The posterior fossa arterivenous malformation(AVM) is uncommon and different from other intracranial AVM in its natural history, diagnosis, treatment, prognosis, and other features. The authors present 3 cases of posterior fossa AVM. A 26-year-old woman with supravermian AVM and a 17-year-old woman with right cerebellopontine angle AVM were treated with surgical removal in good result. And another was occurred in the pons of 20-year-old woman, that was treated with intersecting beam radiation of 5040 rads in cumulative dosage.
Adolescent ; Adult ; Arteriovenous Malformations* ; Cerebellopontine Angle ; Diagnosis ; Female ; Humans ; Natural History ; Pons ; Prognosis ; Young Adult

Yakovlev demonstrated that the Schizencephalies appear to result from injury to developing cerebral vesicles in the second month of intrauterine life. There is a total loss of neurons in restricted symmetrical zones of each developing hemisphere and on either side of the resulting cleft-shaped defects are evidences of subtotal injury to developing neuroblasts. A case of a 4 years old male with Schizencephaly is reported.
Child, Preschool ; Humans ; Male ; Malformations of Cortical Development* ; Neurons

Pseudohypoparathyroidism (PHP) is a rare clinical type of hypoparathyroidism. The patients with PHP show classic clinical and biochemical features of hypoparathyroidism, but elevated serum level of parathyroid hormone (PTH) and characteristic physical appearances termed 'Albright's hereditary osteodystrophy' (AHO). PHP is classified into types Ia, Ib, Ic and II according to the presence of AHO phenotype and the mechanism of PTH resistance. We experienced a case of PHP in a 12 year-old girl with carpopedal spasm, syncope and partial AHO. She showed very low serum calcium level (1.4 mmol/L), high phosphorus level (3.62 mmol/L) and high immunoreactive PTH level (186.6 ng/L). In the Ellsworth-Howard test, urinary cyclic adenosine monophosphate and phosphorus levels after an exogenous PTH injection remained unchanged. Therefore, we were able to classify the patient as either PHP type Ia or Ic. After the patient had been treated with daily calcium carbonate (1 g), cholecalciferol (250 IU) and calcitriol (0.5 microgram), her neurological signs and symptoms as well as her biochemical abnormalities of hypocalcemia and hyperphosphatemia were improved.
Adenosine Monophosphate ; Calcitriol ; Calcium ; Calcium Carbonate ; Child ; Cholecalciferol ; Female ; Humans ; Hyperphosphatemia ; Hypocalcemia ; Hypoparathyroidism ; Parathyroid Hormone ; Phenotype ; Phosphorus ; Pseudohypoparathyroidism* ; Spasm ; Syncope

Pseudohypoparathyroidism (PHP) is a rare clinical type of hypoparathyroidism. The patients with PHP show classic clinical and biochemical features of hypoparathyroidism, but elevated serum level of parathyroid hormone (PTH) and characteristic physical appearances termed 'Albright's hereditary osteodystrophy' (AHO). PHP is classified into types Ia, Ib, Ic and II according to the presence of AHO phenotype and the mechanism of PTH resistance. We experienced a case of PHP in a 12 year-old girl with carpopedal spasm, syncope and partial AHO. She showed very low serum calcium level (1.4 mmol/L), high phosphorus level (3.62 mmol/L) and high immunoreactive PTH level (186.6 ng/L). In the Ellsworth-Howard test, urinary cyclic adenosine monophosphate and phosphorus levels after an exogenous PTH injection remained unchanged. Therefore, we were able to classify the patient as either PHP type Ia or Ic. After the patient had been treated with daily calcium carbonate (1 g), cholecalciferol (250 IU) and calcitriol (0.5 microgram), her neurological signs and symptoms as well as her biochemical abnormalities of hypocalcemia and hyperphosphatemia were improved.
Adenosine Monophosphate ; Calcitriol ; Calcium ; Calcium Carbonate ; Child ; Cholecalciferol ; Female ; Humans ; Hyperphosphatemia ; Hypocalcemia ; Hypoparathyroidism ; Parathyroid Hormone ; Phenotype ; Phosphorus ; Pseudohypoparathyroidism* ; Spasm ; Syncope

BACKGROUND: Since the winter of 1997, Korea has been in an economic crisis. During this period family-the most important and basic social unit- faoed many problems. The purpose of this survey was to assess Korean family's functional status and emphasize family as a social support unit. METHODS: During April- May 1998 Nationwide Telephone survey was done. Sampling was done by Multi State Stratified Random Sampling technique. We questioned subjects on 5 categories of present family problems and used Modified Faces -III Questionnaire for the evaluation of family function. RESULTS: By 5 point scale, the impact of present ecanomic problems to the family showed the highest score(3.7). Other causes had a score range of 1.7-2.1. Economic impact was greater in 4th and 5th life cycle step, those with low income, and those in bereavement. Family type was divided in to 3 groups; extreme type 20%, mid-range 50%, and balanced 30%. Among extreme groups chaotic adoption and enmeshed cohesion type were the most common(10.8%). CONCLUSIONS: In the Korean family 1998, economic problem seems to be the most important impact factor and the proportion of extreme type family is high.
Bereavement ; Humans ; Korea ; Life Cycle Stages ; Telephone ; Surveys and Questionnaires

