Menkes disease is an X-linked recessively inherited neurodegenerative disorder of copper metabolism leading to death in early childhood. This disease is characterized by low serum, liver and brain Cu levels, whereas the Cu content in nearly all other organs is increased. The main clinical features are irregular kinky hair, characteristic face, progresssive degeneration of the central nervous system, bone changes, arterial rupture and thrombosis, and instability of temperature. We have experienced a case of Menkes disease in a eight months old male patient, who manifested by seizure, developmental delay, hypotonia, characteristic hair and face along with low levels of serum copper and ceruloplasmin. We report the characteristic findings of cranial MRI and MR angiography in this Menke's patient. In cranial MRI, he showed progressive brain atrophy with subdural hemorrhage and effusion. MR angiography revealed tortuosity of vessels clearly obviating necessity of more invasive conventional arteriography in diagnosis of this disease. This study suggested that cranial MRI and especially MR angiography might be helpful in making early diagnosis of this disease.
Angiography ; Atrophy ; Brain ; Central Nervous System ; Ceruloplasmin ; Copper ; Diagnosis ; Early Diagnosis ; Hair ; Hematoma, Subdural ; Humans ; Liver ; Magnetic Resonance Imaging ; Male ; Menkes Kinky Hair Syndrome ; Metabolism ; Muscle Hypotonia ; Neurodegenerative Diseases ; Rupture ; Seizures ; Thrombosis

We report 2 cases of pediatric DNTs which presented with intractable seizures and no other associated neurologic abnormalities. They showed typical appearance of DNTs on neuroimaging and histopathology. Most patients with DNT can be cured by surgical treatment with exellent outcome and do not need ratio- or chemotheraphy. This study indicates that it is quite important to consider DNTs as one of differential diagnoses in patients with intractable seizures especially when they present only with seizures without other neurologic symptoms.
Child* ; Diagnosis, Differential ; Humans ; Neoplasms, Neuroepithelial* ; Neuroimaging ; Neurologic Manifestations ; Seizures*

No abstract available.
Otoacoustic Emissions, Spontaneous*

Background: The pain caused by local anesthetic injection can lead to patient anxiety prior to surgery, potentially necessitating sedation or general anesthesia during the excision procedure. In this study, we aim to compare the pain relief efficacy and safety of using a digital automatic anesthetic injector for local anesthesia. Methods: Thirty-three patients undergoing excision of a benign soft tissue tumor under local anesthesia were prospectively enrolled from September 2021 to February 2022. A single-blind, randomized controlled study was conducted. Patients were divided into two groups by randomization: the experimental group with digital automatic anesthetic injector method (I-JECT group) and the control group with conventional injection method. Before surgery, the Amsterdam preoperative anxiety information scale was used to measure the patients’ anxiety. After local anesthetic was administered, the Numeric Pain Rating Scale was used to measure the pain. The amount of anesthetic used was divided by the surface area of the lesion was recorded. Results: Seventeen were assigned to the conventional group and 16 to the I-JECT group. The mean Numeric Pain Rating Scale was 1.75 in the I-JECT group and 3.82 in conventional group. The injection pain was lower in the I-JECT group (p< 0.01). The mean Amsterdam preoperative anxiety information scale was 11.00 in the I-JECT group and 9.65 in conventional group. Patient’s anxiety did not correlate to injection pain regardless of the method of injection (p= 0.47). The amount of local anesthetic used per 1 cm 2 of tumor surface area was 0.74 mL/cm2 in the I-JECT group and 2.31 mL/cm2 in the conventional group. The normalization amount of local anesthetic was less in the I-JECT group (p< 0.01). There was no difference in the incidence of complications. Conclusion The use of a digital automatic anesthetic injector has shown to reduce pain and the amount of local anesthetics without complication.

