Leopard syndrome is a neurocutaneous syndrome which may affect a variety of organ systems in the embryological aspects. It presents lentigines, EKG abnormalities, ocular hypertelorism, pulmonary stenosis, abnormalities of genitalia, retardation of growth, and deafness as major teatures.We report a case of Leopard Syndrome with atypical psychotic features. The patient had many lentigenies on the face, cafe au lait spots on the part of the waist and the buttocks, mild atrial regugitation, clinodactyly, hyperextensibility of distal interphalangeal joint, flat foots, subclinical hypothyrodism, sensorineural deafness, and mild mental retardation. He also had autistic disorder, compulsion, pathologic collection, and violent behaviors as psychiatric problems. This raises a possibility that psychiatric diseases may be related to the variation of neuroectoderm. In clinical practice, when psychopathic patients with specific skin lesions are seen for consultation, the consideration of embryologically common aspects of both disease groups can be helpful to the discovery of abnormalities in other organs and to its treatment.
Autistic Disorder ; Buttocks ; Cafe-au-Lait Spots ; Deafness ; Electrocardiography ; Flatfoot ; Genitalia ; Humans ; Hypertelorism ; Intellectual Disability ; Joints ; Lentigo ; LEOPARD Syndrome* ; Neural Plate ; Neurocutaneous Syndromes ; Panthera* ; Psychotic Disorders* ; Pulmonary Valve Stenosis ; Skin

PURPOSE: The aim of this study is to investigate the reproducibility of the quantitative assessment of segmental wall motion and systolic thickening provided by an automatic quantitation algorithm. MATERIALS AND METHODS: Tc-99m-MIBI gated myocardial SPECT with dipyridamole stress was performed in 31 patients with known or suspected coronary artery disease (4 with single, 6 with two, 11 with triple vessel disease; ejection fraction 51+/-14%) twice consecutively in the same position. Myocardium was divided into 20 segments. Segmental wall motion and systolic thickening were calculated and expressed in mm and % increase respectively, using AutoQUANTTM software. The reproducibility of this quantitative measurement of wall motion and thickening was tested. RESULTS: Correlations between repeated measurements on consecutive gated SPECT were excellent for wall motion (r=0.95) and systolic thickening (r=0.88). On Bland-Altman analysis, two standard deviation was 2 mm for repeated measurement of segmental wall motion, and 20% for that of systolic thickening. The weighted kappa values of repeated measurements were 0.807 for wall motion and 0.708 for systolic thickening. Sex, perfusion, or segmental location had no influence on reproducibility. CONCLUSION: Segmental wall motion and systolic thickening quantified using AutoQUANTTM software on gated myocardial SPECT offers good reproducibility and is significantly different when the change is more than 2 mm for wall motion and more than 20% for systolic thickening.
Coronary Artery Disease ; Dipyridamole ; Humans ; Myocardium ; Perfusion ; Tomography, Emission-Computed, Single-Photon*

Sinus pericranii is a rare vascular anomaly that's characterized by an extracranial vascular mass with anastomotic connections between the intracranial and extracranial systems via the diploic veins of the skull. Preoperative evaluations for making the diagnosis are important to help prevent unexpected intraoperative bleeding. We report here on a case of surgically treated sinus pericranii in a 20-year-old female following minor head trauma. The clinical manifestations, pathogenesis and managements are discussed, and we also review the relevant literature.
Craniocerebral Trauma ; Female ; Hemorrhage ; Humans ; Sinus Pericranii ; Skull ; Veins ; Young Adult

No abstract available.
Aneurysm* ; Angiography* ; Circle of Willis*

A fenestrated middle cerebral artery (MCA) is a rare congenital anomaly, and is related to interference in the normal embryonic development of the MCA. Fenestrated MCA has been regarded to have no clinical significance other than a rare event of hemorrhage from associated aneurysm. However, the fenestration within the arterial trunk can be an obstacle against thrombus migration and may be associated with a major cerebral infarction. Moreover, the presence of this anomaly can be hardly detected prior to thrombolytic procedures, and emergent treatments are proceeded without any information of anatomical configurations. Therefore, the recanalization procedures would carry a high risk of intraprocedural complications. We report a rare case of MCA territory infarction from occlusion of fenestrated M1 segment, and also introduce a safe method of mechanical thrombolysis using coil.
Aneurysm ; Cerebral Infarction ; Embryonic Development ; Female ; Hemorrhage ; Infarction ; Mechanical Thrombolysis ; Middle Cerebral Artery ; Pregnancy ; Thrombosis

OBJECTIVE: Kyphoplasty and vertebroplasty are two minimally invasive procedures for osteoporotic vertebral compression fractures. The purpose of this retrospective study was to compare the radiological findings and clinical outcomes between two procedures. METHODS: Osteoporotic vertebral fractures were treated in 76 vertebrae, using kyphoplasty (n=35 vertebrae) and using vertebroplasty (n=41 vertebrae). Fractured vertebral bodies were diagnosed by correlating the clinical symptoms with radiologic study. The responses of pain symptoms were measured by a self-reported Visual Analog Scale (VAS) score. Plain X-rays were checked preoperatively and postoperatively at admission and 6 months. The vertebral body height and kyphotic angle were measured to assess the reduction of the sagittal alignment. RESULTS: The mean pain scores were decreased significantly for both procedures postoperatively, but there were no significant differences between two groups. Kyphoplasty led to a significant reduction of the vertebral body height and improvement of kyphotic angle. There were no neurological deficits after kyphoplasty, but one patient experienced paraparesis after vertebroplasty. During the 6 months follow-up both procedures provided stabilization of the sagittal alignment. CONCLUSION: Kyphoplasty and vertebroplasty are considered effective minimally invasive techniques for the stabilization of osteoporotic vertebral body fractures, leading to a statistically significant reduction in pain. Kyphoplasty significantly restore sagittal alignment. Also, complications and the incidence of bone cement leakage are significantly lesser than vertebroplasty. Therefore, kyphoplasty seems to be reasonable procedure for osteoporotic vertebral body compression fractures when medical treatment fail.
Body Height ; Follow-Up Studies ; Fractures, Compression* ; Humans ; Incidence ; Kyphoplasty* ; Kyphosis ; Osteoporosis ; Paraparesis ; Retrospective Studies ; Spine ; Vertebroplasty* ; Visual Analog Scale

