Pharmacologic therapy is still the primary management for epilpsy; however, surgical treatment is a reasonable therapeutic option for patients suffering from medically intractable seizures, especially temporal lobe epilepsy having a documented unilateral epileptogenic area. Thirty nine patients with pharmaco-resistant complex partial seizures underwent anterior temporal lobectomy and hippocampectomy in 38 cases and frontal cortisectomy in one case. On pathological examination, hippocampal sclerosis was a predominent pathologic finding and was identified in 18 cases. Other non-neoplastic lesions consisted of 5 cases of vascular lesions(2 cavernous angiomas, 2 arteriovenous malformations and 1 angiomatosis), 3 cases of fibrous nodule, 2 cases of cicatrical changes of cerebral cortex, and 1 case of parasitic infection. Neoplasms including two cases of oligodendroglioma and one case of anaplastic astrocytoma were also noted. In seven cases, there was no detectable lesion on gross and microscopic examination. On post-operative follow-up, seizures were completely terminated in most cases(31 cases, 79%). The rest of the patients also displayed marked alleviation of symptoms. The seizures tended to recur more aften among the patients with neoplasm or no pathologically detectable lesion. In order to detect any minute pathological lesion, thorough gross and microcsopic examinations are considered to be essential.

Goltz syndrome is known as a rare mesoectodermal hereditary disease, characterized by focal dermal atrophies with hernias of adipose tissue and also associated with a multitude of possible skeletal, dental, ophthalmological and other abnormalities. We experienced a case of Goltz syndrome. An one day old female newborn had focal atrophic and telangiectatic skin lesions, microphthalmia, syndactyly and urinary tract abnormality. The finding of skin biopsy was consistent with focal dermal hypoplasia. We report the case with a brief review and related literatures.
Adipose Tissue ; Atrophy ; Biopsy ; Female ; Focal Dermal Hypoplasia* ; Genetic Diseases, Inborn ; Hernia ; Humans ; Infant, Newborn ; Microphthalmos ; Skin ; Syndactyly ; Urinary Tract

Endocardial fibroelastosis (EFE) is characterized by deposition of collagen and elastin leading to ventricular hypertrophy and diffuse endocardial thickening. Here we report (for the first time in Korea) the case of a EFE presenting with heart failure. The patient was a 57-year-old woman who had complained of dyspnea on exertion {New York Heart Association (NYHA) functional class 3} and abdominal distension at the time of hospital admission. Echocardiography showed severe diastolic dysfunction with normal systolic function. On MRI, the contrast-enhanced delayed myocardial image demonstrated hyperenhancement in the endocardium. Owing to progressive heart failure, the patient was transplanted. Histological examination of the explanted heart showed irregularly thickened endocardium with fibrosis and elastosis in the both ventricles, compatible with the diagnosis of EFE.
Cardiomyopathy, Restrictive ; Collagen ; Dyspnea ; Echocardiography ; Elastin ; Endocardial Fibroelastosis ; Endocardium ; Female ; Fibrosis ; Heart ; Heart Failure ; Heart Transplantation ; Humans ; Hypertrophy ; Middle Aged ; Transplants

A 21-year-old male was presented with sudden headache, fever, petechiae and neck stiffness. The diagnosis of meningococcal meningitis was confirmed by examination of cerebrospinal fluid. The clinical symptoms of the illness were improved after treatment of antibiotics. However the patient developed generalized edema, oliguria, azotemia, and heavy proteinuria in the recovery phase of illness. Low serum C3 level was also noted. A kidney biopsy was performed and showed the features of postinfectious glomerulonephritis and typical subepithelial humps on electron-microscopic examination. His symptoms and laboratory findings were improved, and C3 level returned to normal range after conservative treatment. We suggest that a complement deficiency should be ruled out in patients of glomerulonephritis developed during the recovery phase of meningococcal meningitis. C3 nephritic factor detection and renal biopsy should be carefully considered in these patients.
Anti-Bacterial Agents ; Azotemia ; Biopsy ; Cerebrospinal Fluid ; Complement C3 Nephritic Factor ; Complement System Proteins ; Diagnosis ; Edema ; Fever ; Glomerulonephritis* ; Headache ; Humans ; Kidney ; Male ; Meningitis ; Meningitis, Meningococcal* ; Neck ; Neisseria meningitidis ; Oliguria ; Proteinuria ; Purpura ; Reference Values ; Young Adult