BACKGROUND: There have been many in vitro evidences that interleukin-4(IL-4) might be the most important cytokine inducing IgE synthesis from B-cells, and interferon-gamma(IFN-gamma) might be a main cytokine antagonizing IL-4-mediated IgE synthesis. Recently some reports demonstrated that IFN-gamma might be used as a new therapeutic modality in some allergic diseases with high serum IgE level, such as atopic dermatitis or bronchial asthma. To evaluate the in vivo, effect of IFN-gamma in bronchial asthma we tried a clinical study. METHODS: Fifty bronchial asthmatics(serum, IgE level over 200 IU/ml) who did not respond to inhaled or systemic corticosteroid treatment, and 17 healthy nonsmoking volunteers were included in this study. The CD 23 expressions of peripheral B-cells, the IL-4 activities of peripheral T-cells, the serum soluble CD23(sCD23) levels, and the superoxide anton(O2-) generations by peripheral PMN were compared between bronchial asthmatics and normal subjects. The IL-4 activities of peripheral T-cells were analyzed by T-cell supernatant (T-sup)-induced CD23 expression from tonsil B-cells. In bronchial asthmatics the serum IgE levels and histamine PC. in addition to the above parameters were also compared before and after IFN-gamma treatment. IFN-gamma was administered subcutaneously with a weekly dose of 30,000 IU per kilogram of body weight for 4 weeks. RESULTS: The O2-, generations by peripheral PMNs in bronchial asthmatics were higher than normal subjects(8.23+/-0.94 vs 5.00+/-0.68 nmol/l x10(6) cells, P<0.05), and significantly decreased after IFN-gamma treatment compared to initial values(3.69+/-0.88 vs 8.61 +/- 1.53 nmol/l x 106 cells, P<0.05). CD23 expression of peripheral B-cells in bronchial asthmatics was higher than normal subjects(47.47 +/- 2.96% vs 31.62+/-1.92%, P<0.05), but showed no significant change after IFN-gammatreatment. The serum sCD23 levels in bronchial asthmatics were slightly higher than normal subjects( 191.04+/-23.3 U/ml vs 162.85+/-4.85 U/ml). and 11(64.7%) of 17 patients showed a decreasing pattern in their serum sCD23 levels after IFN-gamma treatment. However the means of serum sCD23 levels were not different before and after IFN-gamma treatment The IL-4 activities of peripheral T-cells in bronchial asthmatics were slightly higher than normal subjects 22.48 +/-6.81% vs 18.90+/-2.43%), and slightly increased after IFN-gamma treatment(27.90+/-2.56%). Nine(60%) of 15 patients showed a decreasing pattern in their serum IgE levels after IFN-gamma treatment. And the levels of serum IgE were significantly decreased after LEN-y treatment compared to initial values (658.67 +/- 120.84 IU/ml vs 1394.32 +/- 31442 IU/ml, P<0.05). Ten(83.3%) of 12 patients showed an improving pattern in bronchial hyperresponsiveness after IFN-gamma treatment, and the means of histamine PC20 were significantly increased after IFN-gamma treatment compared to initial values (1.22+/-0.29mg/ml vs 0.69+/-0.17mg/ml P<0.05). CONCLUSION: Our results suggest that IFN-gamma may k useful as well as safety in the treatment of bronchial asthmatics with high serum IgE level and that in vivo effects of IFN-gamma may be different from its in vitro effects on the regulations of IgE synthesis or the respiratory burst of PMN.
Asthma* ; B-Lymphocytes ; Body Weight ; Dermatitis, Atopic ; Family Characteristics ; Histamine ; Humans ; Immunoglobulin E ; Interferon-gamma* ; Interleukin-4 ; Palatine Tonsil ; Respiratory Burst ; Social Control, Formal ; Superoxides ; T-Lymphocytes ; Volunteers

The neurological manifestations caused by Epstein-Barr virus (EBV) occur only rarely in association with its primary infection or reactivation. The mechanism by which EBV produces neurological disease is unknown. This article describes two cases of polymerase-chain-reaction-proven EBV brainstem encephalitis. The sera of both patients contained autoantibodies against N-methyl-D-aspartate receptor (NMDAR), suggesting the presence of a secondary immunological mechanism. Prospective studies are needed to reveal whether the subgroup of patients with EBV encephalitis and anti-NMDAR antibodies have different clinical presentations and would benefit from immunotherapy.
Antibodies ; Autoantibodies ; Brain Stem ; Encephalitis ; Herpesvirus 4, Human ; Humans ; Immunotherapy ; N-Methylaspartate ; Neurologic Manifestations

