No abstract available.
Drug Therapy* ; Germ Cells* ; Neoplasms, Germ Cell and Embryonal*

Sertoli-Leydig cell tumors of the ovary are rare, accounting for no more than 0.5% of all ovarian neoplasms. These tumors occur predominantly in women 10 to 30 years of age. Heterologous elements are found in over 20% of the moderately and poorly differentiated Sertoli- Leydig cell tumors. The most common element is gastrointestinal mucin-secreting epithelium, found in approximately 20% of all cases. Approximately 4% of Sertoli-Leydig cell tumors contain immature skeletal muscle and/or cartilaginous elements. The origin of these neoplasms is regarded as neometaplasia of the mesodermal neoplastic element. While the degree of differentiation of Sertoli-Leydig cell tumors is probably of greater importance in determining prognosis than the presence of heterologous components, recent evidence does point to an unfavorable effect on clinical outcome when heterologous mesenchymal components such as cartilage and striated muscle are present. We experienced two cases of ovarian heterologous Sertoli-Leydig cell tumors with gastrointestinal type epithelium and striated skeletal muscle in 20 and 21 year-old females, respectively. One case involved a patient who had complained of amenorrhea for 3 years while the other case involved a patient who had complained of left lower abdominal tenderness and amenorrhea for one and one half years. It was determined by ultrasonography of the pelvis that the two patients suffered from right and left ovarian masses. Both patients underwent adnexectomy. These tumors were large with partly solid and partly cystic areas. Based on light microscopic examination one case was classified as an intermediate Sertoli-Leydig cell tumor with heterologous gastrointestinal type epithelium, while the other case was classified as a poorly differentiated Sertoli-Leydig cell tumor with heterologous striated skeletal muscle.
Female ; Humans

Congenital ocular motor apraxia is a rare syndrome characterized by rotational head thrusts that attempt to compensate for the lack of voluntary eye movements. We describe the clinical, oculographic and magnetic resonance imaging features of two children with congenital ocular motor apraxia.
Apraxias ; Child ; Cogan Syndrome ; Eye Movements ; Head ; Humans ; Magnetic Resonance Imaging

Isaacs' syndrome is a rare and heterogeneous syndrome of continuous muscle fiber activity that originates from peripheral nerves. We report a 56-year-old male patient who showed symptoms of Isaacs' syndrome after the removal of a malignant thymoma. Needle electromyography revealed spontaneously occurring repetitive myokymic discharge in the affected muscles. Acetylcholine receptor (AChR) antibodies were significantly elevated, but clinical and electrophysiologic findings did not indicate the presence of myasthenia gravis. We deduce that in Isaacs' syndrome, raised AChR antibodies may facilitate rather than inhibit cholinergic action.
Acetylcholine ; Antibodies ; Electromyography ; Humans ; Isaacs Syndrome ; Male ; Middle Aged ; Muscles ; Myasthenia Gravis ; Needles ; Peripheral Nerves ; Thymoma

BACKGROUND: Methyl bromide is toxic to the central and peripheral nervous systems. Two patients with occupational exposure to this agent are described. CASE: Two cases of previously healthy young men were involved. They had worked in a fumigating plant spraying fruits using methyl bromide for several months before admission. They showed general weakness, severe ataxia, bilateral dysmetria and bilateral lateral gaze limitation. And gaze-evoked nystagmus was observed in one of them. Brain MRI showed symmetrically increased signal intensity lesions including the brainstem, cerebellum and splenium of corpus callosum. After conservative treatment, they gradually improved over the next few weeks. CONCLUSIONS: Symmetry and topography of our patients??clinical and radiographic abnormalities support the proposal that methyl bromide exposure can produce symptoms similar to Bickerstaff's brainstem encephalitis.
Ataxia ; Brain ; Brain Stem ; Cerebellar Ataxia ; Cerebellum ; Corpus Callosum ; Encephalitis ; Fruit ; Humans ; Hydrocarbons, Brominated ; Male ; Occupational Exposure ; Peripheral Nervous System ; Plants

CHAP syndorme consists of choreoathetosis, orofacial dyskinesia, affective change, hypotonia, and pseudobulbar signs. CHAP syndrome was firstly des- cribed with onset 1 to 7 days after induced profound hypothermia and complete circulatory arrest for con- genital heart lesion repair. Since then, only a few cases have been reported until now. Case We report a patient who demonstrated all of the elements of the CHAP syndrome in severe cachexic state. Brain SPECT(99mTC-ECD) shows decreased perfusion in left fronto-parieto-temporal lobe. Comments The pathogenesis of CHAP syndrome is obscure. However our case led us to suggest that more essential component pre- disposing to the CHAP syndrome is hypoxia rather than hypothermia. Furthermore, it seems to be related with circulatory insufficiency in extrapyramidal nervous systems.
Anoxia ; Brain ; Heart ; Humans ; Hypothermia ; Movement Disorders ; Muscle Hypotonia ; Nervous System ; Perfusion

No abstract available.
Pregnancy*

No abstract available.
Abortion, Spontaneous* ; Chorion* ; Chorionic Villi* ; Female ; Humans ; Pregnancy

A twenty four-year-old female patient had suffered progressive dyspnea for 6 years until death. She denied any symptoms suggestive of connective tissue disease, or deep vein thrombosis. She suffered an episode of pontine infarct in 1995. Four years after diagnosis of primary pulmonary hypertension, she died of sudden death during hospitalization. Gross features of pulmonary arteries at autopsy were as follows: left main pulmonary artery showed dilation of the lumen and thickening of the wall, and right main pulmonary artery was markedly dilated and contained fresh thrombus. Hematoxylin and eosin-stained sections of lung tissue showed plexiform lesions of pulmonary arteries, complete luminal obliteration of pulmonary arterioles and dilated lesion of pulmonary arterioles, and capillaries. This patient represents a typical case with a primary pulmonary arteriopathy with plexiform lesions with thrombotic lesion, demonstrating the importance of thrombosis in situ in the pathogenesis of primary pulmonary hypertension. To our knowledge, this is the first autopsy report on the primary pulmonary hypertension in Korea.
Arterioles ; Autopsy ; Capillaries ; Connective Tissue Diseases ; Death, Sudden ; Diagnosis ; Dyspnea ; Female ; Hematoxylin ; Hospitalization ; Humans ; Hypertension, Pulmonary* ; Korea ; Lung ; Phenobarbital ; Pulmonary Artery ; Thrombosis ; Venous Thrombosis

No abstract available.
Humans ; Male*