A thymoma often occurs in patients with myasthenia gravis, but the development of multiple thymoma is very rare. The authors report the radiologic and pathologic findings of multiple invasive thymoma in a 59-year-old male with myasthenia gravis.
Humans ; Male ; Middle Aged ; Myasthenia Gravis* ; Thymoma*

No abstract available.

Hypoxia can occur in pancreatic islets in type 2 diabetes mellitus. Pancreatic stellate cells (PSCs) are activated during hypoxia. Here we aimed to investigate whether PSCs within the islet are also activated in hypoxia, causing β-cell injury.

No abstract available.

Cantú syndrome (CS, OMIM 239850) is a very rare autosomal dominantly inherited genetic disease characterized by congenital hypertrichosis, neonatal macrosomia, a distinct facial features such as macrocephaly, and cardiac defects. Since the first description by Cantú et al. in 1982, about 50 cases have been reported to date. Recently, two causative genes for CS has been found by using exome sequencing analyses: ABCC9 and KCNJ8 . Most cases of clinically diagnosed CS have resulted from de novo mutations in ABCC9. In this study, we report three independent Korean children with CS resulting from de novo ABCC9 mutations. Our patients had common clinical findings such as congenital hypertrichosis, distinctive facial features. One of them showed severe pulmonary hypertension and hypertrophic cardiomyopathy, which require medical treatment. And, two patients had a history of patent ductus arteriosus. Although two of our patients had shown early motor developmental delay, it was gradually improved during follow-up periods. Although CS is quite rare, there are the concerns about development of various cardiac problems in the lifetime. Therefore, an accurate diagnosis followed by appropriate management and genetic counseling should be provided to CS patients.
Cardiomyopathy, Hypertrophic ; Child ; Databases, Genetic ; Diagnosis ; Ductus Arteriosus, Patent ; Exome ; Follow-Up Studies ; Genetic Counseling ; Humans ; Hypertension, Pulmonary ; Hypertrichosis ; Megalencephaly

BACKGROUND AND PURPOSE: The advances in the diagnosis and treatment of acute stroke increase the importance of providing these patients with timely medical attention. This study was designed to assess time delays in neurological evaluation and neuroimaging and to determine whether they are important obstacles to performing thrombolytic therapy. METHODS: Data were obtained between May 2004 and September 2006 from 195 consecutive patients who were admitted to Cheju National University Hospital for acute ischemic stroke within 24 hours of the onset of symptoms. We determined the time of the onset of symptoms, arrival time at the emergency department (ED), and times of neurology notification, neurology evaluation, and neuroimaging using interviews and by reviewing the medical record. RESULTS: Short onset-to-door time, performing computed tomography rather than magnetic resonance imaging, presence of aphasia or motor weakness, and severe initial neurological deficit were significantly associated with reduced in-hospital delays. Seventeen (20%) of the 85 patients who arrived within 3 hours of the onset of symptoms received intravenous thrombolysis. Mild neurological deficit, rapidly improving symptoms, and insufficient time to workup were the main causes of the nonreceipt of thrombolytic therapy in these patients. Only one patient did not receive thrombolytic therapy due to delay in neurology consultation. CONCLUSIONS: Whilst in-hospital delays were not major obstacles to performing thrombolytic therapy in this study, there is still a high probability of missing patients with mild-to-moderate stroke symptoms. More effective in-hospital organization is required for the prompt evaluation and treatment of patients with acute ischemic stroke.
Aphasia ; Cerebrovascular Disorders ; Diagnosis ; Emergency Service, Hospital ; Humans ; Jeju-do ; Magnetic Resonance Imaging ; Medical Records ; Neuroimaging ; Neurology ; Stroke* ; Thrombolytic Therapy* ; Time Factors

