Plexiform neurofibroma is considered a pathognomic of Von Recklinghousen's disease, which involves the deep and large nerve trunk. These are large irregular nerve fascicles which result from an increase in endoneural matrix within individual nerve facicles, without an increased number of nerve fibers. We experenced a case of Von Recklinghausen's disease in a 24 year-old male who had variable cutaneous skeletal, and CNS lesions. He presented multiple neurofibromas, cafe-au-lait spots, and axillary freckles as common cutaneous lesions of NF-I and giant pigmentation, sacral hypertrichosis, and plexiform neurofibroma as unusual cutaneous lesions. Also he had a scoliosis, bowing deformity of the humerous and wedging deformity of the body of the 5th cervical spine as a skeletal manifestation and cortical calcification in the occipital area as a CNS manifestation.
Cafe-au-Lait Spots ; Congenital Abnormalities ; Humans ; Hypertrichosis ; Male ; Melanosis ; Nerve Fibers ; Neurofibroma, Plexiform* ; Neurofibromatoses ; Neurofibromatosis 1 ; Pigmentation* ; Scoliosis ; Spine ; Young Adult

PURPOSE: To report a case of Charles Bonnet syndrome that developed after resection of a meningioma. CASE SUMMARY: The authors reviewed the medical record, brain magnetic resonance image, and Goldmann visual field test of a 56-year-old male patient who was diagnosed with a meningioma in the right parietal and occipital lobe and underwent resection of the tumor. The preoperative Goldmann visual field test showed homonymous left inferior quadrantanopsia. Subtotal resection of the mass in the right parietal and occipital lobe was performed, and postoperative histopathologic examination confirmed the diagnosis of a meningioma. Postoperatively, the patient complained of visual hallucination in an area of the eye with visual field defects. However, his consciousness and orientations were intact, and other cognitive functions were also normal. CONCLUSIONS: Visual hallucination can manifest in an area with visual field defects after resection of abrain tumor. In such a case, a diagnosis of Charles Bonnet syndrome should be considered.
Brain ; Brain Neoplasms ; Consciousness ; Eye ; Hallucinations ; Hemianopsia ; Humans ; Magnetic Resonance Spectroscopy ; Male ; Medical Records ; Meningioma ; Middle Aged ; Occipital Lobe ; Orientation ; Visual Field Tests ; Visual Fields

PURPOSE: To report a case of functional bilateral hemianopia which was not associated with any organic causes. CASE SUMMARY: A 35-year-old female patient presented with bilateral disturbance of visual acuity and visual field, which had begun 8 months prior. Goldmann perimetry showed bitemporal hemianopsia respecting the vertical meridian. Pupillary response was normal, and the anterior segment, fundus, and optic nerve were also normal bilaterally. However, the tangent screen test and Humphrey visual field test showed a widening of hemianopia not respecting the vertical meridian, and the crossing of isopters at 1 m and 2 m with the tangent screen test. In addition, multifocal electroretinogram and multifocal visual evoked potential did not reveal any abnormal findings corresponding to the bitemporal hemianopia. Brain magnetic resonance imaging showed no abnormal findings in the orbit and brain. CONCLUSIONS: Bitemporal hemianopsia can be developed functionally, although it may be rare. Repetition of various visual field tests and use of multifocal electrophysiologic studies can be helpful in the differential diagnosis.
Adult ; Brain ; Diagnosis, Differential ; Evoked Potentials, Visual ; Female ; Hemianopsia ; Humans ; Magnetic Resonance Imaging ; Optic Nerve ; Orbit ; Visual Acuity ; Visual Field Tests ; Visual Fields

A 51-year-old woman with breast cancer presented with progressive diplopia. Neuro-ophthalmologic examination revealed right gaze palsy and peripheral facial nerve palsy. Brain magnetic resonance imaging (MRI) was normal. However, two months later a repeat brain MRI revealed an enhancing round nodular mass at the right facial colliculus of the lower pons, at the location of the abducens nucleus. Localized metastasis to the abducens nucleus can cause gaze palsy in a patient with breast cancer.
*Abducens Nerve Diseases ; Breast Neoplasms/*pathology ; Cranial Nerve Neoplasms/*complications/*secondary ; Facial Paralysis/complications ; Female ; Fixation, Ocular ; Humans ; Magnetic Resonance Imaging ; Middle Aged ; Ocular Motility Disorders/*etiology/physiopathology ; Pons/pathology

