A case of 10 months old Korean female infant with congenital binocular cataracts, posterior fossa cyst, and macrodactyly simulating Marinesco-Sjogren Syndrome is presented. The diagnosis of the posterior fossa cyst was added by brain computerized tomogram, conray ventriculogram and vertebral angiogram. The studies demonstrated marked hydrocephalus with a huge posterior fossa cyst displacing 4 th ventricle and cerebellum anteriorly. The cyst was also extending upward displacing the tentorium superiorly. Following ventriculoperioneal shunt an exploration of the cyst was performed. The cerebellar hemispheres and vermis were appeared to be atrophic and displaced anteriorly due to the cyst. The histologic study of the biopsied cyst wall was normal arachnoid membrane. The authors assume that some of the reported cases of Marinesco- sjogren Syndrome could have similar cystic lesion which was found in this case to cause cerebellar ataxia and mental retardation.
Arachnoid ; Brain ; Cataract* ; Cerebellar Ataxia ; Cerebellum ; Diagnosis ; Female ; Humans ; Hydrocephalus ; Infant ; Intellectual Disability ; Membranes ; Sjogren's Syndrome ; Spinocerebellar Degenerations* ; Telescopes*

No abstract available.
Animals ; Baths* ; Blood Glucose* ; Insulin* ; Rats*

No abstract available.
Muscular Dystrophy, Oculopharyngeal*

No abstract available.
Purpura, Schoenlein-Henoch*

No abstract available.
Humans ; Male ; Spinal Cord Injuries* ; Spinal Cord*

No abstract available.
Brain* ; Child* ; Humans ; Magnetic Resonance Imaging*

It is often difficult, on the basis of clinical examination and conventional investigations, to evaluate the functional impairment of brain in children with cerebral palsy in which early detection remains a challenge. This study was designed to know the usefulness of single photon emission tomography(SPECT) of the brain with technetium-99 m hexamethylpropyleneamineoxime (99mTc-HMPAO) as a semiquantitative method of determination of right-left asymmetries in tracer uptake and a change in antero-posterior regional cerebral blood flow(rCBF) distribution. We investigated 33 children with cerebral palsy aged from 4 months to 48 months(mean 19.3 months). The results were as follows: right to left ratio and regional index of a transverse view were useful to quantify the decrease of tracer uptake in left hemiplegia, but not in right hemiplegia who were all mild in severity, and in tetra- and diplegia in which cerebral lesions were found bilaterally; cortico-cerebellar ratios of a sagittal view were found useful to detect a decrease of rCBF distribution in tetra- and diplegia ,which was not distinct by means of right to left ratio and regional index on transverse view. The results suggest that semiquantitative analysis of 99mTc-HMPAO Brain SPECT would be a valuable complementary tool for determination of topographical involvement in cerebral palsy.
Brain* ; Cerebral Palsy* ; Child ; Hemiplegia ; Humans ; Technetium Tc 99m Exametazime* ; Tomography, Emission-Computed, Single-Photon*

Gastrointestinal stromal tumors (GISTs) are the most common primary mesenchymal neoplasms of the gastrointestinal tract and usually appear as a well-circumscribed mass. However, it may be difficult to confirm the extent of the disease for some GISTs. A 70-year-old asymptomatic female presented for a regular physical exam. An esophagogastroduodenoscopy showed a 2.0 cm protruding mass on the gastric fundus. Endoscopic ultrasound revealed an ill-defined heterogenous hypoechoic lesion (3.0×1.5 cm). A computed tomography (CT) scan demonstrated a 4.5 cm multifocal calcified mass at the gastric body as well as at the gastric fundus. Laparoscopic gastric wedge resection was performed according to the extent of multifocal calcifications that are shown on the CT. Intraoperative specimen mammography and intraoperative biopsy might be helpful to obtain a tumor-free margin. Final pathologic diagnosis was an intermediate risk GIST in multilobular form. In patients with diffuse multifocal calcifications in the stomach, the possibility of GIST should be considered.
Aged ; Biopsy ; Calcinosis ; Diagnosis ; Endoscopy, Digestive System ; Female ; Gastric Fundus ; Gastrointestinal Stromal Tumors* ; Gastrointestinal Tract ; Humans ; Mammography ; Stomach* ; Ultrasonography

The authors analyzed 19 cases of unstable cervical spine injuries who had been treated by anterolateral approach with Caspar plate at the department of neurosurgery of Hangang Sacred Heart hospital during 3 years from 1989. The results were as follows: 1) The unstable cervical spine injury was most common in 3rd decade of age. Male to female ratio was 3.75:1. 2) Regarding the level of injury, C5-6 injury was most common. 3) The most common cause of injury was fall down from height(47.4%) and the next was traffic accident(36.8). 4) In all cases received anterior reconstructive surgery using Caspar plate showed significant neurologic improvement. 5) In all cases received anterior reconstructive surgery showed a good restoration of cervical spinal stability. 6) The major surgical complication was esophageal fistula and all complications were treated without any sequelae. 7) Anterior reconstructive surgery using Caspar plate is considered one of the surgical options in patient of cervical spine injury with severe instability.
Esophageal Fistula ; Female ; Heart ; Humans ; Male ; Neurosurgery ; Spine*

Differential diagnosis of invasive aspergillosis from other pulmonary fungal infections including mucormycosis is important because the treatment is pathogen-dependent. Clinically, invasive aspergillosis is often discriminated from other mold infections on the basis of typical histopathologic features in the biopsy specimen. However, biopsy alone is not always complete because different fungal species can display similar histopathologic features. Surrogate markers or molecular-based assays can be useful when the results of conventional diagnostic modalities are conflicting. Here, we present a case of invasive pulmonary aspergillosis histologically mimicking mucormycosis, which was confirmed by fungal polymerase chain reaction.
Aspergillosis ; Biomarkers ; Biopsy ; Diagnosis, Differential ; Fungi ; Invasive Pulmonary Aspergillosis* ; Lung Diseases, Fungal ; Mucormycosis* ; Polymerase Chain Reaction