere in the body and is most often seen in the lower extremities. The typical lesion is a small, slowly growing, round, but firm and mobile nodule. We report a case of angioleiomyoma located on the anterior aspect of the elbow, which was mistaken for extradigital glomus tumor after history taking, physical examination. With point tenderness and worsening sharp pain in cold exposure for several years, the patient was referred for a further evaluation, and the lesion was 5 mm sized well-circumscribed mass in the anterior elbow with vascular signals on color and power Doppler by ultrasonography and finally diagnosed as angioleiomyoma following complete excision and histological evaluation.

Immunoglobulin A (IgA) nephropathy in patients with the clinical findings of rapidly progressive glomerulonephritis usually demonstrate crescentic glomerulonephritis (CrGN). It is characterized by mesangial immune complex deposits containing IgA and is rarely associated with ANCA. The following case report illustrates a CrGN with mesangial & capillary wall IgA deposits by immunofluorescence (IF) and mesangial & endocapillary electron dense deposits by electron microscopy (EM) in patient with positive ANCA serology. A 54-year-old male visited due to the discomfort of right flank. Blood pressure was 150/90 mmHg. BUN and serum Creatinine was 40 mg/dL, 4.4 mg/dL respectively. Urinalysis revealed protein 2+, >30 RBC's/ HPF (dysmorphic), 24hr urine protein 1,612 mg/day and creatinine clearance 19 mL/min/1.73m2. Serological P-ANCA was positive by indirect immunofluorescence & ELISA. The histologic findings showed global scleorsis and fibrocelluar crescent without hypercellularity on LM, mesangial IgA deposition on IF, mesangial and subendothelial electrone dense material depositions on EM. After treatment (Methylprednisolon 500 mg/day for 3 days and then prednisolone 60 mg/day), Serum creatinin level, proteinuria and ANCA titer were decreased.
Antibodies ; Antibodies, Antineutrophil Cytoplasmic* ; Antigen-Antibody Complex ; Blood Pressure ; Capillaries ; Creatinine ; Enzyme-Linked Immunosorbent Assay ; Fluorescent Antibody Technique ; Fluorescent Antibody Technique, Indirect ; Glomerulonephritis ; Glomerulonephritis, IGA* ; Humans ; Immunoglobulin A* ; Male ; Microscopy, Electron ; Middle Aged ; Prednisolone ; Proteinuria ; Urinalysis

Myxoma is the most common primary tumor in the heart. Cardiac myxomas can present in various manners including embolization and fever, sometimes simulating endocarditis. However, they are rarely infected. We report here a case of an infected left atrial myxoma that seeded a normal mitral valve and atypically presented with multiple embolic events in the lower extremities along with multiple splenic and a cerebellar infarction.
Endocarditis ; Fever ; Heart ; Infarction ; Lower Extremity ; Mitral Valve ; Myxoma ; Seeds

A 47-year-old man had suffered oscillopsia associated with palatal myoclonus for 10 years. High-field magnetic resonance imaging (MRI) revealed a cryptic vascular malformation within the "Guillain-Mollaret triangle" which was thought to be the responsible lesion.
Brain Stem/*abnormalities/pathology ; Humans ; *Magnetic Resonance Imaging ; Male ; Middle Aged ; Myoclonus/*etiology

PURPOSE: Reports of neurologic abnormalities associated with acute diarrhea are increasing recently. It was reported that the incidence of the neurologic abnormalities related to gastroenteritis was higher in rotavirus gastroenteritis than in non-rotavirus gastroenteritis. We investigated the incidence, the manifestations and the prognosis of the neurologic abnormalities associated with rotavirus diarrhea and non-rotavirus diarrhea in Korean children. METHODS: Six hundred forty-nine children who showed acute diarrhea and whose stools were examined for rotavirus were enrolled and categorized into the rotavirus positive group(n=186) and the negative group(n=463). The medical records were reviewed retrospectively for neurologic manifestations, diagnoses and the status of follow-up. RESULTS:The incidence of neurologic abnormalities in all children with diarrhea was 9.4%. Neurologic abnormalities associated with diarrhea were more common in the rotavirus positive group than in the rotavirus negative group(16.1% vs 6.7%, P=0.0002). The neurologic diagnoses of the children ranged from simple febrile convulsion to encephalitis. The rate of patients presenting neurologic abnormalities other than seizures was relatively higher in the rotavirus positive group than in the rotavirus negative group(56.7% vs 25.8%, P=0.01). All children with neurologic abnormalities showed complete recovery. CONCLUSION: The incidence of neurologic abnormalities among patients with diarrhea was about 9%. The neurologic diagnoses in patients who showed neurologic abnormalities were diversed. In rotavirus gastroenterits, the rate of patients presenting neurologic abnormalities other than seizure was higher than in non-rotavirus gastroenteritis. The outcome of the children who showed neurologic abnormalities with diarrhea was excellent.
Child ; Diagnosis ; Diarrhea ; Encephalitis ; Follow-Up Studies ; Gastroenteritis* ; Humans ; Incidence ; Medical Records ; Neurologic Manifestations ; Prognosis ; Retrospective Studies ; Rotavirus* ; Seizures ; Seizures, Febrile