The neurological manifestations caused by Epstein-Barr virus (EBV) occur only rarely in association with its primary infection or reactivation. The mechanism by which EBV produces neurological disease is unknown. This article describes two cases of polymerase-chain-reaction-proven EBV brainstem encephalitis. The sera of both patients contained autoantibodies against N-methyl-D-aspartate receptor (NMDAR), suggesting the presence of a secondary immunological mechanism. Prospective studies are needed to reveal whether the subgroup of patients with EBV encephalitis and anti-NMDAR antibodies have different clinical presentations and would benefit from immunotherapy.
Antibodies ; Autoantibodies ; Brain Stem ; Encephalitis ; Herpesvirus 4, Human ; Humans ; Immunotherapy ; N-Methylaspartate ; Neurologic Manifestations

BACKGROUND AND PURPOSE: Anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis is the most common type of autoimmune synaptic encephalitis and it often responds to treatment. We analyzed the clinical characteristics of anti-NMDAR encephalitis in Korea. METHODS: Serum and/or cerebrospinal fluid (CSF) of adult patients (aged > or =18 years) with encephalitis of undetermined cause were screened for anti-NMDAR antibodies using a cell-based indirect immunofluorescence assay. The patients came from 41 university hospitals. RESULTS: Of the 721 patients screened, 40 were identified with anti-NMDAR antibodies and clinical details of 32 patients were obtained (median age, 41.5 years; 15 females). Twenty-two patients (68.8%) presented with psychiatric symptoms, 16 (50%) with seizures, 13 (40.6%) with movement disorders, 15 (46.9%) with dysautonomia, 11 (34.4%) with memory disturbance, and 11 (34.4%) with speech disturbance. Magnetic resonance imaging, electroencephalography, and CSF examinations yielded nonspecific findings. Tumor information was only available for 22 patients: 5 patients had tumors, and 2 of these patients had ovarian teratomas. Twenty-two patients received immunotherapy and/or surgery, and therapeutic responses were analyzed in 21 patients, of which 14 (66.7%) achieved favorable functional outcomes (score on the modified Rankin Scale of 0-2). CONCLUSIONS: This study investigated the clinical characteristics of adult anti-NMDAR encephalitis in Korea. Currently, elderly patients who do not have tumors are commonly diagnosed with this condition. Understanding the detailed clinical characteristics of this disease will improve the early detection of anti-NMDAR encephalitis in patients both young and old.
Adult ; Aged ; Anti-N-Methyl-D-Aspartate Receptor Encephalitis* ; Antibodies ; Cerebrospinal Fluid ; Electroencephalography ; Encephalitis ; Fluorescent Antibody Technique, Indirect ; Hospitals, University ; Humans ; Immunotherapy ; Korea ; Magnetic Resonance Imaging ; Memory ; Movement Disorders ; Primary Dysautonomias ; Seizures ; Teratoma

Background: and PurposeThe first-line medications for the symptomatic treatment of rapid eye movement sleep behavior disorder (RBD) are clonazepam and melatonin taken at bedtime. We aimed to identify the association between depression and treatment response in patients with idiopathic RBD (iRBD). Methods: We reviewed the medical records of 123 consecutive patients (76 males; age, 66.0±7.7 years; and symptom duration, 4.1±4.0 years) with iRBD who were treated with clonazepam and/or melatonin. Clonazepam and melatonin were initially administered at 0.25–0.50 and 2 mg/day, respectively, at bedtime, and the doses were subsequently titrated according to the response of individual patients. Treatment response was defined according to the presence or absence of any improvement in dream-enacting behaviors or unpleasant dreams after treatment. Results: Forty (32.5%) patients were treated with clonazepam, 56 (45.5%) with melatonin, and 27 (22.0%) with combination therapy. The doses of clonazepam and melatonin at followup were 0.5±0.3 and 2.3±0.7 mg, respectively. Ninety-six (78.0%) patients reported improvement in their RBD symptoms during a mean follow-up period of 17.7 months. After adjusting for potential confounders, depression was significantly associated with a negative treatment response (odds ratio=3.76, 95% confidence interval=1.15–12.32, p=0.029). Conclusions We found that comorbid depression is significantly associated with a negative response to clonazepam and/or melatonin in patients with iRBD. Further research with larger numbers of patients is needed to verify our observations and to determine the clinical implications of comorbid depression in the pathophysiology of iRBD.