It has been well documented that transient forebrain global ischemia causes selective neuronal degeneration in hippocampal CA1 pyramidal neurons with a delay of a few days. The mechanism of this delayed hippocampal CA1 pyramidal neuronal death (DND) is still controversial. To delineate the mechanisms of the DND, the effects of treatment with MK-801, an NMDA receptor antagonist, kynurenic acid, a NMDA/non-NMDA receptor antagonist, and/or cycloheximide, a protein synthesis inhibitor, on the DND were investigated in male Wistar rats. To examine the participation of apoptotic neuronal death in the DND, TUNEL staining was performed in ischemic brain section. Global ischemia was induced by 4-vessel occlusion for 20 min. All animals in this study showed the DND 3 and 7 days after the ischemic insult. The DND that occurred 3 days and 7 days after the ischemia were not affected by pretreatment with MK-801 (I mg/kg), but markedly attenuated by the pretreatment with kynurenic acid (500 mg/kg). Treatment with cycloheximide (1 mg/kg) also markedly inhibited the DND. The magnitudes of attenuation by the two drugs were similar. The magnitude of attenuation by co-treatments with kynurenic acid and cycloheximide was not greater than that with any single treatment. TUNEL staining was negative in the sections obtained 1 or 2 days after the ischemic insults, but it was positive at hippocampal CA1 pyramidal cells in sections collected 3 days after the ischemia. These results suggested that the DND should be mediated by the activation of non-NMDA receptor, not by the activation of NMDA receptor and that the activation of AMPA receptor should induce the apoptotic process in the DND.
Animals ; Apoptosis ; Brain* ; Cycloheximide ; Dizocilpine Maleate ; Excitatory Amino Acid Antagonists* ; Glutamic Acid* ; Humans ; In Situ Nick-End Labeling ; Ischemia* ; Kynurenic Acid ; Male ; N-Methylaspartate ; Neurons* ; Prosencephalon ; Pyramidal Cells ; Rats* ; Rats, Wistar ; Receptors, AMPA ; Receptors, Glutamate*

Background: and PurposePeripheral neuropathies (PNs) are a common but poorly understood complication of chronic obstructive pulmonary disease (COPD). To clarify the initial trigger of a PN in COPD, we investigated the excitability of peripheral nerves in patients with COPD. Methods: The automated nerve excitability test (NET) using the threshold-tracking paradigm was applied to 20 COPD patients. The recording protocol calculated the strength–duration time constant, threshold electrotonus (TE), current–threshold relationship, and recovery cycle (RC). Each NET parameter was compared with two control groups: normal controls group (NC group) and smokers without COPD group (smoker group). Results: In the motor NETs, the change in the threshold in the mid-depolarizing phase of TE (40–60 ms) was smaller in the COPD group (50.7%±1.2%, mean±SEM; n=20) than in the NC group (54.5%±0.7%, n=25; p<0.01), as was the prominence of superexcitability in the RC (-22.6%±1.5% and -26.4%±1.1%, respectively; p=0.04). There were no significant differences in the sensory NETs. Comparisons between the COPD and smoker groups (n=25) also showed no differences in either the motor or sensory NETs. Conclusions The pattern of excitability in COPD revealed a membrane depolarization attributable to Na+–K+–ATPase failure in the axolemma of distal motor nerves. This finding suggests that chronic hypoxemia and adaptative process can alter axonal excitability and trigger a resultant neuropathic process that is antecedent to PN in COPD.

Adult ; Biopsy ; Carcinoma, Mucoepidermoid ; Dentigerous Cyst ; Goblet Cells ; Humans ; Male ; Mandible ; Maxilla ; Molar, Third ; Odontogenic Cysts ; Parotid Gland ; Salivary Glands ; Tooth Extraction

Toxoplasmic encephalitis (TE) is the most common cause of opportunistic central nervous system infection in advanced acquired immunodeficiency syndrome (AIDS) patients. The incidence of TE has fallen markedly after the availability of highly active antiretroviral therapy and cotrimoxazole chemoprophylaxis. TE linked to AIDS is a rare entity in Korea, but we must consider TE in the differential diagnosis of the opportunistic infections in AIDS patients. We report a case of toxoplasmic encephalitis in an advanced AIDS patient presenting as progressive right facial palsy.
Acquired Immunodeficiency Syndrome ; Antiretroviral Therapy, Highly Active ; Central Nervous System Infections ; Chemoprevention ; Diagnosis, Differential ; Encephalitis* ; Facial Paralysis ; Humans ; Incidence ; Korea ; Opportunistic Infections ; Trimethoprim, Sulfamethoxazole Drug Combination