PURPOSE: In the Spring 1995, there was an outbreak of adenoviral infection, which caused four death out of ten patients with adenoviral pneumonia in our hospital. Clinical courses of ten patients with severe pneumonia were similar each other, and two were confirmed as adenoviral pneumonia by postmortem autopsy. Although not proven, we believe eight patients had adenoviral pneumonia. Therefore, we report clinical features in ten cases of severe adenoviral pneumonia. METHODS: Two cases with adenoviral pneumonia and eight cases with presumed adenoviral pneumonia were admitted in this hospital from March to June, 1995. Age and sex distribution, clinical manifestations, laboratory data, chest X-ray findings were reviewed. RESULTS: They were young children between 4 to 25 months of age(mean 12.7+/-6.1 months), and male to female ratio was 9:1. They presented with abrupt fever, cough, tachypnea, and dyspnea. Mean duration of fever were 12.7+/-6.1 days. Crackles on auscultation were heard in all patients. Studies for Mycoplasma and Tuberculosis were all negative. Cultures of bacteria and fungi were negative, and they did not respond to the antibiotics. The chest X-ray revealed the diffuse lobar consolidation with varying amount of pleural effusions. The findings of pleural fluid showed characteristics of transudate with predominant monocyte. Eight of our severe adenoviral pneumonia patients were enjoying normal health previously. Only two patients had previous medical problems, one with chronic cytomegalovirus pneumonia and the other with neutropenia induced by phenobarbital. The course of illness suggests that the infection was hospital acquired and the final outcome was fatal. Three of them developed seizure with fever, five change of consciousness, four conjunctivitis, three otitis media, and two gastro-intestinal symptoms. Autopsy was done in two of four patients. Grossly, the lungs were heavy and dark- red in color. There were bilateral pneumonic consolidation with patchy areas of hemorrhage. Microscopically, severe necrotizing bronchitis and bronchiolitis with numerous intranuclear inclusion of Cowdry type A and B were found. Alveoli were edematous and filled with fibrinous exudate, and covered with hyaline membrane. Ultrastructurally, typical adenoviral particles showing hexagonal shape in paracrystalline array symmetry were found in the nucleus of aleveolar lining cells. CONCLUSIONS: Yet, occasionally, adenoviral infection becomes most aggressive form of pneumonia. We should consider adenoviral pneumonia when clinical findings of pneumonia are very similar with baterial pneumonia except poor response to broad spectrum antibiotics. There is no specific treatment for adenoviral infection. So, for prevention of adenoviral pneumonia, we recommend isolation in suspicious adenoviral infection.
Adenoviridae ; Anti-Bacterial Agents ; Auscultation ; Autopsy ; Bacteria ; Bronchiolitis ; Bronchitis ; Child ; Conjunctivitis ; Consciousness ; Cough ; Cytomegalovirus ; Dyspnea ; Exudates and Transudates ; Female ; Fever ; Fibrin ; Fungi ; Hemorrhage ; Humans ; Hyalin ; Intranuclear Inclusion Bodies ; Lung ; Male ; Membranes ; Monocytes ; Mycoplasma ; Neutropenia ; Otitis Media ; Phenobarbital ; Pleural Effusion ; Pneumonia* ; Respiratory Sounds ; Seizures ; Sex Distribution ; Tachypnea ; Thorax ; Tuberculosis

