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*

OBJECTIVE: Most of the studies that have described surgical procedures for moyamoya disease(MMD) have had the aim to improve the blood flow mainly in the middle cerebral artery(MCA) territory. The purpose of this study is to clarify the good indications for a multiple burrhole operation to treat MMD. METHODS: With the patient under local anesthesia, burrhole trephination with a diameter of 1.5 to 2cm at 3 to 5cm lateral to the midline was performed in a total of 18 hemispheres during 12 procedures. The burrholes were opened in the frontal lobe at 19 sites in 10 patients and in the parietal lobe at 22 sites in 12 patients. Revascularization through multiple burrholes and arachnoid openings was performed in 12 patients who suffered from MMD. RESULTS: As seen on the follow up angiograms, good revascularization was achieved in 27 of 41 burrholes, and mainly from the middle meningeal artery and then from the superficial temporal artery. The moyamoya vessels were decreased in four patients. The neovascularization covered 68.4% of the frontal lobe and 63.5% of the parietal lobe. Compared with the preoperative 99mTc-HMPAO-SPECT, 6 patients showed improvement of cerebral blood flow on the postoperative 99mTc-HMPAO-SPECT. Transient ischemic attack disappeared in three of the four patients who presented with this symptom, and the preoperative symptoms improved in both of the patients who presented with headache and cerebral infarction. CONCLUSIONS: A multiple burrhole operation is a simple technique. The results of our series suggest that the revascularization obtained for adult MMD patients who underwent a multiple burrhole operation is similar to that obtained with other indirect surgeries, but long term follow up studies are needed.
Adult ; Anesthesia, Local ; Arachnoid ; Cerebral Infarction ; Follow-Up Studies ; Frontal Lobe ; Headache ; Humans ; Ischemic Attack, Transient ; Meningeal Arteries ; Moyamoya Disease ; Parietal Lobe ; Temporal Arteries

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

OBJECTIVE: Surgical site infection (SSI) is a problem constantly uppermost in the minds of all surgeons, although the actual rate of occurrence is only 1-5% in general surgery. In neurosurgical fields, there have been a few papers published about efficacy of post-operative antimicrobial prophylaxis (PAMP) to prevent SSI, compared to well known effectiveness of pre-operative antibiotics. Thus, infection rates of short-term PAMP groups and those of long-term PAMP groups were investigated to evaluate the effectiveness of PAMP and the efficacy of short-term PAMP compared to long-term PAMP for prevention of SSI. METHODS: Between April 2010 and April 2012, we retrospectively analyzed the data of 35 patients in the aneurysmal neck clipping groups (short-term PAMP group: PAMP for 3 days and fewer, long-term PAMP group: PAMP for 10 days and more) and 79 patients in the microdiscectomy groups (short-term PAMP group: 3 days and fewer, long-term PAMP group: PAMP for 6 days and more). RESULTS: In aneurysmal neck clipping groups, SSI occurred 23.1% of short-term PAMP group and 9.1% of long-term PAMP group (p=0.3370). And in microdiscectomy groups, SSI occurred 6.7% of short-term PAMP group and 4.1% of long-term PAMP group (p=0.9840). CONCLUSION: There is no significant difference between the short-term PAMP group and the long-term PAMP group in terms of SSI, regardless of operation type. We therefore suggest that short-term PAMP usage could be an appropriate therapy for preventing SSI in clean neurosurgical operations.
Aneurysm ; Anti-Bacterial Agents ; Antibiotic Prophylaxis ; Humans ; Neck ; Neurosurgical Procedures ; Retrospective Studies ; Surgical Wound Infection