PURPOSE: To observe MR findings of vascular abnormality in Sturge-Weber syndrome and to determine the value of MRI in diagnosis. MATERIALS AND METHODS: Ten patients with Sturge-Weber syndrome (age : 3 months-32 years)were evaluated by MR imaging ; in six and four cases, respectively, the results were correlated with those of CT and angiography. We retrospectively analysed changes in the cortical vein and deep venous system, including the medullary and subependymal vein, as well as an largement of the choroid plexus, leptomeningeal enhancement, and changes in diploic space. RESULTS: In all cases except one, in which non-contrast enhanced study had been performed, varying degrees of leptomeningeal enhancement were seen. In nine cases the cortical vein became smaller; enlargement of the choroid plexus was seen in eight cases, change in the diploic space in seven (including three in which there was angiomatous involvement), and enlargement of the deep venous system in seven. In younger patients, collateral pathways were less developed and leptomeningeal angiomatous changes were more pronounced than in those who were older. CONCLUSION: MRI is a useful modality for the evaluationn of vascular changes in Sturge-Weber syndrome. These changes vary according to a patient's age and the duration of the disease.
Angiography ; Choroid Plexus ; Diagnosis ; Humans ; Magnetic Resonance Imaging ; Retrospective Studies ; Sturge-Weber Syndrome* ; Veins

Although light microscopic features of muscle are not pathognomonic in most cases of myasthenia gravis (MG), careful examination of neuromuscular junction by electron microscopy (EM) can reveal important clues for this disease. We report here a case of MG confirmed by EM study to emphasize that tissue diagnosis is still the best adjuvant to confirm the diagnosis. An 18-year-old female visited our hospital complaining of progressive muscle weakness for 3 years. She had difficulty in running, going upstairs and doing routine activities. Symptoms were aggravated with continuous work and resolved after rest. She had weakness of bilateral masseter and facial muscles and proximal portions of extremities without definite diurnal variation. Electromyography showed myopathic changes in proximal muscles of extremities. MG was considered but tensilon test was equivocal. Repetitive nerve stimulation tests revealed 20-30 percent decrease in responses to low and high rate stimulation. Muscle biopsy revealed selective type 2 atrophy. Ultrastructurally, abnormalities of neuromuscular junctions, i.e., wide primary synaptic cleft, and wide and shallow secondary synaptic clefts with mild myopathic features were present. These findings were pathognomonic for MG. Later, her symptoms were improved completely 3 months after thymectomy. The histologic finding of thymus was follicular hyperplasia.
Adolescence ; Biopsy ; Case Report ; Female ; Human ; Microscopy, Electron ; Mitochondria/ultrastructure ; Mitochondria/pathology ; Muscle, Skeletal/ultrastructure ; Muscle, Skeletal/pathology ; Muscle, Skeletal/enzymology ; Myasthenia Gravis/pathology* ; Myofibrils/ultrastructure ; Myofibrils/pathology ; Myosin ATPase/analysis ; Neuromuscular Junction/ultrastructure* ; Neuromuscular Junction/pathology*

Although light microscopic features of muscle are not pathognomonic in most cases of myasthenia gravis (MG), careful examination of neuromuscular junction by electron microscopy (EM) can reveal important clues for this disease. We report here a case of MG confirmed by EM study to emphasize that tissue diagnosis is still the best adjuvant to confirm the diagnosis. An 18-year-old female visited our hospital complaining of progressive muscle weakness for 3 years. She had difficulty in running, going upstairs and doing routine activities. Symptoms were aggravated with continuous work and resolved after rest. She had weakness of bilateral masseter and facial muscles and proximal portions of extremities without definite diurnal variation. Electromyography showed myopathic changes in proximal muscles of extremities. MG was considered but tensilon test was equivocal. Repetitive nerve stimulation tests revealed 20-30 percent decrease in responses to low and high rate stimulation. Muscle biopsy revealed selective type 2 atrophy. Ultrastructurally, abnormalities of neuromuscular junctions, i.e., wide primary synaptic cleft, and wide and shallow secondary synaptic clefts with mild myopathic features were present. These findings were pathognomonic for MG. Later, her symptoms were improved completely 3 months after thymectomy. The histologic finding of thymus was follicular hyperplasia.
Adolescence ; Biopsy ; Case Report ; Female ; Human ; Microscopy, Electron ; Mitochondria/ultrastructure ; Mitochondria/pathology ; Muscle, Skeletal/ultrastructure ; Muscle, Skeletal/pathology ; Muscle, Skeletal/enzymology ; Myasthenia Gravis/pathology* ; Myofibrils/ultrastructure ; Myofibrils/pathology ; Myosin ATPase/analysis ; Neuromuscular Junction/ultrastructure* ; Neuromuscular Junction/pathology*