OBJECTIVE: The incidence rate of stroke as a result of intracranial arterial stenosis (ICAS) is higher in Asian countries than in the West. We aimed to analyze the regression, lack of change, or progression of asymptomatic ICAS after the administration of rosuvastatin and associated factors.METHODS: The patients who had undergone computed tomography angiography (CTA) at our hospital and had been diagnosed with ICAS with no ischemic event in the stenosed vascular territory were included in the study. They were administered 20mg of rosuvastatin per day. After a follow-up period of at least 6 months after treatment, the patients were examined using CTA again and the clinical information and imaging results were analyzed.RESULTS: In total, 48 patients were diagnosed with asymptomatic ICAS. During the final follow-up examination, it was found that the stenotic lesion regressed in 30 patients, whereas it remained unchanged or progressed without any adverse effects in 18 patients. In univariate analysis, the regressed group showed significantly higher differences in the levels of total cholesterol and low-density lipoprotein (LDL) between their initial and final values (both, p=0.031 for both). In the multivariate analysis, a significantly higher difference in the levels of LDL between its initial and final measurement was seen in the regressed group (p=0.035, odds ratio(OR) 3.9).CONCLUSIONS: Rosuvastatin was found to have better lipid-lowering effects for total cholesterol and particularly LDL in patients whose ICAS had regressed. We concluded that rosuvastatin administration can be recommended for the treatment of patients with asymptomatic ICAS.
Angiography ; Asian Continental Ancestry Group ; Atherosclerosis ; Cholesterol ; Constriction, Pathologic ; Follow-Up Studies ; Humans ; Hydroxymethylglutaryl-CoA Reductase Inhibitors ; Incidence ; Lipoproteins ; Multivariate Analysis ; Rosuvastatin Calcium ; Stroke

PURPOSE: An attempt was made to determine if the pre-LASIK operative BAC-STAT LASIK Ring plays a role in reducing postoperative infection. METHODS: Candidates for this study were 72 eyes of 36 patients, scheduled to undergo LASIK operation. Eyes were divided into two groups: right eyes with preoperative BAC-STAT bacteriostatic LASIK Ring (BAC-STAT LASIK Ring: American Optisurgical Inc., Lake Forest, California, U.S.A.) and one minute of irrigation (experimental group), and left eyes with only preoperative BSS irrigation (control group). The authors tried to demonstrate a difference of identified pathogens between the two groups after a growth of bulboconjunctival lesion in blood agar plate (BAP), MacConkey agar plate (MCA), Thioglycolate medium broth (TG), and Ogawa egg medium. RESULTS: Among the patients receiving only BSS irrigation, pathogens were identified in eight eyes before surgery and in four eyes after surgery in BAP and MCA medium. Among the patients receiving BAC-STAT LASIK Ring and irrigation, pathogens were identified in nine eyes before surgery and in three eyes after surgery in BAP and MCA culture medium. In addition, among the control population, pathogens were found in 21 eyes both before and after surgery in TG culture medium. However, among the experimental population, pathogens were grown in 23 eyes before surgery but in only 14 eyes after surgery. No growth of pathogens was reported in all patients before and after surgery in a 4-week Ogawa egg culture medium. CONCLUSIONS: This study suggests that preoperative BAC-STAT LASIK Ring insertion followed by irrigation decreases the incidence of postoperative infection.
Agar ; California ; Humans ; Incidence ; Keratomileusis, Laser In Situ* ; Lakes ; Ovum ; Thiram

OBJECTIVES: To compare the urodynamic characteristics between pre- and post-menopausal women with stress urinary incontinence. METHODS: Forty premenopausal women and 44 postmenopausal women with stress urinary incontinenece were enrolled. All of the patients underwent a detailed history, gynaecologic examination, urinalysis and urodynamics including cystometry and pressure-flow analysis. RESULTS: There was no difference in the body mass index between the two groups. The mean age of pre- and post-menopausal women was 40.9 +/- 6.4 years and 62.1 +/- 9.4 years, respectively. The parity in postmenopausal women was greater than premenopausal women (2.8 +/- 1.2 vs 2.2 +/- 0.8, P = 0.026). The valsalva leak point pressure (VLPP) in postmenopausal women was lower than that in premenopausal women (106.4 +/- 30.3 vs 88.2 +/- 25.1 cmH2O, P = 0.04). The maximal urethral closure pressure (MUCP) in postmenopausal women was lower than premenopausal women (44.9 +/- 21.4 vs 77.4 +/- 32.2 cmH2O, P < 0.001). The functional urethral length (FUL) in postmenopausal women was shorter than it in premenopausal women (29.0 +/- 9.7 vs 37.0 +/- 10.9 mm, P = 0.003). There were no significant statistical differences in maximal flow rate, residual urine, maximal bladder capacity and Q tip test. CONCLUSION: The VLPP and MUCP were lower, and the FUL was shorter in postmenopausal women with stress urinary incontinence than premenopausal women. The parity and number of vaginal deliveries were different between the two groups. Further investigation will be needed concerning these variables.
Body Mass Index ; Female ; Humans ; Parity ; Postmenopause ; Premenopause ; Retrospective Studies ; Urinalysis ; Urinary Bladder ; Urinary Incontinence ; Urodynamics