OBJECTIVE: Electrodiagnostic test has shown diagnostic sensitivity and specificity in carpal tunnel syndrome(CTS). This study was to evaluate the correlation between clinical outcome of endoscopic carpal tunnel ligament release(ECTR) and the predictive value of sensory nerve conduction. METHODS: From January 1998 to December 2004, 87 patients (44 right hand, 37 left hand, 6 bilateral hands) with CTS who underwent ECTR were followed up in our hospital for an average of 2.4 months. We retrospectively analyzed the results with previous medical records. All patients underwent electrodiagnostic test and ECTR. The patients were divided into three groups according to the electrodiagnostic test results. Group (A) was normal sensory nerve response, Group (B) was slowing sensory response and Group (C) was no sensory response. Improvement of the symptom after ECTR was assessed using a visual analogue scale(VAS) score. RESULTS: Differences between the three groups on the correlation of severity of sensory potential and duration of preoperative symptoms were significant. The mean value of improved VAS scores for the three groups were 6.0+/-0.96 in the Group A, 6.11+/-0.48 in the Group B and 6.14+/-0.53 in the Group C. There was no statistically significant difference between the severity of sensory nerve response and improvement in VAS score after ECTR. Complications included a wound infection, a case of skin necrosis, and two patients with persistent symptoms without any improvement. CONCLUSION: Although electrodiagnostic test has been known to be useful, sensory nerve response is considered not to be a good prognostic value for carpal tunnel syndrome after ECTR.
Carpal Tunnel Syndrome* ; Hand ; Humans ; Ligaments ; Medical Records ; Necrosis ; Neural Conduction* ; Retrospective Studies ; Sensitivity and Specificity ; Skin ; Wound Infection

OBJECTIVE: Microvascular decompression(MVD) at root exit zone(REZ) of the facial nerve has been largely popularized and it has become the standard treatment for patients with hemifacial spasm(HFS). This sturdy is performed to evaluate the efficacy of MVD over the course of time. METHODS: From 1994 to 2003, 50 patients with HFS who underwent MVD were followed up for more than 6 months. We retrospectively analyzed results with medical records and telephone researches. RESULTS: The mean age of patients at the time operation was 57.6 years and 84% of the patients were female. The mean duration of follow-up after operation was 3.4 years (range 0.5~7.8 years). One day after MVD, 54% of patients had complete relief of spasm immediately. Continuous improvements of HFS were observed during the follow-up period and these improvements were statistically significant with time (P<0.05). Until 6 months after operation, complete relief of spasm was observed in 84% of patients. The delayed relief of spasm was observed in 35.7% of our patients who experienced complete relief. CONCLUSION: The efficacy of MVD in HFS is improves with time. Continuous follow-up evaluations for the duration of more than at least 6 months after MVD are important for the decision of its clinical results because delayed relief of spasms occurs.
Facial Nerve ; Female ; Follow-Up Studies ; Hemifacial Spasm* ; Humans ; Medical Records ; Microvascular Decompression Surgery* ; Retrospective Studies ; Spasm ; Telephone

OBJECTIVE: There have been numerous follow-up studies of patients who had ruptured or unruptured intracranial aneurysms treated by wrapping technique using various materials have been reported. Our objective was to ascertain whether our particular wrapping technique using the temporalis muscle provides protection from rebleeding and any aneurysm configuration changes in follow-up studies. METHODS: Clinical presentation, the location and shape of the aneurysm, outcomes at discharge and last follow-up, and any aneurysm configuration changes on last angiographic study were analyzed retrospectively in 21 patients. Reinforcement was acquired by clipping the wrapped temporalis muscle. Wrapping and clipping after incomplete clipping was also done. Follow-up loss and non-angiographic follow-up patient groups were excluded in this study. RESULTS: The mean age was 53 years (range 29-67), and 15 patients were female. Among 21 patients, 10 patients had ruptured aneurysms (48%). Aneurysms in 21 patients were located in the anterior circulation. Aneurysm shapes were broad neck form (14 cases), fusiform (1 case), and bleb to adjacent vessel (6 cases). Five patients were treated by clipping the wrapped temporalis, and 16 patients by wrapping after partial clipping. The mean Glasgow coma scale (GCS) at admission was 14.2. The mean Glasgow outcome scale (GOS) at discharge was 4.8, and 18 patients were grade 5. The mean period between initial angiography and last angiography was 18.5 months (range 8-44). Aneurysm size was not increased in any of these patients and configuration also did not change. There was no evidence of rebleeding in any of these treated aneurysms. CONCLUSION: Our study results show that wrapping technique, using the temporalis muscle and aneurysm clip(s), for intracranial aneurysm treatment provides protection from rebleeding or regrowth.
Aneurysm ; Aneurysm, Ruptured ; Angiography ; Blister ; Female ; Follow-Up Studies ; Glasgow Coma Scale ; Glasgow Outcome Scale ; Glycosaminoglycans ; Humans ; Intracranial Aneurysm ; Muscles ; Neck ; Reinforcement (Psychology) ; Retrospective Studies ; Temporal Muscle

No abstract available.
Humans ; Langerhans Cells* ; Prognosis*