BACKGROUND: The current various case-finding instruments for detecting depression in Korea are too cucumbersome and time-consuming for routine use in primary care or student and soldier groups because of too many questions. We carried out this study in order to investigate the validity of the two-question case-finding instrument for detecting depression easily in the primary care or the mentioned groups. METHODS: We selected one boy high school in Seoul and 155 sophomer students answered the questionnaire by self-report. The questionnaire included two questions about depressed mood and anhedonia: (1) "During the past month, have you often been bothered by feeling down, depressed or hopeless?" (2) "During the past month, have you often been bothered by little interest or pleasure in doing things(e.g., studying, playing or talking with friends) ?". And then a resident of family medicine interviewed them and made a diagnosis for depression using the diagnostic criteria of DSM-IV. Simultaneously we compared the test characteristics of a two-question case-finding instrument with those of a previously validated Beck Depression Inventory as a currently world-wide used screening instrument for depression. RESULTS: The prevalence of major depression as determined by the interview was 6.6%(10 of 151). The two-question case finding instrument had a Cronbach's alpha of 0.663-internal consistency, a sensitivity of 100%, a specificity of 54.6%, a positive likelihood ratio of 2.20 and a negative predictive value of 1.00.(A "yes" answer to either of the two questions was considered a positive test.) And the BDI had a sensitivity of 90%, a specificity of 68.1%, a positive likelihood ratio of 3.63 and a negative predictive value of 0.99(cut-off point=15). Area under the ROC curves of the two-question case-finding instrument was 0.882, greater than that of the BDI, 0.834. CONCLUSIONS: The test characteristics of a two-question case-finding instrument were higher compared to those of BDI for major depression. Therefore, the two-question case-finding instrument is a useful measure for detecting depression and less time-consuming in primary care and certain groups.
Adolescent ; Anhedonia ; Depression* ; Diagnosis ; Diagnostic and Statistical Manual of Mental Disorders ; Humans ; Korea ; Male ; Mass Screening* ; Military Personnel ; Pleasure ; Prevalence ; Primary Health Care ; ROC Curve ; Sensitivity and Specificity ; Seoul ; Surveys and Questionnaires

Hemangioblastomas are angioblastic tumors of the central nervous system. Cerebellar hemangioblastomas are traditionally classified into two morphologic types-cystic and solid. Cystic hemangioblastomas are associated with peritumoral edema, but solid hemangioblastomas are not. We report a case of solid cerebellar hemangioblastoma with massive peritumoral edema. An 83-year-old female visited our hospital due to a sudden headache. Five years ago, she had been admitted to our hospital with similar headache and diagnosed with cerebellar hemangioblastoma. Follow-up brain MRI 5 years later showed an increased size of a homogeneous enhancing mass with aggravated peritumoral edema in the left lower cerebellar hemisphere. Cerebral angiography showed a highly vascularized mass in the cerebellum, which was compatible with a solid-type hemangioblastoma.
Aged, 80 and over ; Brain ; Central Nervous System ; Cerebellum ; Cerebral Angiography ; Edema* ; Female ; Follow-Up Studies* ; Headache ; Hemangioblastoma* ; Humans ; Magnetic Resonance Imaging

Hemichorea-hemiballsim has been reported as a rare Implication of nonketotic hyperglycemia. Recently, Some reports revealed that brain magnetic resonance images of nonketotic hyperglycemic patients with hemichorea-hemiballism showed characteristic contralateral striatal signal abnormality, We present six patients with hemichorea-heniiballism. All of them had diabetes mellitus and performed brain MR images. The MR images of them showed high signal intensity on Tl-weighted images and low signal intensity on T2 weighted images in the striatum contralateal to the involuntary movement. In general, the striatal high signal intensity on TI weighted images are presumed to have developed following hemorrhagic transformation, osmotic myelinolysis, or mild ischemia with reversible deposition of calcium or other material occurred in association with nonketotic hyperglycemia. However the precise mechanism of this signal change is not clear yet and needs pathological confirmation.
Brain ; Calcium ; Diabetes Mellitus ; Dyskinesias ; Humans ; Hyperglycemia ; Ischemia