PURPOSE: To analyze the MR findings of cyclosporine-induced neurotoxicity in patients receiving high dose ofcyclosporine and to suggest the possible pathogenetic mechanism. MATERIALS AND METHODS: The cases of seven patients (2 males, 5 females ; 18-36 years old) who suffered seizures after receiving high-dose cyclosporine for bone marrow transplantation due to diseases such as a plastic anemia or leukemia were retrospectively reviewed. We evaluated the location and pattern of abnormal signal intensity seen on T2 weighted images, the presence of contrast enhancement, and the changes seen on follow-up MR performed at intervals of 12-30 days after initial MR in five of seven patients. We analyzed levels of blood cyclosporine and magnesium, and investigated the presence of hypertension at the site of the seizure. RESULTS: Locations of the lesions were bilateral(n=5),unilateral(n=2), parietal(n=6), occipital(n=6), temporal(n=4), and in the frontal lobe(n=3). Frontal lesions showed high signal intensities in the borderline ischemic zone of the frontal lobe between the territory of the anterior and middle cerebral arteries. In six of the seven patients, cortical and subcortical areas including subcortical U-fibers were seen on T2-weighted images to be involved in the parietooccipital lobes. Only one of the seven showed high signal intensity in the left basal ganglia. All lesions showed high signal intensity onT2-weighted images, and iso to low signal intensity on T1-weighted. In five of seven patients there was nodefinite enhancement, but in the other two, enhancement was slight. In four of seven patients seizures occurred within high therapeutic ranges (250 - 450 ng/ml), while others suffered such attacks at levels below the therapeutic range. After cyclospirine was administered at a reduced dosage or stopped, follow-up MR images showed the complete or near-total disappearance of the abnormal findings previously described. Only two patients had hypertension, and the others normotension. Five of the seven had hypomagnesemia(1.3 -1.74 mg/dl; N : 1.9 -3.1mg/dl). CONCLUSION: Most patients with cyclosporine neurotoxicity showed high signal intensity in the corticaland subcortical areas of the parietooccipital lobes, including subcortical U-fiber, as seen on T2 weighted images,and no abnormal enhancement after Gd-DTPA injection. These MR findings should be helpful for the diagnosis of cyclosporine neurotoxicity.
Anemia ; Basal Ganglia ; Bone Marrow Transplantation ; Cyclosporine* ; Diagnosis ; Female ; Follow-Up Studies ; Frontal Lobe ; Gadolinium DTPA ; Humans ; Hypertension ; Leukemia ; Magnesium ; Male ; Middle Cerebral Artery ; Plastics ; Retrospective Studies ; Seizures

Cholesteatoma is common disease entity within the middle ear cavity but is rarely found in the paranasal sinuses, especially the maxillary sinus. We experienced a case of cholesteatoma of the maxillary sinus without history of previous trauma or operation. The patient was not improved by functional endoscopic sinus surgery. The mucosa of the maxillary sinus was removed through the Caldwell-Luc approach, and heavy saline irrigation was performed. After reoperation, the postoperative period was uneventful, and there was no sign of recurrence on endoscopic examination.
Cholesteatoma ; Ear, Middle ; Humans ; Maxillary Sinus ; Mucous Membrane ; Paranasal Sinuses ; Postoperative Period ; Recurrence ; Reoperation