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*

Informed consent is the procedure that respects the autonomy of patients ; doctors protect patients' choices and rights through informed consent. Competence and voluntariness are prerequisite for the informed consent. In recent years, the necessity of informed consent from the Psychiatric department has been raised. Regarding informed consent from the Psychiatric department, there is a need to consider from two perspectives : respect for the patient's decision-making ability and the psychiatrist's legal self-protection. In competence and voluntariness, psychiatric patients were excluded due to lack of decision-making ability and willingness. However, not all psychiatric patients were able to perform the given task. Therefore, informed consent is needed in the point of view regarding the decision-making ability of psychiatric patients. Psychotropic medications, particularly antipsychotics, can cause several side effects, including extrapyramidal syndrome and metabolic syndrome. Therefore, preannouncement of these patients for these side effects is needed. However, when the informed consent is only applied to psychiatric hospitals, it may increase prejudices and misconceptions about psychiatric medications and treatment. Therefore, the informed consent should first be considered carefully and thoughtfully.
Antipsychotic Agents ; Hospitals, Psychiatric ; Human Rights ; Humans ; Informed Consent* ; Mental Competency ; Prejudice

OBJECTIVES: Advance directives (AD) are designed to protect patients’ autonomy and self-determination, which mean the end of life care planning should precede before loss of their decision ability. We aimed to analyze our experience of AD at field of oncology, focusing on preference of end-of-life care and outcome in advanced cancer patients. METHODS: A retrospective review was conducted on advanced cancer patients who underwent AD at the department of Oncology of Hanil General Hospital, between April 2013 and January 2014. AD are composed of decision about end of life care (resuscitation, ventilator, artificial tube feeding) and determination of proxy. RESULTS: Among 23 patients who were recommended AD during study period, 19 patients (83%) successfully underwent AD. The median age was 67 years (range, 50 to 95 years) and male was predominance (84%) was observed. Most of them (90%) have not heard of AD in the past. With regard to decision of end-of-life care, decision for resuscitation and ventilator were selected only by 1 patient (5%), respectively, while 10 patients (52%) decided to receive tube feeding. Among 5 patients who underwent AD during chemotherapy, there was neither transfer to other hospital for anti-cancer treatment nor follow up loss. CONCLUSION: AD might be applicable in advanced cancer patients at field of oncology, including also patients treated with palliative chemotherapy.
Advance Directives* ; Drug Therapy ; Enteral Nutrition ; Follow-Up Studies ; Hospitals, General ; Humans ; Male ; Proxy ; Resuscitation ; Retrospective Studies ; Terminal Care ; Ventilators, Mechanical

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*