STUDY DESIGN: Case report OBJECTIVES: To report a case of video-assisted thoracoscopic (VAT) minimally invasive anterior interbody fusion of the T11-T12 level using direct lateral interbody fusion (DLIF) devices. SUMMARY OF LITERATURE REVIEW: Interbody fusion of the thoracolumbar junction (especially T11-T12) is technically challenging from anterior, lateral, or posterior approaches. A VAT anterior interbody fusion approach using DLIF devices is a safe, minimally invasive alternative approach to the thoracolumbar spine. MATERIALS AND METHODS: A 37-year-old male pedestrian was struck by a car sustaining fracture-dislocation at the T11-T12 level. The accident resulted in complete paraplegia of both lower extremities and multiple lower extremity fractures. A classical instrumented posterolateral fusion from T8 to L3 and staged VAT anterior interbody fusion at the T11-T12 level were performed. RESULTS: At one year postoperatively, he was capable of independent ambulation using a wheelchair without back pain, and plain radiographs and CT scans showed a solid fusion at the T11-T12 level. CONCLUSIONS: VAT anterior interbody fusion using DLIF devices provides excellent access to the anterior spinal column with the added benefits of an improved field of view and can be a safe and effective alternative to open thoracotomy in the management of various thoracolumbar junction problems.
Adult ; Back Pain ; Humans ; Lower Extremity ; Male ; Minimally Invasive Surgical Procedures ; Paraplegia ; Spine ; Thoracic Surgery, Video-Assisted ; Thoracotomy ; Tomography, X-Ray Computed ; Walking ; Wheelchairs

The purpose of this study was to set the guidelines for selection of patients to do early surgery in ruptured intracranial aneurysm. We assessed 706 patients with single rupture and without large hematoma, who underwent aneurysm surgery from 1985 to 1995. The male and female ratio was 1:1.5. Among the 706 patients, early surgery was performed in 214 cases. The results of early surgery were good in 193 cases(90.2%), fair in 13 cases(6.0%), poor in 1 case(0.5%) and dead in 7 cases(3.3%). The rate of dead outcome in the early surgery group was higher compared to other timing groups. The Fisher group 1, 2 and 3 reveale good outcome in early surgery group;92.6%, 96.3%, 88.8% respectively. The incidence of delayed ischemic deficits(DID) of early surgery group was same as other groups. However, in Fisher group 3, the incidence of DID was significantly low, 32.5%, in early surgery group. It is suggested that the criteria of selection of early surgery in patients with ruptured intracranial aneurysm would include as follows:1) patients with good clinical grade, 2) poor grade patients with marked irritability, acute hydrocephalus, and poorly controlled hypertension, 3) none-complex aneurysm requiring less brain retraction, dissection and brief temporary clipping, 4) age under 60 or over 60 with good physical status, and 5) Fisher group 3 requiring cisternal larvage and anticipated triple-H therapy.
Aneurysm ; Brain ; Female ; Hematoma ; Humans ; Hydrocephalus ; Hypertension ; Incidence ; Intracranial Aneurysm* ; Male ; Patient Selection* ; Rupture

BACKGROUND AND OBJECTIVES: With the increasing use of high resolution sonography and fine needle aspiration biopsy, the proportion of newly diagnosed thyroid papillary carcinomas have increased. However, it is not entirely clear whether microcarcinomas detectable by the above technology the should be considered a threshold for risk evaluation. The purpose of this study is to evaluate the clinical behavior of papillary carcinomas smaller than 1.5 cm. SUBJECTS AND METHOD: A retrospective chart review was conducted for 181 patients who underwent surgery for thyroid cancer and was proven to have papillary carcinoma smaller than 1.5 cm in size from 1997 to 2006. The patients were divided into 3 groups according to cancer size. The patient's gender, age, surgical method, pathology, initial neck node and neck recurrence was analyzed by chi-square test, analysis of variance (ANOVA) test and multinomial logistic regression analysis. RESULTS: Progressively increasing frequency of signs of tumor aggressiveness (multifocal, bilateral, extracapsular spread) was observed with increasing size. The rate of lymph node metastasis increased also, but it did not reach a significant value. Despite the increasing rate of aggressiveness and lymph node metastasis, there was no significant difference in recurrence between these groups. CONCLUSION: Although the long term outcome does not seem to directly depend on tumor size in these small thyroid cancers, a progressing frequency of aggressiveness with increasing cancer size at presentation is evident.
Biopsy ; Biopsy, Fine-Needle ; Carcinoma ; Carcinoma, Papillary ; Humans ; Logistic Models ; Lymph Nodes ; Neck ; Neoplasm Metastasis ; Recurrence ; Retrospective Studies ; Thyroid Gland ; Thyroid Neoplasms