BACKGROUND: Patient controlled analgesia (PCA) device is known to be an effective method for acute and chronic pain control and the administration route for PCA pump is various. The representative routes are intravenous and epidural space. The aim of this study is to investigate the influence of catheter diameter on flow rate. METHODS: IV extension tube or epidural catheter connected to Mechanical- or balloon-type PCA devices were examined (100 ml, 2 ml/hr). There were four groups each of 5 experiments: Group I: Mechanical-type pump + IV extension tube, Group II: Mechanical-type pump + epidural catheter, Group III: Balloon-type pump + IV extension tube, Group IV: Balloon-type pump + epidural catheter. The flow rate was indirectly measured by the weight change of collecting infusate bottle. Infusion fluid was distilled water. Experiment was carried out in a laboratory room with a constant room temperature of 20-22degrees C. RESULTS: Infusion rate differed significantly among the groups, exhibiting flow rates within +/-15% of their expected rate for 28% (group IV) to 100% (group I) of their infusion duration. The mean Infusion rate in group I was significantly more rapid than that in other groups. The mean flow rate in group III was higher than that in group IV (P<0.05). CONCLUSIONS: These results indicate that the use of an epidural catheter with a small internal diameter could decrease the flow rate of PCA device.
Analgesia, Patient-Controlled ; Catheters ; Chronic Pain ; Epidural Space ; Humans ; Passive Cutaneous Anaphylaxis ; Water

The tests for sputum acid fast bacilli and sputum cytology for malignancy were negative. The PC20 of bronchial methacholine challenge test was 20.2 mg/ml. Transbronchial lung biopsy showed bronchial inflammatory change with infiltration of eosinophils and the existence of fungal hypae. Antibody index for serum IgE-Af and IgG-Af was 10.2 and 2.1 respectively, comparing with Af-sensitive asthma patients. Recently we experienced an allergic bron-chopulmonary aspergillosis with atypical respiratory symptoms. Forty-seven years old female was admitted to our hospital because of cough, sputum, right pleuritic pain for 2 weeks. She had experienced pneumonia several times, but had been not confirmed any causative organism. On physical examination, breathing sound was decreased over right upper lung, but wheezing and crackle were not heard over both lungs. The chest X-ray showed segmental consolidation in right upper lobe, and HRCT showed tubular bronchiectasis and obstruction of right upper lobal bronchus due to mucoid impaction. Allergic skin prick test against Aspergillus fumigatus(Af) and serum preeipitin were negative, but intrader mal skin test was positive for Af. Specific IgE for Af was positive with class I. Total eosinophil count was 650/mm3, total IgE level was more than 3000 IU/ml, ESR was 62mm/hr, and eosinophils in induced sputum was 35%. The test for sputum acid fast bacilli and spu-Tum cytology for malignancy were negative. The PC20 of bronchial methacholine challenge-Test was 20.2 mg/ml. Transbronchial lung bi-Opsy showed bronchial inflammatory change With infiltration of eosinophils and the exis-Serum lgE-Af and lgG-Af was 10.2 and 2.1 Respectively, comparing with Af-sensitive Asthma patients.
Aspergillosis ; Aspergillus ; Asthma* ; Biopsy ; Bronchi ; Bronchiectasis ; Cough ; Eosinophils ; Female ; Humans ; Immunoglobulin E ; Lung ; Methacholine Chloride ; Physical Examination ; Pneumonia ; Respiratory Sounds ; Skin ; Skin Tests ; Sputum ; Thorax

PURPOSE: Vitamin D deficiency is a public health problem in many countries. There has been a reappearance of rickets from vitamin D deficiency in recent decades as a result of multiple factors. One of the factors is breast feeding. The purpose of this study was to describe the clinical presentation of rickets in breastfed infants. METHODS: Retrospective review of patients presenting to Ajou University hospital between 2003 and 2008 with rickets caused by vitamin D deficiency during breast feeding. RESULTS: Seventeen patients (10 boys and 7 girls) were diagnosed with vitamin D deficiency. There were six in the asymptomatic and eleven in the symptomatic patients. The mean age of the patients was 8.5+/-0.5 months. The mean 25-hydroxycholecalciferol was 3.55+/-1.88 ng/mL. 25-hydroxycholecalciferol levels were below 5 ng/mL in 13 patients. The mean serum alkaline phosphatase was 765.53+/-563.9 IU/L, the mean intact parathyroid hormone was 231.6+/-225.7 pg/mL. All except 3 patients were showed cupping and fraying of metaphysis. CONCLUSION: Breast feeding is associated with increased risk of rickets. We recommend vitamin D supplementation of all breastfed infants to prevent rickets. Supplementation should begin within the first 2 months of life. Also, we hope to initiate further research and debate about guideline of vitamin D supplementation
Alkaline Phosphatase ; Breast Feeding ; Calcifediol ; Humans ; Infant ; Parathyroid Hormone ; Public Health ; Retrospective Studies ; Rickets ; Vitamin D ; Vitamin D Deficiency ; Vitamins