Synovial sarcoma is a rare tumor that usually occur in the extremities of young adults. The head and neck is relatively a rare location as fewer than 100 cases of synovial sarcoma have been reported in the head and neck area. In the head and neck region, however, the most common site is the hypopharynx. There are 12 cases with laryngeal localization in the literature. We experienced a 30-year-old female patient with an endolaryngeal mass. Initial suspension laryngoscopic biopsy specimen of the primary lesion was consistent with laryngeal synovial sarcoma. The tumor arose from the right false vocal cord. It was treated by extended vertical hemilaryngectomy and postoperative radiotherapy. Microscopically, the tumor was characterized by a monophasic fibrous appearance. We report this rare case with a literature review.
Adult ; Biopsy ; Extremities ; Female ; Head ; Humans ; Hypopharynx ; Larynx ; Neck ; Radiotherapy ; Sarcoma, Synovial* ; Vocal Cords ; Young Adult

Chelatable zinc ions from synaptic vesicles have been shown to contribute to neuronal death caused by stroke, epilepsy and head trauma. Elevated glucocorticoid concentration exacerbates such neuron loss, while low levels protect. We have tested the notion that the neuroprotective effect of prior glucocorticoid reduction is mediated by a reduction of zinc ions, i.e. a decrease in the level of zinc ions contained in zinc-enriched (ZEN) synaptic vesicles. The level of vesicular zinc ions was evaluated by zinc selenium AMG (ZnSeAMG) staining at 10 and 30 days after adrenalectomy (ADX). The staining intensity was significantly decreased in the hippocampus following the ADX. Adrenalectomized rats showed proconvulsive seizure behavior, i.e. shortened latency to seizure onset time and increased seizure score 3 hrs after i.p injection of kainic acid (KA). However, adrenalectomized rats showed decreased hippocampal CA3 neuronal death 24 hrs following the KA injection. The convulsive seizure in the ADX rats results from the decreased contents of the vesicular zinc in the presynaptic neurons of the hippocampus. The decreased zinc translocation following the KA injection contributes to the increased neuronal survival rates of postsynaptic neurons against the zinc toxicity in the ADX rats. The present data suggest that the glucocorticoid influences vesicular zinc concentration in the CNS.
Adrenalectomy* ; Animals ; Craniocerebral Trauma ; Epilepsy ; Hippocampus* ; Ions ; Kainic Acid ; Neurons* ; Neuroprotective Agents ; Rats* ; Seizures ; Selenium ; Stroke ; Survival Rate ; Synaptic Vesicles ; Zinc

Oncocytic neoplasm of the head and neck region accounts for approximately 1% of all salivary gland tumors, but only 5% of oncocytic neoplasm is malignant. Oncocytic carcinoma arising in the submandibular gland is exceedingly rare. We encountered a sixty seven-year-old male patient who presented with multiple mass in the right neck. Fine needle aspiration biopsy revealed a salivary gland tumor of predominantly oncocytic form, and a differential diagnosis included oncocytic adenoma or mucoepidermoid carcinoma. A right submandibular gland resection and modified radical neck dissection were performed. Histologically, the tumor cells showed nuclear pleomorphism, and stromal invasion, which were compatible with oncocytic carcinoma. After surgery, the entire neck region was irradiated. Seventeen months after the initial surgery, multiple metastases to the bone and lung were detected from the incidental pathologic bone fracture of the right humerus; palliative chemotherapy was performed to resolve this. We report a case of oncocytic carcinoma in the submandibular gland with a review of literature.
Adenoma ; Biopsy ; Biopsy, Fine-Needle ; Carcinoma, Mucoepidermoid ; Diagnosis, Differential ; Fractures, Bone ; Head ; Humans ; Lung ; Lymphatic Metastasis ; Male ; Neck ; Neck Dissection ; Neoplasm Metastasis ; Salivary Glands ; Submandibular Gland ; Submandibular Gland Neoplasms

BACKGROUND AND PURPOSE: Serial nerve conduction studies (NCSs) are recommended for differentiating axonal and demyelinating Guillain-Barré syndrome (GBS), but this approach is not suitable for early diagnoses. This study was designed to identify possible NCS parameters for differentiating GBS subtypes. METHODS: We retrospectively reviewed the medical records of 70 patients with GBS who underwent NCS within 10 days of symptom onset. Patients with axonal GBS and acute inflammatory demyelinating polyneuropathy (AIDP) were selected based on clinical characteristics and serial NCSs. An antiganglioside antibody study was used to increase the diagnostic certainty. RESULTS: The amplitudes of median and ulnar nerve sensory nerve action potentials (SNAPs) were significantly smaller in the AIDP group than in the axonal-GBS group. Classification and regression-tree analysis revealed that the distal ulnar sensory nerve SNAP amplitude was the best predictor of axonal GBS. CONCLUSIONS: Early upper extremity sensory NCS findings are helpful in differentiating axonal-GBS patients with antiganglioside antibodies from AIDP patients.
Action Potentials ; Antibodies ; Axons* ; Classification ; Diagnosis ; Early Diagnosis ; Electrodiagnosis ; Guillain-Barre Syndrome* ; Humans ; Medical Records ; Neural Conduction ; Retrospective Studies ; Ulnar Nerve ; Upper Extremity*